Recurrence of Linear Basal Cell Carcinoma

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Recurrence of Linear Basal Cell Carcinoma
Author(s)
Jordan Maxwell Ward, MD
Mark Russell, MD

Case Report

A 63-year-old man was evaluated in the Mohs clinic for a lesion on the right supraclavicular neck, which he described as a linear asymptomatic “birthmark” that had been present since childhood and stable for many years. It began to enlarge approximately 5 years prior, became increasingly red, and had occasional crusting. The lesion also gradually became more irritated with repeated mild trauma when he carried a backpack while hiking. On physical examination, a 10×2-cm, linear, pink plaque with an irregular border, translucent rolled edges, and central smooth atrophic skin was seen on the right supraclavicular neck (Figure). There was no visible epidermal nevus or nevus sebaceous in the area. A shave biopsy of the lesion confirmed the pathologic diagnosis of basal cell carcinoma, nodular type, along with the morphologic diagnosis of linear basal cell carcinoma (LBCC). The tumor was completely removed with standard excision using 5-mm margins.

Linear pink plaque on the right supraclavicular neck.

Approximately 10 months after the original excision, the patient developed an irritated erosion that occasionally bled when his backpack rubbed against it. He returned to the clinic after the erosion failed to heal. Physical examination revealed a 1.4×0.7-cm, eroded, pink papule with large telangiectases at the superior pole of the excision scar. A shave biopsy confirmed the diagnosis of a recurrent infiltrative basal cell carcinoma. The tumor was then completely excised using Mohs micrographic surgery.

Comment

Linear basal cell carcinoma, first described by Lewis1 in 1985, is a rare morphologic variant of basal cell carcinoma. In 2011, Al-Niaimi and Lyon2 performed a comprehensive literature search on LBCC (1985-2008) and found only 39 cases (including 2 of their own) had been published since the pioneer case in 1985. It was determined that the most common sites affected were the periorbital area and neck (n=13 each [67%]), and the majority were histologically nodular (n=27 [69%]). Mohs micrographic surgery was the most common treatment method (n=23 [59%]), followed by primary excision (n=17 [44%]). A history of trauma, radiotherapy, or prior operation in association with the site of the LBCC was discovered in only 7 cases (18%).2 Although Peschen et al3 proposed that trauma—both physical and surgical—and radiotherapy may play a role in the development of LBCCs, the low incidence reported suggests that other factors may be involved. To determine if genetic factors were contributing to the development of LBCCs, Yamaguchi et al4 investigated the expression of p27 and PCTAIRE1, both known to contribute to tumorigenesis when mutated, as well as somatic gene mutations using deep sequencing in a case of LBCC; they found no associated genetic mutation.

Reported Cases of LBCC
According to a PubMed search of articles indexed for MEDLINE using the terms linear and basal cell carcinoma, 67 cases (including the current case) of LBCC have been published since 1985. The patient demographics, anatomic location, histologic subtype, treatment methods, and frequency of recurrence for all reported cases of LBCC are summarized in the Table.1-24 There were 36 women and 31 men, with an average age of 70 years (range, 40–92 years). The most commonly affected sites were the periocular region (n=27) and neck (n=18). Histologically, most LBCCs were nodular (n=35), with the next most common histologic subtype being infiltrative (n=20), which included the morphoeic, metatypical, and micronodular subtypes under the overarching infiltrative subtype. The most frequently chosen treatment option was primary excision (n=38 [57%]), followed by Mohs micrographic surgery (n=28 [42%]). Risk factors previously identified by Al-Niaimi and Lyon,2 including trauma, radiotherapy, or prior operation, were reported in 12 of 67 cases. Recurrence was reported in only 2 of 67 cases, 1 being the current case; however, an accurate recurrence rate could not be calculated due to lack of follow-up or short length of follow-up in most of the reported cases.



Presentation and Treatment
Currently, there are no set criteria for the diagnosis of LBCC, but it has been shown to follow a characteristic morphologic pattern, favoring extension in one direction leading to a length-to-width ratio that typically is at least 3 to 1.5 With most lesions presenting in the periocular region along relaxed skin tension lines, it has been speculated that these tumors expand along wrinkles.2 Pierard and Lapiere25 proposed that the preferential parallel orientation and a straightening of thin collagen bundles and elastic fibers within the reticular dermis combined with relaxed skin tension lines and muscle contraction perpendicular to these stromal parts may influence the growth of tumors preferentially in one direction, contributing to linearity of the lesion. In addition, the clinical appearance is not a reliable indicator of subclinical extension.2 Therefore, Lim et al6 recommended Mohs micrographic surgery as the best initial treatment of LBCCs.

Conclusion

Linear basal cell carcinoma should be considered a distinct morphologic variant of basal cell carcinoma. Although likely underreported, this variant is uncommon. It presents most often in the periocular and neck regions. The most common histologic subtypes are nodular and infiltrative. Because of the likelihood of subclinical spread, LBCC should be regarded as a high-risk subtype. As such, Mohs micrographic surgery or excision with complete circumferential peripheral and deep margin assessment is recommended as first-line treatment of LBCC.6

