Spitz nevi were first described in 1948.¹ Spitz¹ originally called these lesions benign juvenile melanoma. She was able to identify and describe a separate class of benign melanocytic neoplasms in children that were previously diagnosed and treated as melanoma.² Prior to this discovery, the standard of care was to remove all suspicious pigmented lesions in children prior to adulthood to prevent possible malignant transformation.^2,3 Today, Spitz nevus is the more commonly used term for benign juvenile melanoma because it is encountered occasionally in adults and the term melanoma carries a negative connotation.⁴ Other synonyms include juvenile melanoma, Spitz tumor, nevus of large spindle and/or epithelioid cells, and spindle cell and epithelioid nevus.^3,5 

Classic Spitz Nevus

Spitz nevi are uncommon. The approximate incidence is 7 per 100,000 people. Spitz nevi are more frequently found in children and adolescents but can occur in adults.^6,7 Spitz nevi occur predominantly in the white population and slightly more often in females.^4,8

A Spitz nevus can arise de novo or in association with an existing melanocytic nevus. The lesions can be asymptomatic or have a history of rapid but limited growth. Clinical features of Spitz nevi are well-circumscribed, symmetrical, small- to medium-sized firm papules with smooth discrete borders and a uniform color (typically pink or flesh colored).⁹ Spitz nevi can occur in various shapes. In a study of 211 cases of Spitz nevi, 19% were described as flat or uneven, 24% as polypoid, and 57% as plateau or elevated.⁷ Spitz nevi usually are found on the face, neck, or lower extremities but can occur anywhere on the body.^7,9 Size is typically less than 6 mm (Figures 1 and 2).

PLEASE REFER TO THE PDF TO VIEW THE FIGURES

The classic Spitz nevus histologically consists of large spindle and/or epithelioid melanocytes arrayed as epidermal nests grouped in a vertical orientation (called "bunches of bananas" or "raining down pattern"), with clefting artifact at the perimeter (Figure 3).^4,9,10 The nests are fairly uniform, nonconfluent, and evenly spaced. There is little or no pagetoid spread pattern. Epidermal changes include acanthosis, hypergranulosis, and hyperkeratosis. The intradermal pattern displays maturation, with single-file or single-unit arrays descending to the base. Eosinophilic Kamino bodies frequently are found along the dermoepidermal interface. Kamino bodies are globular clusters that represent apoptotic degenerative melanocytes (Figure 4). They stain positive with both periodic acid-Schiff and trichrome stains. At the dermal base, there is no mitosis, no pushing deep margins, and lack of significant pleomorphism. Little or no melanin is present.^4,9,10 The classic Spitz nevus behaves in a benign manner.¹ The differential diagnosis of the Spitz nevus includes pyogenic granuloma, mastocytoma, juvenile xanthogranuloma, and malignant melanoma.

PLEASE REFER TO THE PDF TO VIEW THE FIGURES

Atypical Spitz Nevus

The atypical Spitz nevus is difficult to formally define. Instead, it is loosely defined. An atypical Spitz nevus shares histologic features with the classic Spitz nevus, but it may have one or more atypical features, which can be characteristic of malignancy.^10-12 Gross atypical features may include irregular shape, nonuniform color, large size, or ulcerations. Histologically, there can be one or more of the following features: pleomorphism; increased cellularity; loss of cellular cohesion; epidermal pagetoid spread; minimal epidermal changes; absence of Kamino bodies; lack of maturation in the intradermal pattern; high-grade nuclear atypia; high basal mitotic rate; pushing deep margins into the dermal base or subcutis; and nests variable in size, shape, and orientation.^9,10,13

The behavior of any atypical Spitz nevus is unpredictable. There are case reports of metastasizing and malignant lesions with Spitz-like characteristics causing fatal outcomes.^11,13 However, there also are studies that show Spitz nevi acting in a benign manner, even with a history of metastases.^11,13-15 Some researchers try to explain this phenomenon by theorizing that Spitz nevi and melanoma exist along a continuum with the classic benign Spitz nevus at one end of the spectrum and the aggressive malignant melanoma at the opposite end, with a diverse range of atypical Spitz-like lesions with features of both in between.^4,10-12,14 Other researchers refute this claim and view the unequivocal Spitz nevus as benign and unrelated to melanoma. They point out that many of these case reports of melanomas with Spitz-like features do not fit the diagnosis of the Spitz nevus.¹⁶

In general, the more features an atypical Spitz nevus shares with melanoma, the greater the risk for malignant behavior. In 1999, Spatz et al¹² proposed formal and specific criteria for determining the risk for malignant behavior in atypical Spitz nevi in children. In the retrospective study, atypical features were used to define atypical Spitz nevi and grade their risk for metastasis. The 5 major factors were age, size, presence of ulceration, involvement of subcutaneous fat, and mitotic activity. Positive risk factors that increased the grade included age greater than 10 years, diameter greater than 10.0 mm, lesions with fat involvement, presence of ulceration, and dermal component mitotic activity greater than 5 mitoses/mm². The higher the grade, the higher the risk for malignancy and metastasis.¹² Since its publication, this grading system for categorizing atypical Spitz nevi has been put to use in a few case reports and studies.^17,18 Additional prospective studies using these criteria will be helpful in determining the true clinical nature of atypical Spitz nevi in children, the usefulness of this grading system, and the possible application of this grading system in adults. 

Problems Differentiating Classic and Atypical Spitz Nevi From Melanoma

Melanoma is a major part of the differential diagnosis of Spitz nevi. The classic Spitz nevus typically has a benign nature, while the atypical Spitz nevus displays unpredictable behavior that appears to be dependent on the degree of atypia.^1,3,16 In contrast, melanoma is potentially fatal. Fortunately, Spitz nevi typically occur in children and the risk for having childhood melanoma is rare.^6,8,19 Though risk is minimal, rare cases of melanoma have been reported in children.^{8,11,14,15,19-21} Therefore, making a correct diagnosis and ruling out melanoma is important.

Unfortunately, even with clinical and histologic guidelines, sometimes it is difficult to distinguish classic and atypical Spitz nevi from melanoma. The major problem is histologic overlap with Spitz nevi and melanoma. Many researchers have emphasized that there is no single discriminating factor for Spitz nevi and melanoma because virtually every trait of Spitz nevi has been described in melanoma.^{2,10,13,20,22,23} Results of multiple studies show variability among researchers on the analysis of melanocytic nevi and melanoma lesions, and the final diagnosis was subjective.^5,22 In one retrospective study where clinical outcome was already known, 30 melanocytic lesions were evaluated independently by a panel of 10 dermatopathologists and categorized as either a typical Spitz nevus, atypical Spitz nevus, melanoma, tumor with unknown biologic potential, or other melanocytic lesion.⁵ The dermatopathologists were blinded to the clinical data. Evaluation of 17 Spitzoid lesions yielded no clear diagnostic consensus and a few lethal lesions were identified by most dermatopathologists as either typical or atypical Spitz nevi. The authors maintain that these results show that current objective criteria are deficient and inadequate to permit the discrimination of Spitz nevi with atypical features from melanoma.⁵

Given these histologic analysis limitations, many investigators are researching other tools and techniques that may help enhance diagnostic accuracy. Promising genetic analysis techniques include comparative genomic hybridization and fluorescent in situ hybridization.²⁴ In one study,²⁴ researchers compared Spitz nevi with primary cutaneous melanomas using comparative genomic hybridization and fluorescent in situ hybridization and discovered differences. In the study, Spitz nevi were found to have no chromosomal aberrations or gains in chromosome 11p or 7q21qter. In comparison, primary cutaneous melanomas had frequent chromosome deletions of chromosomes 9p, 10q, 6q, and 8p, and gains of chromosomes 7, 8, 6p, and 1q.^24,25 Immunohistochemistry is another potential tool for improving diagnostic accuracy. Examples of promising immunohistochemical markers include antibody MIB-1,^26-28 BCL-2,²⁹ and anti-S100A6.³⁰ Studies have shown that most melanomas are immunoreactive to MIB-1 and BCL-2, whereas Spitz nevi are not.^26-29 Recently, anti-S100A6 protein also was shown to be a potential immunohistochemical marker to differentiate a Spitz nevus from melanoma.³⁰ Anti-S100A6 is different from anti-S100 because it is more specific to a subclass of normal cell types and certain cancer cell lines. Investigators found strong, uniform, and diffuse S100A6 protein expression in the junctional and dermal components of all 42 Spitz nevi they studied versus weak and patchy S100A6 protein expression found mainly in the dermal component of 35 of 105 melanoma specimens they studied.³⁰ Although these techniques show exceptional potential, further research will be required to prove their reliability. 