References
  1. Lewis JE. Linear basal cell epithelioma. Int J Dermatol. 1985;24:124-125.
  2. Al-Niaimi F, Lyon CC. Linear basal cell carcinoma: a distinct condition? Clin Exp Dermatol. 2011;36:231-234.
  3. Peschen M, Lo JS, Snow SN, et al. Linear basal cell carcinoma. Cutis. 1993;51:287-289.
  4. Yamaguchi Y, Yanagi T, Imafuku K, et al. A case of linear basal cell carcinoma: evaluation of proliferative activity by immunohistochemical staining of PCTAIRE1 and p27. J Eur Acad Dermatol Venereol. 2017;31:E359-E362.
  5. Mavirakis I, Malhotra R, Selva D, et al. Linear basal cell carcinoma: a distinct clinical entity. J Plast Reconstr Aesthet Surg. 2006;59:419-423.
  6. Lim KK, Randle HW, Roenigk RK, et al. Linear basal cell carcinoma: report of seventeen cases and review of the presentation and treatment. Dermatol Surg. 1999;25:63-67.
  7. Pardavila R, Rosón E, De la torre C, et al. Linear basal cell carcinoma. report of two cases [in Spanish]. Actas Dermosifiliogr. 2007;98:291.
  8. Shinsuke K, Hirohiko K, Yasuhiro T, et al. Linear basal cell carcinoma in an Asian patient. Open Ophthalmol J. 2007;1:20-22.
  9. Ning C, Chao S. Linear basal cell carcinoma of the scrotum. Dermatol Sinica. 2002;20:57-62.
  10. Chopra KF, Cohen PR. Linear basal cell carcinomas: report of multiple sequential tumors localized to a radiotherapy port and review of the literature. Tex Med. 1997;93:57-59.
  11. da Silva MO, Dadalt P, Santos OL, et al. Linear basal cell carcinoma. Int J Dermatol. 1995;34:488.
  12. Warthan TL, Lewis JE. Giant linear basal cell epithelioma. Int J Dermatol. 1994;33:284.
  13. Lewis JE. Linear basal cell epithelioma. Int J Dermatol. 1989;28:682-684.
  14. Alcántara-Reifs CM, Salido-Vallejo R, González-Menchen A, et al. Linear basal cell carcinoma: report of three cases with dermoscopic findings. Indian J Dermatol Venereol Leprol. 2016;82:708-711.
  15. Lee MS, Cho E, Lee JH, et al. Linearly curved, blackish macule on the wrist. Cutis. 2016;97:384, 406-407.
  16. Bajaj S, Sharma PK, Kar HK. Linear adamantinoid basal cell carcinoma in the axilla. Dermatol Online J. 2015;21. pii:13030/qt8k0713nb.
  17. Iga N, Sakurai K, Fujii H, et al. Linear basal cell carcinoma at the external genitalia. J Dermatol. 2014;41:275-276.
  18. Ichinokawa Y, Ohtuki A, Hattori M, et al. Linear basal cell carcinoma: a case report. Case Rep Dermatol. 2011;3:142-146.
  19. Becher GL, Affleck A, Fleming C, et al. Linear basal cell carcinoma occurs most commonly on the lower eyelid. Clin Exp Dermatol. 2011;36:311-312.
  20. Jellouli A, Triki S, Zghal M, et al. Linear basal cell carcinoma. Actas Dermosifiliogr. 2010;101:648-650.
  21. Takiyoshi N, Nakano H, Kaneko T, et al. A linear basal cell carcinoma undergoing spontaneous regression. Clin Exp Dermatol. 2009;34:E411-E413.
  22. Yoleri L, Ozden S, Kandiloglu A. A 46-year-old male with an ulcerated linear lesion on his neck. Ann Saudi Med. 2008;28:57-58.
  23. Palleschi GM, Corradini D, Bruscino N, et al. Linear basal cell carcinoma: clinical significance and better surgical approach. G Ital Dermatol Venereol. 2016;151:119-121.
  24. Rodriguez-Garijo N, Redondo P. Linear basal cell carcinoma of the lower eyelid: reconstruction with a musculocutaneous transposition flap. JAAD Case Rep. 2018;4:633-635.
  25. Pierard GE, Lapiere CM. Microanatomy of the dermis in relation to relaxed skin tension lines and Langer’s lines. Am J Dermatopathol. 1987;9:219-224.
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Author and Disclosure Information

Dr. Ward is from the Department of Medicine, Augusta University, Georgia. Dr. Russell is from the Department of Dermatology, University of Virginia Health System, Charlottesville.

The authors report no conflict of interest.

Correspondence: Jordan Maxwell Ward, MD, 1120 15th St, Augusta, GA 30912 ([email protected]).

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Case Reports
Author(s)
Jordan Maxwell Ward, MD
Mark Russell, MD
Author(s)
Jordan Maxwell Ward, MD
Mark Russell, MD
Author and Disclosure Information

Dr. Ward is from the Department of Medicine, Augusta University, Georgia. Dr. Russell is from the Department of Dermatology, University of Virginia Health System, Charlottesville.

The authors report no conflict of interest.

Correspondence: Jordan Maxwell Ward, MD, 1120 15th St, Augusta, GA 30912 ([email protected]).

Author and Disclosure Information

Dr. Ward is from the Department of Medicine, Augusta University, Georgia. Dr. Russell is from the Department of Dermatology, University of Virginia Health System, Charlottesville.

The authors report no conflict of interest.

Correspondence: Jordan Maxwell Ward, MD, 1120 15th St, Augusta, GA 30912 ([email protected]).

Article PDF
Ward CT104002114.PDF
Article PDF
Ward CT104002114.PDF

Case Report

A 63-year-old man was evaluated in the Mohs clinic for a lesion on the right supraclavicular neck, which he described as a linear asymptomatic “birthmark” that had been present since childhood and stable for many years. It began to enlarge approximately 5 years prior, became increasingly red, and had occasional crusting. The lesion also gradually became more irritated with repeated mild trauma when he carried a backpack while hiking. On physical examination, a 10×2-cm, linear, pink plaque with an irregular border, translucent rolled edges, and central smooth atrophic skin was seen on the right supraclavicular neck (Figure). There was no visible epidermal nevus or nevus sebaceous in the area. A shave biopsy of the lesion confirmed the pathologic diagnosis of basal cell carcinoma, nodular type, along with the morphologic diagnosis of linear basal cell carcinoma (LBCC). The tumor was completely removed with standard excision using 5-mm margins.

Linear pink plaque on the right supraclavicular neck.

Approximately 10 months after the original excision, the patient developed an irritated erosion that occasionally bled when his backpack rubbed against it. He returned to the clinic after the erosion failed to heal. Physical examination revealed a 1.4×0.7-cm, eroded, pink papule with large telangiectases at the superior pole of the excision scar. A shave biopsy confirmed the diagnosis of a recurrent infiltrative basal cell carcinoma. The tumor was then completely excised using Mohs micrographic surgery.

Comment

Linear basal cell carcinoma, first described by Lewis1 in 1985, is a rare morphologic variant of basal cell carcinoma. In 2011, Al-Niaimi and Lyon2 performed a comprehensive literature search on LBCC (1985-2008) and found only 39 cases (including 2 of their own) had been published since the pioneer case in 1985. It was determined that the most common sites affected were the periorbital area and neck (n=13 each [67%]), and the majority were histologically nodular (n=27 [69%]). Mohs micrographic surgery was the most common treatment method (n=23 [59%]), followed by primary excision (n=17 [44%]). A history of trauma, radiotherapy, or prior operation in association with the site of the LBCC was discovered in only 7 cases (18%).2 Although Peschen et al3 proposed that trauma—both physical and surgical—and radiotherapy may play a role in the development of LBCCs, the low incidence reported suggests that other factors may be involved. To determine if genetic factors were contributing to the development of LBCCs, Yamaguchi et al4 investigated the expression of p27 and PCTAIRE1, both known to contribute to tumorigenesis when mutated, as well as somatic gene mutations using deep sequencing in a case of LBCC; they found no associated genetic mutation.

Reported Cases of LBCC
According to a PubMed search of articles indexed for MEDLINE using the terms linear and basal cell carcinoma, 67 cases (including the current case) of LBCC have been published since 1985. The patient demographics, anatomic location, histologic subtype, treatment methods, and frequency of recurrence for all reported cases of LBCC are summarized in the Table.1-24 There were 36 women and 31 men, with an average age of 70 years (range, 40–92 years). The most commonly affected sites were the periocular region (n=27) and neck (n=18). Histologically, most LBCCs were nodular (n=35), with the next most common histologic subtype being infiltrative (n=20), which included the morphoeic, metatypical, and micronodular subtypes under the overarching infiltrative subtype. The most frequently chosen treatment option was primary excision (n=38 [57%]), followed by Mohs micrographic surgery (n=28 [42%]). Risk factors previously identified by Al-Niaimi and Lyon,2 including trauma, radiotherapy, or prior operation, were reported in 12 of 67 cases. Recurrence was reported in only 2 of 67 cases, 1 being the current case; however, an accurate recurrence rate could not be calculated due to lack of follow-up or short length of follow-up in most of the reported cases.



Presentation and Treatment
Currently, there are no set criteria for the diagnosis of LBCC, but it has been shown to follow a characteristic morphologic pattern, favoring extension in one direction leading to a length-to-width ratio that typically is at least 3 to 1.5 With most lesions presenting in the periocular region along relaxed skin tension lines, it has been speculated that these tumors expand along wrinkles.2 Pierard and Lapiere25 proposed that the preferential parallel orientation and a straightening of thin collagen bundles and elastic fibers within the reticular dermis combined with relaxed skin tension lines and muscle contraction perpendicular to these stromal parts may influence the growth of tumors preferentially in one direction, contributing to linearity of the lesion. In addition, the clinical appearance is not a reliable indicator of subclinical extension.2 Therefore, Lim et al6 recommended Mohs micrographic surgery as the best initial treatment of LBCCs.

Conclusion

Linear basal cell carcinoma should be considered a distinct morphologic variant of basal cell carcinoma. Although likely underreported, this variant is uncommon. It presents most often in the periocular and neck regions. The most common histologic subtypes are nodular and infiltrative. Because of the likelihood of subclinical spread, LBCC should be regarded as a high-risk subtype. As such, Mohs micrographic surgery or excision with complete circumferential peripheral and deep margin assessment is recommended as first-line treatment of LBCC.6

Case Report

A 63-year-old man was evaluated in the Mohs clinic for a lesion on the right supraclavicular neck, which he described as a linear asymptomatic “birthmark” that had been present since childhood and stable for many years. It began to enlarge approximately 5 years prior, became increasingly red, and had occasional crusting. The lesion also gradually became more irritated with repeated mild trauma when he carried a backpack while hiking. On physical examination, a 10×2-cm, linear, pink plaque with an irregular border, translucent rolled edges, and central smooth atrophic skin was seen on the right supraclavicular neck (Figure). There was no visible epidermal nevus or nevus sebaceous in the area. A shave biopsy of the lesion confirmed the pathologic diagnosis of basal cell carcinoma, nodular type, along with the morphologic diagnosis of linear basal cell carcinoma (LBCC). The tumor was completely removed with standard excision using 5-mm margins.

Linear pink plaque on the right supraclavicular neck.