Management of Classic and Atypical Spitz Nevi

There is controversy regarding the treatment of a classic Spitz nevus. Some investigators recommend conservative treatment because a Spitz nevus is benign. They find that the Spitz nevus may be removed or left alone.³ Others agree but would add that complete excision with clinical follow-up is appropriate if there are atypical features found on the Spitz nevus.^16,23,31 Other investigators are more aggressive and recommend complete excision with clear margins of all Spitz nevi, unequivocal or not, because Spitz nevi have histologic overlap with melanoma, and recurrent lesions may present with pseudomelanomatous changes, which makes differentiation more difficult later.^4,32 They conclude that the benefits of complete excision outweigh the risks of partial treatment.⁴ Regardless of how a Spitz nevus case is managed, regular follow-up with a dermatologist is recommended to look for any changes or recurrences suggestive of malignancy.

Currently, there are no available evidence-based recommendations with predictive value for the specific management of atypical Spitz nevi because their clinical course is mostly unknown and unpredictable. Most articles that do address the management of atypical Spitz nevi state that they should be completely excised and followed periodically.^11,33 Murphy et al³⁴ suggest that an atypical Spitz nevus should be completely excised to avoid the rare possibility of a melanoma masquerading as an atypical Spitz nevus. Furthermore, if the physician is suspicious of malignancy, it is recommended that the lesion be managed like a melanoma and be removed in accordance with current melanoma margin guidelines or with comprehensive margin control via Mohs micrographic surgery.^34,35 Gurbuz et al¹⁷ stated that surgical margin excision, sentinel lymph node dissection, and clinical follow-up is recommended for atypical Spitz tumors. However, currently there are no prospective studies that have tested these various recommendations on atypical Spitz nevi management.

Within the last few years, sentinel lymph node biopsy (SLNB) has been proposed as a useful tool in the management of melanocytic neoplasms of uncertain behavior, such as the atypical Spitz nevus.³⁶ Researchers recommend SLNB in atypical Spitz nevi greater than 1.0-mm thick.^18,36,37 Supporters maintain that it increases the sensitivity of the diagnosis of melanoma (vs atypical Spitz nevus) and identifies patients who may potentially benefit from early lymph node dissection and/or adjuvant therapy. They state that a positive SLNB supports the diagnosis of malignancy and recommend that the lesion be treated aggressively. If the SLNB is negative, melanoma cannot be completely ruled out, but there is more reassurance that the lesion may be confined to the skin and can be completely removed by excision.^18,36,37 Other advantages of SLNB include minimal invasiveness and morbidity. Some researchers believe melanocytic neoplasms in which melanoma cannot be ruled out should undergo complete surgical excision with wide margins in accordance with current melanoma guidelines,^34,35 which can be as much as 3 cm.^36,38 A negative SLNB offers the advantage of planning a complete excision of an atypical Spitz nevus that preserves surrounding margins and is cosmetically more acceptable,³⁶ and avoiding the morbidity (ie, lymphedema, paresthesia) associated with regional or elective lymph node dissection.¹⁸

However, some researchers argue that a positive SLNB in an atypical Spitz nevus is not metastatic melanoma and point out articles that have shown classic and atypical Spitz nevi spreading to lymphatic vessels and lymph nodes but behaving in a benign manner.^{11,13,15,21,37} Therefore, more studies are needed to assess the prognostic significance of positive SLNB in atypical Spitz nevi.¹⁸

Spitz nevi were first described in 1948.¹ Spitz¹ originally called these lesions benign juvenile melanoma. She was able to identify and describe a separate class of benign melanocytic neoplasms in children that were previously diagnosed and treated as melanoma.² Prior to this discovery, the standard of care was to remove all suspicious pigmented lesions in children prior to adulthood to prevent possible malignant transformation.^2,3 Today, Spitz nevus is the more commonly used term for benign juvenile melanoma because it is encountered occasionally in adults and the term melanoma carries a negative connotation.⁴ Other synonyms include juvenile melanoma, Spitz tumor, nevus of large spindle and/or epithelioid cells, and spindle cell and epithelioid nevus.^3,5 

Classic Spitz Nevus

Spitz nevi are uncommon. The approximate incidence is 7 per 100,000 people. Spitz nevi are more frequently found in children and adolescents but can occur in adults.^6,7 Spitz nevi occur predominantly in the white population and slightly more often in females.^4,8

A Spitz nevus can arise de novo or in association with an existing melanocytic nevus. The lesions can be asymptomatic or have a history of rapid but limited growth. Clinical features of Spitz nevi are well-circumscribed, symmetrical, small- to medium-sized firm papules with smooth discrete borders and a uniform color (typically pink or flesh colored).⁹ Spitz nevi can occur in various shapes. In a study of 211 cases of Spitz nevi, 19% were described as flat or uneven, 24% as polypoid, and 57% as plateau or elevated.⁷ Spitz nevi usually are found on the face, neck, or lower extremities but can occur anywhere on the body.^7,9 Size is typically less than 6 mm (Figures 1 and 2).

PLEASE REFER TO THE PDF TO VIEW THE FIGURES

The classic Spitz nevus histologically consists of large spindle and/or epithelioid melanocytes arrayed as epidermal nests grouped in a vertical orientation (called "bunches of bananas" or "raining down pattern"), with clefting artifact at the perimeter (Figure 3).^4,9,10 The nests are fairly uniform, nonconfluent, and evenly spaced. There is little or no pagetoid spread pattern. Epidermal changes include acanthosis, hypergranulosis, and hyperkeratosis. The intradermal pattern displays maturation, with single-file or single-unit arrays descending to the base. Eosinophilic Kamino bodies frequently are found along the dermoepidermal interface. Kamino bodies are globular clusters that represent apoptotic degenerative melanocytes (Figure 4). They stain positive with both periodic acid-Schiff and trichrome stains. At the dermal base, there is no mitosis, no pushing deep margins, and lack of significant pleomorphism. Little or no melanin is present.^4,9,10 The classic Spitz nevus behaves in a benign manner.¹ The differential diagnosis of the Spitz nevus includes pyogenic granuloma, mastocytoma, juvenile xanthogranuloma, and malignant melanoma.

PLEASE REFER TO THE PDF TO VIEW THE FIGURES

Atypical Spitz Nevus

The atypical Spitz nevus is difficult to formally define. Instead, it is loosely defined. An atypical Spitz nevus shares histologic features with the classic Spitz nevus, but it may have one or more atypical features, which can be characteristic of malignancy.^10-12 Gross atypical features may include irregular shape, nonuniform color, large size, or ulcerations. Histologically, there can be one or more of the following features: pleomorphism; increased cellularity; loss of cellular cohesion; epidermal pagetoid spread; minimal epidermal changes; absence of Kamino bodies; lack of maturation in the intradermal pattern; high-grade nuclear atypia; high basal mitotic rate; pushing deep margins into the dermal base or subcutis; and nests variable in size, shape, and orientation.^9,10,13

The behavior of any atypical Spitz nevus is unpredictable. There are case reports of metastasizing and malignant lesions with Spitz-like characteristics causing fatal outcomes.^11,13 However, there also are studies that show Spitz nevi acting in a benign manner, even with a history of metastases.^11,13-15 Some researchers try to explain this phenomenon by theorizing that Spitz nevi and melanoma exist along a continuum with the classic benign Spitz nevus at one end of the spectrum and the aggressive malignant melanoma at the opposite end, with a diverse range of atypical Spitz-like lesions with features of both in between.^4,10-12,14 Other researchers refute this claim and view the unequivocal Spitz nevus as benign and unrelated to melanoma. They point out that many of these case reports of melanomas with Spitz-like features do not fit the diagnosis of the Spitz nevus.¹⁶

In general, the more features an atypical Spitz nevus shares with melanoma, the greater the risk for malignant behavior. In 1999, Spatz et al¹² proposed formal and specific criteria for determining the risk for malignant behavior in atypical Spitz nevi in children. In the retrospective study, atypical features were used to define atypical Spitz nevi and grade their risk for metastasis. The 5 major factors were age, size, presence of ulceration, involvement of subcutaneous fat, and mitotic activity. Positive risk factors that increased the grade included age greater than 10 years, diameter greater than 10.0 mm, lesions with fat involvement, presence of ulceration, and dermal component mitotic activity greater than 5 mitoses/mm². The higher the grade, the higher the risk for malignancy and metastasis.¹² Since its publication, this grading system for categorizing atypical Spitz nevi has been put to use in a few case reports and studies.^17,18 Additional prospective studies using these criteria will be helpful in determining the true clinical nature of atypical Spitz nevi in children, the usefulness of this grading system, and the possible application of this grading system in adults. 

Problems Differentiating Classic and Atypical Spitz Nevi From Melanoma

Melanoma is a major part of the differential diagnosis of Spitz nevi. The classic Spitz nevus typically has a benign nature, while the atypical Spitz nevus displays unpredictable behavior that appears to be dependent on the degree of atypia.^1,3,16 In contrast, melanoma is potentially fatal. Fortunately, Spitz nevi typically occur in children and the risk for having childhood melanoma is rare.^6,8,19 Though risk is minimal, rare cases of melanoma have been reported in children.^{8,11,14,15,19-21} Therefore, making a correct diagnosis and ruling out melanoma is important.