Approximately 10 months after the original excision, the patient developed an irritated erosion that occasionally bled when his backpack rubbed against it. He returned to the clinic after the erosion failed to heal. Physical examination revealed a 1.4×0.7-cm, eroded, pink papule with large telangiectases at the superior pole of the excision scar. A shave biopsy confirmed the diagnosis of a recurrent infiltrative basal cell carcinoma. The tumor was then completely excised using Mohs micrographic surgery.

Comment

Linear basal cell carcinoma, first described by Lewis1 in 1985, is a rare morphologic variant of basal cell carcinoma. In 2011, Al-Niaimi and Lyon2 performed a comprehensive literature search on LBCC (1985-2008) and found only 39 cases (including 2 of their own) had been published since the pioneer case in 1985. It was determined that the most common sites affected were the periorbital area and neck (n=13 each [67%]), and the majority were histologically nodular (n=27 [69%]). Mohs micrographic surgery was the most common treatment method (n=23 [59%]), followed by primary excision (n=17 [44%]). A history of trauma, radiotherapy, or prior operation in association with the site of the LBCC was discovered in only 7 cases (18%).2 Although Peschen et al3 proposed that trauma—both physical and surgical—and radiotherapy may play a role in the development of LBCCs, the low incidence reported suggests that other factors may be involved. To determine if genetic factors were contributing to the development of LBCCs, Yamaguchi et al4 investigated the expression of p27 and PCTAIRE1, both known to contribute to tumorigenesis when mutated, as well as somatic gene mutations using deep sequencing in a case of LBCC; they found no associated genetic mutation.

Reported Cases of LBCC
According to a PubMed search of articles indexed for MEDLINE using the terms linear and basal cell carcinoma, 67 cases (including the current case) of LBCC have been published since 1985. The patient demographics, anatomic location, histologic subtype, treatment methods, and frequency of recurrence for all reported cases of LBCC are summarized in the Table.1-24 There were 36 women and 31 men, with an average age of 70 years (range, 40–92 years). The most commonly affected sites were the periocular region (n=27) and neck (n=18). Histologically, most LBCCs were nodular (n=35), with the next most common histologic subtype being infiltrative (n=20), which included the morphoeic, metatypical, and micronodular subtypes under the overarching infiltrative subtype. The most frequently chosen treatment option was primary excision (n=38 [57%]), followed by Mohs micrographic surgery (n=28 [42%]). Risk factors previously identified by Al-Niaimi and Lyon,2 including trauma, radiotherapy, or prior operation, were reported in 12 of 67 cases. Recurrence was reported in only 2 of 67 cases, 1 being the current case; however, an accurate recurrence rate could not be calculated due to lack of follow-up or short length of follow-up in most of the reported cases.



Presentation and Treatment
Currently, there are no set criteria for the diagnosis of LBCC, but it has been shown to follow a characteristic morphologic pattern, favoring extension in one direction leading to a length-to-width ratio that typically is at least 3 to 1.5 With most lesions presenting in the periocular region along relaxed skin tension lines, it has been speculated that these tumors expand along wrinkles.2 Pierard and Lapiere25 proposed that the preferential parallel orientation and a straightening of thin collagen bundles and elastic fibers within the reticular dermis combined with relaxed skin tension lines and muscle contraction perpendicular to these stromal parts may influence the growth of tumors preferentially in one direction, contributing to linearity of the lesion. In addition, the clinical appearance is not a reliable indicator of subclinical extension.2 Therefore, Lim et al6 recommended Mohs micrographic surgery as the best initial treatment of LBCCs.

Conclusion

Linear basal cell carcinoma should be considered a distinct morphologic variant of basal cell carcinoma. Although likely underreported, this variant is uncommon. It presents most often in the periocular and neck regions. The most common histologic subtypes are nodular and infiltrative. Because of the likelihood of subclinical spread, LBCC should be regarded as a high-risk subtype. As such, Mohs micrographic surgery or excision with complete circumferential peripheral and deep margin assessment is recommended as first-line treatment of LBCC.6