Unfortunately, even with clinical and histologic guidelines, sometimes it is difficult to distinguish classic and atypical Spitz nevi from melanoma. The major problem is histologic overlap with Spitz nevi and melanoma. Many researchers have emphasized that there is no single discriminating factor for Spitz nevi and melanoma because virtually every trait of Spitz nevi has been described in melanoma.^{2,10,13,20,22,23} Results of multiple studies show variability among researchers on the analysis of melanocytic nevi and melanoma lesions, and the final diagnosis was subjective.^5,22 In one retrospective study where clinical outcome was already known, 30 melanocytic lesions were evaluated independently by a panel of 10 dermatopathologists and categorized as either a typical Spitz nevus, atypical Spitz nevus, melanoma, tumor with unknown biologic potential, or other melanocytic lesion.⁵ The dermatopathologists were blinded to the clinical data. Evaluation of 17 Spitzoid lesions yielded no clear diagnostic consensus and a few lethal lesions were identified by most dermatopathologists as either typical or atypical Spitz nevi. The authors maintain that these results show that current objective criteria are deficient and inadequate to permit the discrimination of Spitz nevi with atypical features from melanoma.⁵

Given these histologic analysis limitations, many investigators are researching other tools and techniques that may help enhance diagnostic accuracy. Promising genetic analysis techniques include comparative genomic hybridization and fluorescent in situ hybridization.²⁴ In one study,²⁴ researchers compared Spitz nevi with primary cutaneous melanomas using comparative genomic hybridization and fluorescent in situ hybridization and discovered differences. In the study, Spitz nevi were found to have no chromosomal aberrations or gains in chromosome 11p or 7q21qter. In comparison, primary cutaneous melanomas had frequent chromosome deletions of chromosomes 9p, 10q, 6q, and 8p, and gains of chromosomes 7, 8, 6p, and 1q.^24,25 Immunohistochemistry is another potential tool for improving diagnostic accuracy. Examples of promising immunohistochemical markers include antibody MIB-1,^26-28 BCL-2,²⁹ and anti-S100A6.³⁰ Studies have shown that most melanomas are immunoreactive to MIB-1 and BCL-2, whereas Spitz nevi are not.^26-29 Recently, anti-S100A6 protein also was shown to be a potential immunohistochemical marker to differentiate a Spitz nevus from melanoma.³⁰ Anti-S100A6 is different from anti-S100 because it is more specific to a subclass of normal cell types and certain cancer cell lines. Investigators found strong, uniform, and diffuse S100A6 protein expression in the junctional and dermal components of all 42 Spitz nevi they studied versus weak and patchy S100A6 protein expression found mainly in the dermal component of 35 of 105 melanoma specimens they studied.³⁰ Although these techniques show exceptional potential, further research will be required to prove their reliability. 

Management of Classic and Atypical Spitz Nevi

There is controversy regarding the treatment of a classic Spitz nevus. Some investigators recommend conservative treatment because a Spitz nevus is benign. They find that the Spitz nevus may be removed or left alone.³ Others agree but would add that complete excision with clinical follow-up is appropriate if there are atypical features found on the Spitz nevus.^16,23,31 Other investigators are more aggressive and recommend complete excision with clear margins of all Spitz nevi, unequivocal or not, because Spitz nevi have histologic overlap with melanoma, and recurrent lesions may present with pseudomelanomatous changes, which makes differentiation more difficult later.^4,32 They conclude that the benefits of complete excision outweigh the risks of partial treatment.⁴ Regardless of how a Spitz nevus case is managed, regular follow-up with a dermatologist is recommended to look for any changes or recurrences suggestive of malignancy.

Currently, there are no available evidence-based recommendations with predictive value for the specific management of atypical Spitz nevi because their clinical course is mostly unknown and unpredictable. Most articles that do address the management of atypical Spitz nevi state that they should be completely excised and followed periodically.^11,33 Murphy et al³⁴ suggest that an atypical Spitz nevus should be completely excised to avoid the rare possibility of a melanoma masquerading as an atypical Spitz nevus. Furthermore, if the physician is suspicious of malignancy, it is recommended that the lesion be managed like a melanoma and be removed in accordance with current melanoma margin guidelines or with comprehensive margin control via Mohs micrographic surgery.^34,35 Gurbuz et al¹⁷ stated that surgical margin excision, sentinel lymph node dissection, and clinical follow-up is recommended for atypical Spitz tumors. However, currently there are no prospective studies that have tested these various recommendations on atypical Spitz nevi management.

Within the last few years, sentinel lymph node biopsy (SLNB) has been proposed as a useful tool in the management of melanocytic neoplasms of uncertain behavior, such as the atypical Spitz nevus.³⁶ Researchers recommend SLNB in atypical Spitz nevi greater than 1.0-mm thick.^18,36,37 Supporters maintain that it increases the sensitivity of the diagnosis of melanoma (vs atypical Spitz nevus) and identifies patients who may potentially benefit from early lymph node dissection and/or adjuvant therapy. They state that a positive SLNB supports the diagnosis of malignancy and recommend that the lesion be treated aggressively. If the SLNB is negative, melanoma cannot be completely ruled out, but there is more reassurance that the lesion may be confined to the skin and can be completely removed by excision.^18,36,37 Other advantages of SLNB include minimal invasiveness and morbidity. Some researchers believe melanocytic neoplasms in which melanoma cannot be ruled out should undergo complete surgical excision with wide margins in accordance with current melanoma guidelines,^34,35 which can be as much as 3 cm.^36,38 A negative SLNB offers the advantage of planning a complete excision of an atypical Spitz nevus that preserves surrounding margins and is cosmetically more acceptable,³⁶ and avoiding the morbidity (ie, lymphedema, paresthesia) associated with regional or elective lymph node dissection.¹⁸

However, some researchers argue that a positive SLNB in an atypical Spitz nevus is not metastatic melanoma and point out articles that have shown classic and atypical Spitz nevi spreading to lymphatic vessels and lymph nodes but behaving in a benign manner.^{11,13,15,21,37} Therefore, more studies are needed to assess the prognostic significance of positive SLNB in atypical Spitz nevi.¹⁸

Spitz nevi were first described in 1948.¹ Spitz¹ originally called these lesions benign juvenile melanoma. She was able to identify and describe a separate class of benign melanocytic neoplasms in children that were previously diagnosed and treated as melanoma.² Prior to this discovery, the standard of care was to remove all suspicious pigmented lesions in children prior to adulthood to prevent possible malignant transformation.^2,3 Today, Spitz nevus is the more commonly used term for benign juvenile melanoma because it is encountered occasionally in adults and the term melanoma carries a negative connotation.⁴ Other synonyms include juvenile melanoma, Spitz tumor, nevus of large spindle and/or epithelioid cells, and spindle cell and epithelioid nevus.^3,5 

Classic Spitz Nevus

Spitz nevi are uncommon. The approximate incidence is 7 per 100,000 people. Spitz nevi are more frequently found in children and adolescents but can occur in adults.^6,7 Spitz nevi occur predominantly in the white population and slightly more often in females.^4,8

A Spitz nevus can arise de novo or in association with an existing melanocytic nevus. The lesions can be asymptomatic or have a history of rapid but limited growth. Clinical features of Spitz nevi are well-circumscribed, symmetrical, small- to medium-sized firm papules with smooth discrete borders and a uniform color (typically pink or flesh colored).⁹ Spitz nevi can occur in various shapes. In a study of 211 cases of Spitz nevi, 19% were described as flat or uneven, 24% as polypoid, and 57% as plateau or elevated.⁷ Spitz nevi usually are found on the face, neck, or lower extremities but can occur anywhere on the body.^7,9 Size is typically less than 6 mm (Figures 1 and 2).

PLEASE REFER TO THE PDF TO VIEW THE FIGURES

The classic Spitz nevus histologically consists of large spindle and/or epithelioid melanocytes arrayed as epidermal nests grouped in a vertical orientation (called "bunches of bananas" or "raining down pattern"), with clefting artifact at the perimeter (Figure 3).^4,9,10 The nests are fairly uniform, nonconfluent, and evenly spaced. There is little or no pagetoid spread pattern. Epidermal changes include acanthosis, hypergranulosis, and hyperkeratosis. The intradermal pattern displays maturation, with single-file or single-unit arrays descending to the base. Eosinophilic Kamino bodies frequently are found along the dermoepidermal interface. Kamino bodies are globular clusters that represent apoptotic degenerative melanocytes (Figure 4). They stain positive with both periodic acid-Schiff and trichrome stains. At the dermal base, there is no mitosis, no pushing deep margins, and lack of significant pleomorphism. Little or no melanin is present.^4,9,10 The classic Spitz nevus behaves in a benign manner.¹ The differential diagnosis of the Spitz nevus includes pyogenic granuloma, mastocytoma, juvenile xanthogranuloma, and malignant melanoma.

PLEASE REFER TO THE PDF TO VIEW THE FIGURES

Atypical Spitz Nevus

The atypical Spitz nevus is difficult to formally define. Instead, it is loosely defined. An atypical Spitz nevus shares histologic features with the classic Spitz nevus, but it may have one or more atypical features, which can be characteristic of malignancy.^10-12 Gross atypical features may include irregular shape, nonuniform color, large size, or ulcerations. Histologically, there can be one or more of the following features: pleomorphism; increased cellularity; loss of cellular cohesion; epidermal pagetoid spread; minimal epidermal changes; absence of Kamino bodies; lack of maturation in the intradermal pattern; high-grade nuclear atypia; high basal mitotic rate; pushing deep margins into the dermal base or subcutis; and nests variable in size, shape, and orientation.^9,10,13

The behavior of any atypical Spitz nevus is unpredictable. There are case reports of metastasizing and malignant lesions with Spitz-like characteristics causing fatal outcomes.^11,13 However, there also are studies that show Spitz nevi acting in a benign manner, even with a history of metastases.^11,13-15 Some researchers try to explain this phenomenon by theorizing that Spitz nevi and melanoma exist along a continuum with the classic benign Spitz nevus at one end of the spectrum and the aggressive malignant melanoma at the opposite end, with a diverse range of atypical Spitz-like lesions with features of both in between.^4,10-12,14 Other researchers refute this claim and view the unequivocal Spitz nevus as benign and unrelated to melanoma. They point out that many of these case reports of melanomas with Spitz-like features do not fit the diagnosis of the Spitz nevus.¹⁶

In general, the more features an atypical Spitz nevus shares with melanoma, the greater the risk for malignant behavior. In 1999, Spatz et al¹² proposed formal and specific criteria for determining the risk for malignant behavior in atypical Spitz nevi in children. In the retrospective study, atypical features were used to define atypical Spitz nevi and grade their risk for metastasis. The 5 major factors were age, size, presence of ulceration, involvement of subcutaneous fat, and mitotic activity. Positive risk factors that increased the grade included age greater than 10 years, diameter greater than 10.0 mm, lesions with fat involvement, presence of ulceration, and dermal component mitotic activity greater than 5 mitoses/mm². The higher the grade, the higher the risk for malignancy and metastasis.¹² Since its publication, this grading system for categorizing atypical Spitz nevi has been put to use in a few case reports and studies.^17,18 Additional prospective studies using these criteria will be helpful in determining the true clinical nature of atypical Spitz nevi in children, the usefulness of this grading system, and the possible application of this grading system in adults. 

Problems Differentiating Classic and Atypical Spitz Nevi From Melanoma

Melanoma is a major part of the differential diagnosis of Spitz nevi. The classic Spitz nevus typically has a benign nature, while the atypical Spitz nevus displays unpredictable behavior that appears to be dependent on the degree of atypia.^1,3,16 In contrast, melanoma is potentially fatal. Fortunately, Spitz nevi typically occur in children and the risk for having childhood melanoma is rare.^6,8,19 Though risk is minimal, rare cases of melanoma have been reported in children.^{8,11,14,15,19-21} Therefore, making a correct diagnosis and ruling out melanoma is important.

Unfortunately, even with clinical and histologic guidelines, sometimes it is difficult to distinguish classic and atypical Spitz nevi from melanoma. The major problem is histologic overlap with Spitz nevi and melanoma. Many researchers have emphasized that there is no single discriminating factor for Spitz nevi and melanoma because virtually every trait of Spitz nevi has been described in melanoma.^{2,10,13,20,22,23} Results of multiple studies show variability among researchers on the analysis of melanocytic nevi and melanoma lesions, and the final diagnosis was subjective.^5,22 In one retrospective study where clinical outcome was already known, 30 melanocytic lesions were evaluated independently by a panel of 10 dermatopathologists and categorized as either a typical Spitz nevus, atypical Spitz nevus, melanoma, tumor with unknown biologic potential, or other melanocytic lesion.⁵ The dermatopathologists were blinded to the clinical data. Evaluation of 17 Spitzoid lesions yielded no clear diagnostic consensus and a few lethal lesions were identified by most dermatopathologists as either typical or atypical Spitz nevi. The authors maintain that these results show that current objective criteria are deficient and inadequate to permit the discrimination of Spitz nevi with atypical features from melanoma.⁵

Given these histologic analysis limitations, many investigators are researching other tools and techniques that may help enhance diagnostic accuracy. Promising genetic analysis techniques include comparative genomic hybridization and fluorescent in situ hybridization.²⁴ In one study,²⁴ researchers compared Spitz nevi with primary cutaneous melanomas using comparative genomic hybridization and fluorescent in situ hybridization and discovered differences. In the study, Spitz nevi were found to have no chromosomal aberrations or gains in chromosome 11p or 7q21qter. In comparison, primary cutaneous melanomas had frequent chromosome deletions of chromosomes 9p, 10q, 6q, and 8p, and gains of chromosomes 7, 8, 6p, and 1q.^24,25 Immunohistochemistry is another potential tool for improving diagnostic accuracy. Examples of promising immunohistochemical markers include antibody MIB-1,^26-28 BCL-2,²⁹ and anti-S100A6.³⁰ Studies have shown that most melanomas are immunoreactive to MIB-1 and BCL-2, whereas Spitz nevi are not.^26-29 Recently, anti-S100A6 protein also was shown to be a potential immunohistochemical marker to differentiate a Spitz nevus from melanoma.³⁰ Anti-S100A6 is different from anti-S100 because it is more specific to a subclass of normal cell types and certain cancer cell lines. Investigators found strong, uniform, and diffuse S100A6 protein expression in the junctional and dermal components of all 42 Spitz nevi they studied versus weak and patchy S100A6 protein expression found mainly in the dermal component of 35 of 105 melanoma specimens they studied.³⁰ Although these techniques show exceptional potential, further research will be required to prove their reliability. 

Management of Classic and Atypical Spitz Nevi

There is controversy regarding the treatment of a classic Spitz nevus. Some investigators recommend conservative treatment because a Spitz nevus is benign. They find that the Spitz nevus may be removed or left alone.³ Others agree but would add that complete excision with clinical follow-up is appropriate if there are atypical features found on the Spitz nevus.^16,23,31 Other investigators are more aggressive and recommend complete excision with clear margins of all Spitz nevi, unequivocal or not, because Spitz nevi have histologic overlap with melanoma, and recurrent lesions may present with pseudomelanomatous changes, which makes differentiation more difficult later.^4,32 They conclude that the benefits of complete excision outweigh the risks of partial treatment.⁴ Regardless of how a Spitz nevus case is managed, regular follow-up with a dermatologist is recommended to look for any changes or recurrences suggestive of malignancy.

Currently, there are no available evidence-based recommendations with predictive value for the specific management of atypical Spitz nevi because their clinical course is mostly unknown and unpredictable. Most articles that do address the management of atypical Spitz nevi state that they should be completely excised and followed periodically.^11,33 Murphy et al³⁴ suggest that an atypical Spitz nevus should be completely excised to avoid the rare possibility of a melanoma masquerading as an atypical Spitz nevus. Furthermore, if the physician is suspicious of malignancy, it is recommended that the lesion be managed like a melanoma and be removed in accordance with current melanoma margin guidelines or with comprehensive margin control via Mohs micrographic surgery.^34,35 Gurbuz et al¹⁷ stated that surgical margin excision, sentinel lymph node dissection, and clinical follow-up is recommended for atypical Spitz tumors. However, currently there are no prospective studies that have tested these various recommendations on atypical Spitz nevi management.

Within the last few years, sentinel lymph node biopsy (SLNB) has been proposed as a useful tool in the management of melanocytic neoplasms of uncertain behavior, such as the atypical Spitz nevus.³⁶ Researchers recommend SLNB in atypical Spitz nevi greater than 1.0-mm thick.^18,36,37 Supporters maintain that it increases the sensitivity of the diagnosis of melanoma (vs atypical Spitz nevus) and identifies patients who may potentially benefit from early lymph node dissection and/or adjuvant therapy. They state that a positive SLNB supports the diagnosis of malignancy and recommend that the lesion be treated aggressively. If the SLNB is negative, melanoma cannot be completely ruled out, but there is more reassurance that the lesion may be confined to the skin and can be completely removed by excision.^18,36,37 Other advantages of SLNB include minimal invasiveness and morbidity. Some researchers believe melanocytic neoplasms in which melanoma cannot be ruled out should undergo complete surgical excision with wide margins in accordance with current melanoma guidelines,^34,35 which can be as much as 3 cm.^36,38 A negative SLNB offers the advantage of planning a complete excision of an atypical Spitz nevus that preserves surrounding margins and is cosmetically more acceptable,³⁶ and avoiding the morbidity (ie, lymphedema, paresthesia) associated with regional or elective lymph node dissection.¹⁸

However, some researchers argue that a positive SLNB in an atypical Spitz nevus is not metastatic melanoma and point out articles that have shown classic and atypical Spitz nevi spreading to lymphatic vessels and lymph nodes but behaving in a benign manner.^{11,13,15,21,37} Therefore, more studies are needed to assess the prognostic significance of positive SLNB in atypical Spitz nevi.¹⁸

Evidence summary

Mild persistent asthma is defined as forced expiratory volume over 1 second (FEV₁) ≥80% predicted, with daytime symptoms more than twice per week but less than once daily, and nighttime symptoms more often than twice monthly.¹

Low-dose inhaled corticosteroids

Two large randomized trials support using low-dose inhaled corticosteroids in these children. The Childhood Asthma Management Program (CAMP) study, which included 1041 children, evaluated treatment with either budesonide or nedocromil vs placebo. Patients taking budesonide had a lower rate of urgent care visits (absolute risk reduction [ARR]=10%; number needed to treat [NNT]=10; P=.02) compared with children taking nedocromil (ARR=6%; NNT=17; P=.02). The urgent care visits were reported as number of visits per 100 person-years.