References
  1. Lewis JE. Linear basal cell epithelioma. Int J Dermatol. 1985;24:124-125.
  2. Al-Niaimi F, Lyon CC. Linear basal cell carcinoma: a distinct condition? Clin Exp Dermatol. 2011;36:231-234.
  3. Peschen M, Lo JS, Snow SN, et al. Linear basal cell carcinoma. Cutis. 1993;51:287-289.
  4. Yamaguchi Y, Yanagi T, Imafuku K, et al. A case of linear basal cell carcinoma: evaluation of proliferative activity by immunohistochemical staining of PCTAIRE1 and p27. J Eur Acad Dermatol Venereol. 2017;31:E359-E362.
  5. Mavirakis I, Malhotra R, Selva D, et al. Linear basal cell carcinoma: a distinct clinical entity. J Plast Reconstr Aesthet Surg. 2006;59:419-423.
  6. Lim KK, Randle HW, Roenigk RK, et al. Linear basal cell carcinoma: report of seventeen cases and review of the presentation and treatment. Dermatol Surg. 1999;25:63-67.
  7. Pardavila R, Rosón E, De la torre C, et al. Linear basal cell carcinoma. report of two cases [in Spanish]. Actas Dermosifiliogr. 2007;98:291.
  8. Shinsuke K, Hirohiko K, Yasuhiro T, et al. Linear basal cell carcinoma in an Asian patient. Open Ophthalmol J. 2007;1:20-22.
  9. Ning C, Chao S. Linear basal cell carcinoma of the scrotum. Dermatol Sinica. 2002;20:57-62.
  10. Chopra KF, Cohen PR. Linear basal cell carcinomas: report of multiple sequential tumors localized to a radiotherapy port and review of the literature. Tex Med. 1997;93:57-59.
  11. da Silva MO, Dadalt P, Santos OL, et al. Linear basal cell carcinoma. Int J Dermatol. 1995;34:488.
  12. Warthan TL, Lewis JE. Giant linear basal cell epithelioma. Int J Dermatol. 1994;33:284.
  13. Lewis JE. Linear basal cell epithelioma. Int J Dermatol. 1989;28:682-684.
  14. Alcántara-Reifs CM, Salido-Vallejo R, González-Menchen A, et al. Linear basal cell carcinoma: report of three cases with dermoscopic findings. Indian J Dermatol Venereol Leprol. 2016;82:708-711.
  15. Lee MS, Cho E, Lee JH, et al. Linearly curved, blackish macule on the wrist. Cutis. 2016;97:384, 406-407.
  16. Bajaj S, Sharma PK, Kar HK. Linear adamantinoid basal cell carcinoma in the axilla. Dermatol Online J. 2015;21. pii:13030/qt8k0713nb.
  17. Iga N, Sakurai K, Fujii H, et al. Linear basal cell carcinoma at the external genitalia. J Dermatol. 2014;41:275-276.
  18. Ichinokawa Y, Ohtuki A, Hattori M, et al. Linear basal cell carcinoma: a case report. Case Rep Dermatol. 2011;3:142-146.
  19. Becher GL, Affleck A, Fleming C, et al. Linear basal cell carcinoma occurs most commonly on the lower eyelid. Clin Exp Dermatol. 2011;36:311-312.
  20. Jellouli A, Triki S, Zghal M, et al. Linear basal cell carcinoma. Actas Dermosifiliogr. 2010;101:648-650.
  21. Takiyoshi N, Nakano H, Kaneko T, et al. A linear basal cell carcinoma undergoing spontaneous regression. Clin Exp Dermatol. 2009;34:E411-E413.
  22. Yoleri L, Ozden S, Kandiloglu A. A 46-year-old male with an ulcerated linear lesion on his neck. Ann Saudi Med. 2008;28:57-58.
  23. Palleschi GM, Corradini D, Bruscino N, et al. Linear basal cell carcinoma: clinical significance and better surgical approach. G Ital Dermatol Venereol. 2016;151:119-121.
  24. Rodriguez-Garijo N, Redondo P. Linear basal cell carcinoma of the lower eyelid: reconstruction with a musculocutaneous transposition flap. JAAD Case Rep. 2018;4:633-635.
  25. Pierard GE, Lapiere CM. Microanatomy of the dermis in relation to relaxed skin tension lines and Langer’s lines. Am J Dermatopathol. 1987;9:219-224.
References
  1. Lewis JE. Linear basal cell epithelioma. Int J Dermatol. 1985;24:124-125.
  2. Al-Niaimi F, Lyon CC. Linear basal cell carcinoma: a distinct condition? Clin Exp Dermatol. 2011;36:231-234.
  3. Peschen M, Lo JS, Snow SN, et al. Linear basal cell carcinoma. Cutis. 1993;51:287-289.
  4. Yamaguchi Y, Yanagi T, Imafuku K, et al. A case of linear basal cell carcinoma: evaluation of proliferative activity by immunohistochemical staining of PCTAIRE1 and p27. J Eur Acad Dermatol Venereol. 2017;31:E359-E362.
  5. Mavirakis I, Malhotra R, Selva D, et al. Linear basal cell carcinoma: a distinct clinical entity. J Plast Reconstr Aesthet Surg. 2006;59:419-423.
  6. Lim KK, Randle HW, Roenigk RK, et al. Linear basal cell carcinoma: report of seventeen cases and review of the presentation and treatment. Dermatol Surg. 1999;25:63-67.
  7. Pardavila R, Rosón E, De la torre C, et al. Linear basal cell carcinoma. report of two cases [in Spanish]. Actas Dermosifiliogr. 2007;98:291.
  8. Shinsuke K, Hirohiko K, Yasuhiro T, et al. Linear basal cell carcinoma in an Asian patient. Open Ophthalmol J. 2007;1:20-22.
  9. Ning C, Chao S. Linear basal cell carcinoma of the scrotum. Dermatol Sinica. 2002;20:57-62.
  10. Chopra KF, Cohen PR. Linear basal cell carcinomas: report of multiple sequential tumors localized to a radiotherapy port and review of the literature. Tex Med. 1997;93:57-59.
  11. da Silva MO, Dadalt P, Santos OL, et al. Linear basal cell carcinoma. Int J Dermatol. 1995;34:488.
  12. Warthan TL, Lewis JE. Giant linear basal cell epithelioma. Int J Dermatol. 1994;33:284.
  13. Lewis JE. Linear basal cell epithelioma. Int J Dermatol. 1989;28:682-684.
  14. Alcántara-Reifs CM, Salido-Vallejo R, González-Menchen A, et al. Linear basal cell carcinoma: report of three cases with dermoscopic findings. Indian J Dermatol Venereol Leprol. 2016;82:708-711.
  15. Lee MS, Cho E, Lee JH, et al. Linearly curved, blackish macule on the wrist. Cutis. 2016;97:384, 406-407.
  16. Bajaj S, Sharma PK, Kar HK. Linear adamantinoid basal cell carcinoma in the axilla. Dermatol Online J. 2015;21. pii:13030/qt8k0713nb.
  17. Iga N, Sakurai K, Fujii H, et al. Linear basal cell carcinoma at the external genitalia. J Dermatol. 2014;41:275-276.
  18. Ichinokawa Y, Ohtuki A, Hattori M, et al. Linear basal cell carcinoma: a case report. Case Rep Dermatol. 2011;3:142-146.
  19. Becher GL, Affleck A, Fleming C, et al. Linear basal cell carcinoma occurs most commonly on the lower eyelid. Clin Exp Dermatol. 2011;36:311-312.
  20. Jellouli A, Triki S, Zghal M, et al. Linear basal cell carcinoma. Actas Dermosifiliogr. 2010;101:648-650.
  21. Takiyoshi N, Nakano H, Kaneko T, et al. A linear basal cell carcinoma undergoing spontaneous regression. Clin Exp Dermatol. 2009;34:E411-E413.
  22. Yoleri L, Ozden S, Kandiloglu A. A 46-year-old male with an ulcerated linear lesion on his neck. Ann Saudi Med. 2008;28:57-58.
  23. Palleschi GM, Corradini D, Bruscino N, et al. Linear basal cell carcinoma: clinical significance and better surgical approach. G Ital Dermatol Venereol. 2016;151:119-121.
  24. Rodriguez-Garijo N, Redondo P. Linear basal cell carcinoma of the lower eyelid: reconstruction with a musculocutaneous transposition flap. JAAD Case Rep. 2018;4:633-635.
  25. Pierard GE, Lapiere CM. Microanatomy of the dermis in relation to relaxed skin tension lines and Langer’s lines. Am J Dermatopathol. 1987;9:219-224.
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Recurrence of Linear Basal Cell Carcinoma
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Recurrence of Linear Basal Cell Carcinoma
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Practice Points