In practical terms, this means that in order to decrease 1 urgent care visit, 1 patient would need to take budesonide for 10 years. However, because rates are not necessarily homogenous over time, the number of visits decreased during the first year may be different than the number of events decreased throughout the tenth year.

Children taking budesonide experienced 21.5% more episode-free days than those taking placebo (P=.01). No change was observed in the nedocromil group.² In the inhaled Steroid Treatment As Regular Therapy (START) in early asthma study, budesonide demonstrated a 44% relative reduction in time to first severe asthma related event, compared with placebo (95% confidence interval [CI], 0.45–0.71; NNT=44; P=.0001).³

Theophylline

Theophylline is considered an alternative to inhaled corticosteroids. One study compared beclomethasone with theophylline in 195 children. This study found near-equivalent efficacy in doctor visits, hospitalizations, monthly peak expiratory flow rates, and FEV₁; however, beclomethasone was superior to theophylline in maintaining symptom control and decreasing the use of inhaled bronchodilators and systemic steroids.

When compared with beclomethasone, theophylline was linked to 14% more central nervous system adverse effects (P<.001) and 17% more gastrointestinal disturbances (P<.001). Although beclomethasone induced more oral candidiasis compared with theophylline (8.9% vs 2.4%; P<.001), the incidence of this infection can be reduced by using a spacer.

Long-term systemic effects

The potential long-term adverse systemic effects of inhaled corticosteroids on growth, bone metabolism, and pituitary-adrenal function call for longer-term studies.⁴ A systematic review of 15 trials reported that the protective effect of leukotriene receptor antagonists is inferior to inhaled corticosteroids for adults (relative risk [RR]=1.71; 95% CI, 1.40–2.09); however, evidence is insufficient to extrapolate this to children.⁵

Beta-agonists

Evidence does not support use of long-acting beta-agonists as monotherapy or in combination with other medications for children with mild persistent asthma. Although 1 study showed an improvement in lung function for children taking budesonide plus formoterol compared with budesonide alone, the rate of severe exacerbations was lower for those taking budesonide alone (62% decrease vs 55.8% decrease; P=.001). Both groups had a 32% decrease in the number of rescue inhalations per day when compared with placebo (P=.0008).⁶

Recommendations from others

Recommendations are listed in the TABLE.^1,7,8 Unlike the NAEPP and GINA asthma guidelines, the BTS/SIGN asthma guidelines define no objective measurement or staging classification to diagnose asthma among children. Diagnosis is determined by a child’s response to medication.⁸ Independent of any daily controller medication use, all children should have a short acting bronchodilator on hand in case of an acute attack.^1,8

TABLE
Recommendations for treating mild persistent asthma

Evidence summary

Mild persistent asthma is defined as forced expiratory volume over 1 second (FEV₁) ≥80% predicted, with daytime symptoms more than twice per week but less than once daily, and nighttime symptoms more often than twice monthly.¹

Low-dose inhaled corticosteroids

Two large randomized trials support using low-dose inhaled corticosteroids in these children. The Childhood Asthma Management Program (CAMP) study, which included 1041 children, evaluated treatment with either budesonide or nedocromil vs placebo. Patients taking budesonide had a lower rate of urgent care visits (absolute risk reduction [ARR]=10%; number needed to treat [NNT]=10; P=.02) compared with children taking nedocromil (ARR=6%; NNT=17; P=.02). The urgent care visits were reported as number of visits per 100 person-years.

In practical terms, this means that in order to decrease 1 urgent care visit, 1 patient would need to take budesonide for 10 years. However, because rates are not necessarily homogenous over time, the number of visits decreased during the first year may be different than the number of events decreased throughout the tenth year.

Children taking budesonide experienced 21.5% more episode-free days than those taking placebo (P=.01). No change was observed in the nedocromil group.² In the inhaled Steroid Treatment As Regular Therapy (START) in early asthma study, budesonide demonstrated a 44% relative reduction in time to first severe asthma related event, compared with placebo (95% confidence interval [CI], 0.45–0.71; NNT=44; P=.0001).³

Theophylline

Theophylline is considered an alternative to inhaled corticosteroids. One study compared beclomethasone with theophylline in 195 children. This study found near-equivalent efficacy in doctor visits, hospitalizations, monthly peak expiratory flow rates, and FEV₁; however, beclomethasone was superior to theophylline in maintaining symptom control and decreasing the use of inhaled bronchodilators and systemic steroids.

When compared with beclomethasone, theophylline was linked to 14% more central nervous system adverse effects (P<.001) and 17% more gastrointestinal disturbances (P<.001). Although beclomethasone induced more oral candidiasis compared with theophylline (8.9% vs 2.4%; P<.001), the incidence of this infection can be reduced by using a spacer.

Long-term systemic effects

The potential long-term adverse systemic effects of inhaled corticosteroids on growth, bone metabolism, and pituitary-adrenal function call for longer-term studies.⁴ A systematic review of 15 trials reported that the protective effect of leukotriene receptor antagonists is inferior to inhaled corticosteroids for adults (relative risk [RR]=1.71; 95% CI, 1.40–2.09); however, evidence is insufficient to extrapolate this to children.⁵

Beta-agonists

Evidence does not support use of long-acting beta-agonists as monotherapy or in combination with other medications for children with mild persistent asthma. Although 1 study showed an improvement in lung function for children taking budesonide plus formoterol compared with budesonide alone, the rate of severe exacerbations was lower for those taking budesonide alone (62% decrease vs 55.8% decrease; P=.001). Both groups had a 32% decrease in the number of rescue inhalations per day when compared with placebo (P=.0008).⁶

Recommendations from others

Recommendations are listed in the TABLE.^1,7,8 Unlike the NAEPP and GINA asthma guidelines, the BTS/SIGN asthma guidelines define no objective measurement or staging classification to diagnose asthma among children. Diagnosis is determined by a child’s response to medication.⁸ Independent of any daily controller medication use, all children should have a short acting bronchodilator on hand in case of an acute attack.^1,8

TABLE
Recommendations for treating mild persistent asthma

Evidence summary

Mild persistent asthma is defined as forced expiratory volume over 1 second (FEV₁) ≥80% predicted, with daytime symptoms more than twice per week but less than once daily, and nighttime symptoms more often than twice monthly.¹

Low-dose inhaled corticosteroids

Two large randomized trials support using low-dose inhaled corticosteroids in these children. The Childhood Asthma Management Program (CAMP) study, which included 1041 children, evaluated treatment with either budesonide or nedocromil vs placebo. Patients taking budesonide had a lower rate of urgent care visits (absolute risk reduction [ARR]=10%; number needed to treat [NNT]=10; P=.02) compared with children taking nedocromil (ARR=6%; NNT=17; P=.02). The urgent care visits were reported as number of visits per 100 person-years.

In practical terms, this means that in order to decrease 1 urgent care visit, 1 patient would need to take budesonide for 10 years. However, because rates are not necessarily homogenous over time, the number of visits decreased during the first year may be different than the number of events decreased throughout the tenth year.

Children taking budesonide experienced 21.5% more episode-free days than those taking placebo (P=.01). No change was observed in the nedocromil group.² In the inhaled Steroid Treatment As Regular Therapy (START) in early asthma study, budesonide demonstrated a 44% relative reduction in time to first severe asthma related event, compared with placebo (95% confidence interval [CI], 0.45–0.71; NNT=44; P=.0001).³

Theophylline

Theophylline is considered an alternative to inhaled corticosteroids. One study compared beclomethasone with theophylline in 195 children. This study found near-equivalent efficacy in doctor visits, hospitalizations, monthly peak expiratory flow rates, and FEV₁; however, beclomethasone was superior to theophylline in maintaining symptom control and decreasing the use of inhaled bronchodilators and systemic steroids.

When compared with beclomethasone, theophylline was linked to 14% more central nervous system adverse effects (P<.001) and 17% more gastrointestinal disturbances (P<.001). Although beclomethasone induced more oral candidiasis compared with theophylline (8.9% vs 2.4%; P<.001), the incidence of this infection can be reduced by using a spacer.