  • Linear basal cell carcinoma (LBCC) follows a characteristic morphologic pattern of a length-to-width ratio that typically is at least 3 to 1.
  • Linear basal cell carcinomas most commonly present in the periocular region and on the neck along relaxed skin tension lines.
  • Because of the likelihood of subclinical spread, LBCC should be regarded as a high-risk subtype of basal cell carcinoma.
  • Mohs micrographic surgery or excision with complete circumferential peripheral and deep-margin assessment is recommended as first-line treatment.
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Nodule on the Second Toe in an Infant

Article Type
Quiz
Changed
Thu, 01/10/2019 - 13:22
Display Headline
Nodule on the Second Toe in an Infant
Author(s)
Katherine Gordon, MD
Michael Marchetti, MD
Mark Russell, MD

The Diagnosis: Infantile Digital Fibromatosis

On examination, the patient appeared well developed, well nourished, and had a 1×0.5-cm, flesh-colored, firm, nontender nodule on the dorsolateral aspect of the left second toe. After excision by a pediatric surgeon, the specimen was submitted for histopathologic examination. Dense bands of collagen with spindled myofibroblasts containing characteristic eosinophilic cytoplasmic inclusion bodies staining with phosphotungstic acid hematoxylin confirmed the diagnosis of infantile digital fibromatosis (Figure). Postoperatively the patient did well with normal healing and no complications. After 4 months, a recurrence was noted and the parents were considering reexcision.

Histopathology revealed a dense spindled cell proliferation in the dermis with low power (A)(H&E, original magnification ×4). High-power examination showed myofibroblasts with characteristic round and ovoid, eosinophilic, cytoplasmic inclusion bodies (B)(H&E, original magnification ×40). Inclusion bodies stained purple with phosphotungstic acid hematoxylin stain (C)(original magnification ×40).

Infantile digital fibromatosis is a rare, benign, often spontaneously regressing, fibrous tissue tumor of infancy and childhood.1 The prevalence of this tumor is unknown. It can be present at birth or more commonly appears in the first year of life. The lesions present as 1- to 2-cm, firm, flesh-colored nodules that initially grow slowly but have the potential for rapid growth in subsequent months. They occur preferentially on the extensor aspects of the digits, typically sparing the thumb and great toe.1 The clinical differential diagnosis includes keloids or hypertrophic scars, granuloma annulare, sarcoidosis, acral fibrokeratomas, periungual fibromas, supernumerary digits, pachydermodactyly, juvenile aponeurotic fibroma, and terminal osseous dysplasia and pigmentary defects.2

The histology of infantile digital fibromatosis is distinctive. Spindled myofibroblasts that contain round or ovoid, eosinophilic, cytoplasmic inclusion bodies composed of an accumulation of actin and vimentin filaments are characteristic.1 Inclusions are typically juxtanuclear and may indent the adjacent nucleus. The inclusion bodies stain red with Masson trichrome stain and purple with phosphotungstic acid hematoxylin stain.1 The histopathologic differential diagnosis includes scar, angiofibroma, dermatofibroma, neurofibroma, and angiofibromatous verruca vulgaris.

Although many treatments exist for infantile digital fibromatosis, optimal therapy is not standardized. Most lesions spontaneously regress, but func-tional disability with deforming contractures can occur if untreated. Topical therapy with imiquimod cream 5% and diflucortolone valerate cream have been reported to produce no effect on tumor size.3 Intralesional 5-fluorouracil was successful in treating a patient after 5 monthly injections.4 Intralesional triamcinolone 10 mg/cc injections were shown to be a well-tolerated and successful treatment in a case series of 7 patients.5 The most utilized intervention appears to be standard surgery, with a few patients treated with Mohs micrographic surgery.6,7

Treatment with surgical excision often results in recurrence, with studies showing a 50% to 75% recurrence rate.8,9 Our case is not atypical and illustrates this phenomenon. Other reported complications of surgical management include hypertrophic scarring and reduced distal interphalangeal joint mobility.5 Unless infantile digital fibromatosis causes mobility dysfunction or related disabilities, observation with regular follow-up should be considered, as lesions can spontaneously regress.

References

1. Heymann WR. Infantile digital fibromatosis. J Am Acad Dermatol. 2008;59:122-123.

2. Niamba P, Léauté-Labrèze C, Boralevi F, et al. Further documentation of spontaneous regression of infantile digital fibromatosis. Pediatr Dermatol. 2007;24:280-284.

3. Failla V, Wauters O, Nikkels-Tassoudji N, et al. Congenital infantile digital fibromatosis: a case report and review of the literature. Rare Tumors. 2009;1:e47.

4. Oh CK, Son HS, Kwon YW, et al. Intralesional fluorouracil injection in infantile digital fibromatosis. Arch Dermatol. 2005;141:549-550

5. Holmes WJ, Mishra A, McArthur P. Intra-lesional steroid for the management of symptomatic infantile digital fibromatosis. J Plast Reconstr Aesthet Surg. 2011;64:632-637.

6. Campbell LB, Petrick MG. Mohs micrographic surgery for a problematic infantile digital fibroma. Dermatol Surg. 2007;33:385-387.

7. Albertini JG, Welsch MJ, Conger LA, et al. Infantile digital fibroma treated with Mohs micrographic surgery. Dermatol Surg. 2002;28:959-961.

8. Kang SK, Chang SE, Choi JH, et al. A case of congenital infantile digital fibromatosis. Pediatr Dermatol. 2002;19:462-463.

9. Rimareix F, Bardot J, Andrac L, et al. Infantile digital fibroma—report on eleven cases. Eur J Pediatr Surg. 1997;7:345-348.

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Katherine Gordon, MD; Michael Marchetti, MD; Mark Russell, MD

Dr. Gordon is from the Department of Dermatology, University of Texas Southwestern, Dallas. Dr. Marchetti is from the Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York. Dr. Russell is from the Department of Dermatology, University of Virginia, Charlottesville.

The authors report no conflict of interest.

Correspondence: Katherine Gordon, MD, University of Texas Southwestern, 5323 Harry Hines Blvd, Dallas, TX 75390-9069 ([email protected]).

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Katherine Gordon, MD; Michael Marchetti, MD; Mark Russell, MD

Dr. Gordon is from the Department of Dermatology, University of Texas Southwestern, Dallas. Dr. Marchetti is from the Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York. Dr. Russell is from the Department of Dermatology, University of Virginia, Charlottesville.

The authors report no conflict of interest.

Correspondence: Katherine Gordon, MD, University of Texas Southwestern, 5323 Harry Hines Blvd, Dallas, TX 75390-9069 ([email protected]).

Author and Disclosure Information

Katherine Gordon, MD; Michael Marchetti, MD; Mark Russell, MD

Dr. Gordon is from the Department of Dermatology, University of Texas Southwestern, Dallas. Dr. Marchetti is from the Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York. Dr. Russell is from the Department of Dermatology, University of Virginia, Charlottesville.

The authors report no conflict of interest.

Correspondence: Katherine Gordon, MD, University of Texas Southwestern, 5323 Harry Hines Blvd, Dallas, TX 75390-9069 ([email protected]).

Article PDF
CT095040004-e.pdf
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The Diagnosis: Infantile Digital Fibromatosis

On examination, the patient appeared well developed, well nourished, and had a 1×0.5-cm, flesh-colored, firm, nontender nodule on the dorsolateral aspect of the left second toe. After excision by a pediatric surgeon, the specimen was submitted for histopathologic examination. Dense bands of collagen with spindled myofibroblasts containing characteristic eosinophilic cytoplasmic inclusion bodies staining with phosphotungstic acid hematoxylin confirmed the diagnosis of infantile digital fibromatosis (Figure). Postoperatively the patient did well with normal healing and no complications. After 4 months, a recurrence was noted and the parents were considering reexcision.

Histopathology revealed a dense spindled cell proliferation in the dermis with low power (A)(H&E, original magnification ×4). High-power examination showed myofibroblasts with characteristic round and ovoid, eosinophilic, cytoplasmic inclusion bodies (B)(H&E, original magnification ×40). Inclusion bodies stained purple with phosphotungstic acid hematoxylin stain (C)(original magnification ×40).

Infantile digital fibromatosis is a rare, benign, often spontaneously regressing, fibrous tissue tumor of infancy and childhood.1 The prevalence of this tumor is unknown. It can be present at birth or more commonly appears in the first year of life. The lesions present as 1- to 2-cm, firm, flesh-colored nodules that initially grow slowly but have the potential for rapid growth in subsequent months. They occur preferentially on the extensor aspects of the digits, typically sparing the thumb and great toe.1 The clinical differential diagnosis includes keloids or hypertrophic scars, granuloma annulare, sarcoidosis, acral fibrokeratomas, periungual fibromas, supernumerary digits, pachydermodactyly, juvenile aponeurotic fibroma, and terminal osseous dysplasia and pigmentary defects.2

The histology of infantile digital fibromatosis is distinctive. Spindled myofibroblasts that contain round or ovoid, eosinophilic, cytoplasmic inclusion bodies composed of an accumulation of actin and vimentin filaments are characteristic.1 Inclusions are typically juxtanuclear and may indent the adjacent nucleus. The inclusion bodies stain red with Masson trichrome stain and purple with phosphotungstic acid hematoxylin stain.1 The histopathologic differential diagnosis includes scar, angiofibroma, dermatofibroma, neurofibroma, and angiofibromatous verruca vulgaris.