Long-term systemic effects

The potential long-term adverse systemic effects of inhaled corticosteroids on growth, bone metabolism, and pituitary-adrenal function call for longer-term studies.⁴ A systematic review of 15 trials reported that the protective effect of leukotriene receptor antagonists is inferior to inhaled corticosteroids for adults (relative risk [RR]=1.71; 95% CI, 1.40–2.09); however, evidence is insufficient to extrapolate this to children.⁵

Beta-agonists

Evidence does not support use of long-acting beta-agonists as monotherapy or in combination with other medications for children with mild persistent asthma. Although 1 study showed an improvement in lung function for children taking budesonide plus formoterol compared with budesonide alone, the rate of severe exacerbations was lower for those taking budesonide alone (62% decrease vs 55.8% decrease; P=.001). Both groups had a 32% decrease in the number of rescue inhalations per day when compared with placebo (P=.0008).⁶

Recommendations from others

Recommendations are listed in the TABLE.^1,7,8 Unlike the NAEPP and GINA asthma guidelines, the BTS/SIGN asthma guidelines define no objective measurement or staging classification to diagnose asthma among children. Diagnosis is determined by a child’s response to medication.⁸ Independent of any daily controller medication use, all children should have a short acting bronchodilator on hand in case of an acute attack.^1,8

TABLE
Recommendations for treating mild persistent asthma

Q I performed a McCall culdoplasty following vaginal hysterectomy, but the insurance company denied payment for the culdoplasty, stating that this procedure is included in the hysterectomy. How do I appeal?

ADenial could take place only if the incorrect code combination was billed. For example, if your billing staff itemized the procedures by reporting 58260 for the vaginal hysterectomy and 57268 [Repair of enterocele, vaginal approach (separate procedure)], then the enterocele repair (McCall) would be denied as inclusive, as these two codes are bundled. But they are bundled because there are 4 codes that combine enterocele repair with vaginal hysterectomy, depending on the documented weight of the uterus and whether you took, or left, the tubes and ovaries.

Your code choices are:

58263 Vaginal hysterectomy, for uterus 250 g or less; with removal of tube(s), and/or ovary(s), with repair of enterocele

58270 Vaginal hysterectomy, for uterus 250 g or less; with repair of enterocele

58292 Vaginal hysterectomy, for uterus greater than 250 g; with removal of tube(s) and/or ovary(s), with repair of enterocele

58294 Vaginal hysterectomy, for uterus greater than 250 g; with repair of enterocele

Don’t blame your billing staff if this is what occurred. The term “McCall culdoplasty” appears nowhere in the CPT book, so your billers would need to know that you actually performed an enterocele repair.

Correctly communicating what you did is an important step in getting the claim paid in a timely manner. Refile with the correct code!

Read a description of the technique of McCall culdoplasty.

Complete and transvaginal US scan must be specified

Q Regarding ultrasonography (US) codes 76856 and 76857, are these codes for an abdominal or a vaginal approach? Recently, we scanned a patient transvaginally for a complete US study (uterine, ovary, stripe, etc) but could not determine which code to use. My understanding has been that code 76830 is for a limited transvaginal scan.

ACodes 76856 [Ultrasound, pelvic (nonobstetric), B-scan and/or real time with image documentation; complete] and 76857 [Ultrasound…; limited or follow-up (eg, for follicles)] describe a transabdominal approach. If you performed a complete transvaginal scan, the code would be 76830, which is not a limited scan. In fact, the physician work relative value units assigned to these codes are identical, at .69. The only code for a limited gynecologic US would be 76857. If you performed a limited US by a vaginal approach, however, you can bill 76830 with a modifier -52 (reduced services) added to indicate that you did not perform a complete scan.

Q I performed a McCall culdoplasty following vaginal hysterectomy, but the insurance company denied payment for the culdoplasty, stating that this procedure is included in the hysterectomy. How do I appeal?

ADenial could take place only if the incorrect code combination was billed. For example, if your billing staff itemized the procedures by reporting 58260 for the vaginal hysterectomy and 57268 [Repair of enterocele, vaginal approach (separate procedure)], then the enterocele repair (McCall) would be denied as inclusive, as these two codes are bundled. But they are bundled because there are 4 codes that combine enterocele repair with vaginal hysterectomy, depending on the documented weight of the uterus and whether you took, or left, the tubes and ovaries.

Your code choices are:

58263 Vaginal hysterectomy, for uterus 250 g or less; with removal of tube(s), and/or ovary(s), with repair of enterocele

58270 Vaginal hysterectomy, for uterus 250 g or less; with repair of enterocele

58292 Vaginal hysterectomy, for uterus greater than 250 g; with removal of tube(s) and/or ovary(s), with repair of enterocele

58294 Vaginal hysterectomy, for uterus greater than 250 g; with repair of enterocele

Don’t blame your billing staff if this is what occurred. The term “McCall culdoplasty” appears nowhere in the CPT book, so your billers would need to know that you actually performed an enterocele repair.

Correctly communicating what you did is an important step in getting the claim paid in a timely manner. Refile with the correct code!

Read a description of the technique of McCall culdoplasty.

Complete and transvaginal US scan must be specified

Q Regarding ultrasonography (US) codes 76856 and 76857, are these codes for an abdominal or a vaginal approach? Recently, we scanned a patient transvaginally for a complete US study (uterine, ovary, stripe, etc) but could not determine which code to use. My understanding has been that code 76830 is for a limited transvaginal scan.

ACodes 76856 [Ultrasound, pelvic (nonobstetric), B-scan and/or real time with image documentation; complete] and 76857 [Ultrasound…; limited or follow-up (eg, for follicles)] describe a transabdominal approach. If you performed a complete transvaginal scan, the code would be 76830, which is not a limited scan. In fact, the physician work relative value units assigned to these codes are identical, at .69. The only code for a limited gynecologic US would be 76857. If you performed a limited US by a vaginal approach, however, you can bill 76830 with a modifier -52 (reduced services) added to indicate that you did not perform a complete scan.

Q I performed a McCall culdoplasty following vaginal hysterectomy, but the insurance company denied payment for the culdoplasty, stating that this procedure is included in the hysterectomy. How do I appeal?

ADenial could take place only if the incorrect code combination was billed. For example, if your billing staff itemized the procedures by reporting 58260 for the vaginal hysterectomy and 57268 [Repair of enterocele, vaginal approach (separate procedure)], then the enterocele repair (McCall) would be denied as inclusive, as these two codes are bundled. But they are bundled because there are 4 codes that combine enterocele repair with vaginal hysterectomy, depending on the documented weight of the uterus and whether you took, or left, the tubes and ovaries.

Your code choices are:

58263 Vaginal hysterectomy, for uterus 250 g or less; with removal of tube(s), and/or ovary(s), with repair of enterocele

58270 Vaginal hysterectomy, for uterus 250 g or less; with repair of enterocele

58292 Vaginal hysterectomy, for uterus greater than 250 g; with removal of tube(s) and/or ovary(s), with repair of enterocele

58294 Vaginal hysterectomy, for uterus greater than 250 g; with repair of enterocele

Don’t blame your billing staff if this is what occurred. The term “McCall culdoplasty” appears nowhere in the CPT book, so your billers would need to know that you actually performed an enterocele repair.

Correctly communicating what you did is an important step in getting the claim paid in a timely manner. Refile with the correct code!

Read a description of the technique of McCall culdoplasty.

Complete and transvaginal US scan must be specified

Q Regarding ultrasonography (US) codes 76856 and 76857, are these codes for an abdominal or a vaginal approach? Recently, we scanned a patient transvaginally for a complete US study (uterine, ovary, stripe, etc) but could not determine which code to use. My understanding has been that code 76830 is for a limited transvaginal scan.

ACodes 76856 [Ultrasound, pelvic (nonobstetric), B-scan and/or real time with image documentation; complete] and 76857 [Ultrasound…; limited or follow-up (eg, for follicles)] describe a transabdominal approach. If you performed a complete transvaginal scan, the code would be 76830, which is not a limited scan. In fact, the physician work relative value units assigned to these codes are identical, at .69. The only code for a limited gynecologic US would be 76857. If you performed a limited US by a vaginal approach, however, you can bill 76830 with a modifier -52 (reduced services) added to indicate that you did not perform a complete scan.

Psychotic patient declines hospital admission, drives into an office building

Cook County (IL) Circuit Court

The patient, age 43, had been treated for mental illness for many years. He was voluntarily admitted to a hospital under the care of his psychiatrist, and was discharged at his own request a few days later. He had improved and was not considered a candidate for involuntary admission because he was not a danger to himself or others.

The patient then informed the psychiatrist that he did not want to continue treatment and said he had an appointment with a new psychiatrist within 2 weeks.

Five days later, the patient went to another hospital for voluntary admission. He was seen by an emergency room physician, who determined the patient was a candidate for voluntary admission. The patient, however, decided to leave the hospital while a bed was being arranged.

Two days later, the patient began having auditory and visual hallucinations. He then drove his car through the glass doors of an office building. No one was injured, but the patient was arrested and convicted of felony damage to property.

In his suit, the patient alleged his longtime psychiatrist was negligent and failed to properly treat him to avoid development of hallucinations. The psychiatrist argued that involuntary admission was not indicated and that the care given was appropriate.

• A defense verdict was returned
  

Patient commits suicide after discharge

Cook County (IL) Circuit Court

A patient, age 45, committed suicide by taking lethal doses of medication prescribed by her psychiatrist. The patient had suffered from severe depression, personality disorder, and substance abuse. The day before her death, she went to a hospital emergency room, where she was assessed for suicide and released without the psychiatrist having been notified.

The patient’s family claimed that the psychiatrist was negligent because he did not adequately assess or monitor the patient’s clinical condition at sufficient intervals over the 3 months preceding her suicide. The family also alleged that the psychiatrist prescribed oxycodone inappropriately.

The psychiatrist argued that proper care was given and that the patient failed to provide a complete, accurate medical history at the emergency room visit and did not to consent to admission.

• A defense verdict was returned
  

Could admission have prevented patient’s suicide?

Douglas County (NE) District Court

A patient in his mid-60s with a history of depression committed suicide with a gunshot wound to the head. Before his suicide, the patient was seeing a psychiatrist and psychologist for depression and emotional problems.

The patient’s family alleged the psychiatrist failed to diagnose the severity of the patient’s problems and admit him to a hospital for treatment and observation. The psychiatrist and psychologist denied negligence.

• A defense verdict was returned
  

Medical malpractice law is constantly evolving to determine what constitutes “negligent care.” The legal standard requires a patient who brings a negligence claim against a psychiatrist to prove:

• a relationship between patient and psychiatrist such that a duty of care exists
  
• the duty was breached—meaning the standard of care was not met
  
• the breach of duty caused the injury.
  

Relationship rules

The first case highlights issues surrounding the patient-psychiatrist relationship. In general, once you have agreed to treat a patient, a doctor-patient relationship and duty of care exists.

In the first case, the patient informed his longtime psychiatrist that he no longer wanted to continue care after discharge. A psychiatrist who terminates a doctor-patient relationship should provide written notice, an explanation of termination, and referrals and continue to care for the patient for a reasonable period.¹ No such duty exists, however, when the patient ends treatment. Courts have found that the patient has not been abandoned when he or she voluntarily and unilaterally terminates the relationship.^2,3

The relationship ends the moment the patient terminates care, unless the patient is not competent to make that unilateral decision. In that situation, your duty of care to the patient continues.² When a competent patient terminates care, document the date and time of termination and the patient’s competence.

When relationships begin

The patient in the first case had an appointment with a new psychiatrist within 2 weeks. Is the new psychiatrist liable for what happens in the intervening period or does the relationship begin when the patient has been examined or treated? The legal question of when a physician-patient relationship is created remains problematic. Standards vary from state to state, but general principles offer some guidance.

The physician-patient relationship is a contract. The court would examine parties’ actions to ascertain their intent to determine if the patient reasonably believed that the physician—by actions or words—agreed to provide necessary medical care. Additionally, whether a relationship exists depends on the specific facts and circumstances of each situation.

There is some authority, across many jurisdictions, that a physician-patient relationship is established only when a physician conducts the initial history and physical examination. In some cases, however, the relationship has been found to exist at an earlier point, such as when a physician gave a referred patient an appointment for a consultation. When in doubt, assume the relationship exists.⁴

Duty of care

These cases raise areas where possible duty of care was breached:

• negligent prescription of medication
  
• failure to assess suicidal thinking.
  

Assessing suicide risk. Negligence in the second and third cases is based upon failure to assess suicidal thoughts. The legal system recognizes that psychiatrists cannot predict suicide,⁶ and mistakes in clinical judgment are not the same as negligence. Psychiatrists, however, are required to assess suicide risk and intervene appropriately.

When defending a negligence claim, the profession’s custom—reflected by the standard of care common to others with the practitioner’s training—is the benchmark against which the courts measure negligence. Therefore, take steps determined appropriate by the profession and document this risk assessment.⁷ For example, ask the patient about:

• suicidal thoughts and intent
  
• stressors
  
• history of suicidal behavior/attempts
  
• substance use
  
• signs and symptoms of depression
  
• bipolar disorder
  
• psychosis.⁸
  

Prescriptions. No clear line defines negligence when potentially dangerous medications are prescribed to a suicidal patient. Some psychiatrists dispense limited quantities of medications and see the patient weekly to monitor mood and medication. But even then a psychiatrist cannot prevent suicide—for example, the patient may have multiple prescribers or hoard medications. The concept of “sufficient intervals” to see a patient is determined case-by-case.

Documentation. Make suicide assessments an ongoing process. Document all aspects of the patient’s care, stability, and suicide risk, and reasons for the visit intervals. Indicate in the records your risk-benefit assessment in making treatment decisions.

Cases are selected byfrom Medical Malpractice Verdicts, Settlements & Experts, with permission of its editor, Lewis Laska of Nashville, TN (www.verdictslaska.com). Information may be incomplete in some instances, but these cases represent clinical situations that typically result in litigation.

Drug brand name

• Oxycodone • Percocet
  

Psychotic patient declines hospital admission, drives into an office building

Cook County (IL) Circuit Court

The patient, age 43, had been treated for mental illness for many years. He was voluntarily admitted to a hospital under the care of his psychiatrist, and was discharged at his own request a few days later. He had improved and was not considered a candidate for involuntary admission because he was not a danger to himself or others.

The patient then informed the psychiatrist that he did not want to continue treatment and said he had an appointment with a new psychiatrist within 2 weeks.

Five days later, the patient went to another hospital for voluntary admission. He was seen by an emergency room physician, who determined the patient was a candidate for voluntary admission. The patient, however, decided to leave the hospital while a bed was being arranged.

Two days later, the patient began having auditory and visual hallucinations. He then drove his car through the glass doors of an office building. No one was injured, but the patient was arrested and convicted of felony damage to property.

In his suit, the patient alleged his longtime psychiatrist was negligent and failed to properly treat him to avoid development of hallucinations. The psychiatrist argued that involuntary admission was not indicated and that the care given was appropriate.

• A defense verdict was returned
  

Patient commits suicide after discharge

Cook County (IL) Circuit Court

A patient, age 45, committed suicide by taking lethal doses of medication prescribed by her psychiatrist. The patient had suffered from severe depression, personality disorder, and substance abuse. The day before her death, she went to a hospital emergency room, where she was assessed for suicide and released without the psychiatrist having been notified.

The patient’s family claimed that the psychiatrist was negligent because he did not adequately assess or monitor the patient’s clinical condition at sufficient intervals over the 3 months preceding her suicide. The family also alleged that the psychiatrist prescribed oxycodone inappropriately.

The psychiatrist argued that proper care was given and that the patient failed to provide a complete, accurate medical history at the emergency room visit and did not to consent to admission.

• A defense verdict was returned
  

Could admission have prevented patient’s suicide?

Douglas County (NE) District Court

A patient in his mid-60s with a history of depression committed suicide with a gunshot wound to the head. Before his suicide, the patient was seeing a psychiatrist and psychologist for depression and emotional problems.

The patient’s family alleged the psychiatrist failed to diagnose the severity of the patient’s problems and admit him to a hospital for treatment and observation. The psychiatrist and psychologist denied negligence.

• A defense verdict was returned
  

Medical malpractice law is constantly evolving to determine what constitutes “negligent care.” The legal standard requires a patient who brings a negligence claim against a psychiatrist to prove:

• a relationship between patient and psychiatrist such that a duty of care exists
  
• the duty was breached—meaning the standard of care was not met
  
• the breach of duty caused the injury.
  

Relationship rules

The first case highlights issues surrounding the patient-psychiatrist relationship. In general, once you have agreed to treat a patient, a doctor-patient relationship and duty of care exists.

In the first case, the patient informed his longtime psychiatrist that he no longer wanted to continue care after discharge. A psychiatrist who terminates a doctor-patient relationship should provide written notice, an explanation of termination, and referrals and continue to care for the patient for a reasonable period.¹ No such duty exists, however, when the patient ends treatment. Courts have found that the patient has not been abandoned when he or she voluntarily and unilaterally terminates the relationship.^2,3

The relationship ends the moment the patient terminates care, unless the patient is not competent to make that unilateral decision. In that situation, your duty of care to the patient continues.² When a competent patient terminates care, document the date and time of termination and the patient’s competence.

When relationships begin

The patient in the first case had an appointment with a new psychiatrist within 2 weeks. Is the new psychiatrist liable for what happens in the intervening period or does the relationship begin when the patient has been examined or treated? The legal question of when a physician-patient relationship is created remains problematic. Standards vary from state to state, but general principles offer some guidance.

The physician-patient relationship is a contract. The court would examine parties’ actions to ascertain their intent to determine if the patient reasonably believed that the physician—by actions or words—agreed to provide necessary medical care. Additionally, whether a relationship exists depends on the specific facts and circumstances of each situation.

There is some authority, across many jurisdictions, that a physician-patient relationship is established only when a physician conducts the initial history and physical examination. In some cases, however, the relationship has been found to exist at an earlier point, such as when a physician gave a referred patient an appointment for a consultation. When in doubt, assume the relationship exists.⁴

Duty of care

These cases raise areas where possible duty of care was breached:

• negligent prescription of medication
  
• failure to assess suicidal thinking.
  

Assessing suicide risk. Negligence in the second and third cases is based upon failure to assess suicidal thoughts. The legal system recognizes that psychiatrists cannot predict suicide,⁶ and mistakes in clinical judgment are not the same as negligence. Psychiatrists, however, are required to assess suicide risk and intervene appropriately.

When defending a negligence claim, the profession’s custom—reflected by the standard of care common to others with the practitioner’s training—is the benchmark against which the courts measure negligence. Therefore, take steps determined appropriate by the profession and document this risk assessment.⁷ For example, ask the patient about:

• suicidal thoughts and intent
  
• stressors
  
• history of suicidal behavior/attempts
  
• substance use
  
• signs and symptoms of depression
  
• bipolar disorder
  
• psychosis.⁸
  

Prescriptions. No clear line defines negligence when potentially dangerous medications are prescribed to a suicidal patient. Some psychiatrists dispense limited quantities of medications and see the patient weekly to monitor mood and medication. But even then a psychiatrist cannot prevent suicide—for example, the patient may have multiple prescribers or hoard medications. The concept of “sufficient intervals” to see a patient is determined case-by-case.

Documentation. Make suicide assessments an ongoing process. Document all aspects of the patient’s care, stability, and suicide risk, and reasons for the visit intervals. Indicate in the records your risk-benefit assessment in making treatment decisions.

Cases are selected byfrom Medical Malpractice Verdicts, Settlements & Experts, with permission of its editor, Lewis Laska of Nashville, TN (www.verdictslaska.com). Information may be incomplete in some instances, but these cases represent clinical situations that typically result in litigation.

Drug brand name

• Oxycodone • Percocet
  

Psychotic patient declines hospital admission, drives into an office building

Cook County (IL) Circuit Court

The patient, age 43, had been treated for mental illness for many years. He was voluntarily admitted to a hospital under the care of his psychiatrist, and was discharged at his own request a few days later. He had improved and was not considered a candidate for involuntary admission because he was not a danger to himself or others.

The patient then informed the psychiatrist that he did not want to continue treatment and said he had an appointment with a new psychiatrist within 2 weeks.

Five days later, the patient went to another hospital for voluntary admission. He was seen by an emergency room physician, who determined the patient was a candidate for voluntary admission. The patient, however, decided to leave the hospital while a bed was being arranged.

Two days later, the patient began having auditory and visual hallucinations. He then drove his car through the glass doors of an office building. No one was injured, but the patient was arrested and convicted of felony damage to property.

In his suit, the patient alleged his longtime psychiatrist was negligent and failed to properly treat him to avoid development of hallucinations. The psychiatrist argued that involuntary admission was not indicated and that the care given was appropriate.

• A defense verdict was returned
  

Patient commits suicide after discharge

Cook County (IL) Circuit Court

A patient, age 45, committed suicide by taking lethal doses of medication prescribed by her psychiatrist. The patient had suffered from severe depression, personality disorder, and substance abuse. The day before her death, she went to a hospital emergency room, where she was assessed for suicide and released without the psychiatrist having been notified.

The patient’s family claimed that the psychiatrist was negligent because he did not adequately assess or monitor the patient’s clinical condition at sufficient intervals over the 3 months preceding her suicide. The family also alleged that the psychiatrist prescribed oxycodone inappropriately.

The psychiatrist argued that proper care was given and that the patient failed to provide a complete, accurate medical history at the emergency room visit and did not to consent to admission.

• A defense verdict was returned
  

Could admission have prevented patient’s suicide?

Douglas County (NE) District Court

A patient in his mid-60s with a history of depression committed suicide with a gunshot wound to the head. Before his suicide, the patient was seeing a psychiatrist and psychologist for depression and emotional problems.

The patient’s family alleged the psychiatrist failed to diagnose the severity of the patient’s problems and admit him to a hospital for treatment and observation. The psychiatrist and psychologist denied negligence.

• A defense verdict was returned
  

Medical malpractice law is constantly evolving to determine what constitutes “negligent care.” The legal standard requires a patient who brings a negligence claim against a psychiatrist to prove:

• a relationship between patient and psychiatrist such that a duty of care exists
  
• the duty was breached—meaning the standard of care was not met
  
• the breach of duty caused the injury.
  

Relationship rules

The first case highlights issues surrounding the patient-psychiatrist relationship. In general, once you have agreed to treat a patient, a doctor-patient relationship and duty of care exists.

In the first case, the patient informed his longtime psychiatrist that he no longer wanted to continue care after discharge. A psychiatrist who terminates a doctor-patient relationship should provide written notice, an explanation of termination, and referrals and continue to care for the patient for a reasonable period.¹ No such duty exists, however, when the patient ends treatment. Courts have found that the patient has not been abandoned when he or she voluntarily and unilaterally terminates the relationship.^2,3

The relationship ends the moment the patient terminates care, unless the patient is not competent to make that unilateral decision. In that situation, your duty of care to the patient continues.² When a competent patient terminates care, document the date and time of termination and the patient’s competence.

When relationships begin

The patient in the first case had an appointment with a new psychiatrist within 2 weeks. Is the new psychiatrist liable for what happens in the intervening period or does the relationship begin when the patient has been examined or treated? The legal question of when a physician-patient relationship is created remains problematic. Standards vary from state to state, but general principles offer some guidance.

The physician-patient relationship is a contract. The court would examine parties’ actions to ascertain their intent to determine if the patient reasonably believed that the physician—by actions or words—agreed to provide necessary medical care. Additionally, whether a relationship exists depends on the specific facts and circumstances of each situation.

There is some authority, across many jurisdictions, that a physician-patient relationship is established only when a physician conducts the initial history and physical examination. In some cases, however, the relationship has been found to exist at an earlier point, such as when a physician gave a referred patient an appointment for a consultation. When in doubt, assume the relationship exists.⁴

Duty of care

These cases raise areas where possible duty of care was breached:

• negligent prescription of medication
  
• failure to assess suicidal thinking.
  

Assessing suicide risk. Negligence in the second and third cases is based upon failure to assess suicidal thoughts. The legal system recognizes that psychiatrists cannot predict suicide,⁶ and mistakes in clinical judgment are not the same as negligence. Psychiatrists, however, are required to assess suicide risk and intervene appropriately.

When defending a negligence claim, the profession’s custom—reflected by the standard of care common to others with the practitioner’s training—is the benchmark against which the courts measure negligence. Therefore, take steps determined appropriate by the profession and document this risk assessment.⁷ For example, ask the patient about:

• suicidal thoughts and intent
  
• stressors
  
• history of suicidal behavior/attempts
  
• substance use
  
• signs and symptoms of depression
  
• bipolar disorder
  
• psychosis.⁸
  

Prescriptions. No clear line defines negligence when potentially dangerous medications are prescribed to a suicidal patient. Some psychiatrists dispense limited quantities of medications and see the patient weekly to monitor mood and medication. But even then a psychiatrist cannot prevent suicide—for example, the patient may have multiple prescribers or hoard medications. The concept of “sufficient intervals” to see a patient is determined case-by-case.

Documentation. Make suicide assessments an ongoing process. Document all aspects of the patient’s care, stability, and suicide risk, and reasons for the visit intervals. Indicate in the records your risk-benefit assessment in making treatment decisions.

Cases are selected byfrom Medical Malpractice Verdicts, Settlements & Experts, with permission of its editor, Lewis Laska of Nashville, TN (www.verdictslaska.com). Information may be incomplete in some instances, but these cases represent clinical situations that typically result in litigation.

Drug brand name

• Oxycodone • Percocet
  

The method for skin grafting surgical defects of the nasal alar region known as the drumhead graft was invented and developed by Dr. J. Michael Wentzell of Billings, Mont. ('Drumhead' Technique May Spare Alar Graft Depressions, SKIN & ALLERGY NEWS, January 2007, p. 32). Dr. Bradley K. Draper of Billings presented Dr. Wentzell's technique at the annual meeting of the American Society for Dermatologic Surgery.

The method for skin grafting surgical defects of the nasal alar region known as the drumhead graft was invented and developed by Dr. J. Michael Wentzell of Billings, Mont. ('Drumhead' Technique May Spare Alar Graft Depressions, SKIN & ALLERGY NEWS, January 2007, p. 32). Dr. Bradley K. Draper of Billings presented Dr. Wentzell's technique at the annual meeting of the American Society for Dermatologic Surgery.

The method for skin grafting surgical defects of the nasal alar region known as the drumhead graft was invented and developed by Dr. J. Michael Wentzell of Billings, Mont. ('Drumhead' Technique May Spare Alar Graft Depressions, SKIN & ALLERGY NEWS, January 2007, p. 32). Dr. Bradley K. Draper of Billings presented Dr. Wentzell's technique at the annual meeting of the American Society for Dermatologic Surgery.