Although many treatments exist for infantile digital fibromatosis, optimal therapy is not standardized. Most lesions spontaneously regress, but func-tional disability with deforming contractures can occur if untreated. Topical therapy with imiquimod cream 5% and diflucortolone valerate cream have been reported to produce no effect on tumor size.3 Intralesional 5-fluorouracil was successful in treating a patient after 5 monthly injections.4 Intralesional triamcinolone 10 mg/cc injections were shown to be a well-tolerated and successful treatment in a case series of 7 patients.5 The most utilized intervention appears to be standard surgery, with a few patients treated with Mohs micrographic surgery.6,7

Treatment with surgical excision often results in recurrence, with studies showing a 50% to 75% recurrence rate.8,9 Our case is not atypical and illustrates this phenomenon. Other reported complications of surgical management include hypertrophic scarring and reduced distal interphalangeal joint mobility.5 Unless infantile digital fibromatosis causes mobility dysfunction or related disabilities, observation with regular follow-up should be considered, as lesions can spontaneously regress.

The Diagnosis: Infantile Digital Fibromatosis

On examination, the patient appeared well developed, well nourished, and had a 1×0.5-cm, flesh-colored, firm, nontender nodule on the dorsolateral aspect of the left second toe. After excision by a pediatric surgeon, the specimen was submitted for histopathologic examination. Dense bands of collagen with spindled myofibroblasts containing characteristic eosinophilic cytoplasmic inclusion bodies staining with phosphotungstic acid hematoxylin confirmed the diagnosis of infantile digital fibromatosis (Figure). Postoperatively the patient did well with normal healing and no complications. After 4 months, a recurrence was noted and the parents were considering reexcision.

Histopathology revealed a dense spindled cell proliferation in the dermis with low power (A)(H&E, original magnification ×4). High-power examination showed myofibroblasts with characteristic round and ovoid, eosinophilic, cytoplasmic inclusion bodies (B)(H&E, original magnification ×40). Inclusion bodies stained purple with phosphotungstic acid hematoxylin stain (C)(original magnification ×40).

Infantile digital fibromatosis is a rare, benign, often spontaneously regressing, fibrous tissue tumor of infancy and childhood.1 The prevalence of this tumor is unknown. It can be present at birth or more commonly appears in the first year of life. The lesions present as 1- to 2-cm, firm, flesh-colored nodules that initially grow slowly but have the potential for rapid growth in subsequent months. They occur preferentially on the extensor aspects of the digits, typically sparing the thumb and great toe.1 The clinical differential diagnosis includes keloids or hypertrophic scars, granuloma annulare, sarcoidosis, acral fibrokeratomas, periungual fibromas, supernumerary digits, pachydermodactyly, juvenile aponeurotic fibroma, and terminal osseous dysplasia and pigmentary defects.2

The histology of infantile digital fibromatosis is distinctive. Spindled myofibroblasts that contain round or ovoid, eosinophilic, cytoplasmic inclusion bodies composed of an accumulation of actin and vimentin filaments are characteristic.1 Inclusions are typically juxtanuclear and may indent the adjacent nucleus. The inclusion bodies stain red with Masson trichrome stain and purple with phosphotungstic acid hematoxylin stain.1 The histopathologic differential diagnosis includes scar, angiofibroma, dermatofibroma, neurofibroma, and angiofibromatous verruca vulgaris.

Although many treatments exist for infantile digital fibromatosis, optimal therapy is not standardized. Most lesions spontaneously regress, but func-tional disability with deforming contractures can occur if untreated. Topical therapy with imiquimod cream 5% and diflucortolone valerate cream have been reported to produce no effect on tumor size.3 Intralesional 5-fluorouracil was successful in treating a patient after 5 monthly injections.4 Intralesional triamcinolone 10 mg/cc injections were shown to be a well-tolerated and successful treatment in a case series of 7 patients.5 The most utilized intervention appears to be standard surgery, with a few patients treated with Mohs micrographic surgery.6,7

Treatment with surgical excision often results in recurrence, with studies showing a 50% to 75% recurrence rate.8,9 Our case is not atypical and illustrates this phenomenon. Other reported complications of surgical management include hypertrophic scarring and reduced distal interphalangeal joint mobility.5 Unless infantile digital fibromatosis causes mobility dysfunction or related disabilities, observation with regular follow-up should be considered, as lesions can spontaneously regress.

References

1. Heymann WR. Infantile digital fibromatosis. J Am Acad Dermatol. 2008;59:122-123.

2. Niamba P, Léauté-Labrèze C, Boralevi F, et al. Further documentation of spontaneous regression of infantile digital fibromatosis. Pediatr Dermatol. 2007;24:280-284.

3. Failla V, Wauters O, Nikkels-Tassoudji N, et al. Congenital infantile digital fibromatosis: a case report and review of the literature. Rare Tumors. 2009;1:e47.

4. Oh CK, Son HS, Kwon YW, et al. Intralesional fluorouracil injection in infantile digital fibromatosis. Arch Dermatol. 2005;141:549-550

5. Holmes WJ, Mishra A, McArthur P. Intra-lesional steroid for the management of symptomatic infantile digital fibromatosis. J Plast Reconstr Aesthet Surg. 2011;64:632-637.

6. Campbell LB, Petrick MG. Mohs micrographic surgery for a problematic infantile digital fibroma. Dermatol Surg. 2007;33:385-387.

7. Albertini JG, Welsch MJ, Conger LA, et al. Infantile digital fibroma treated with Mohs micrographic surgery. Dermatol Surg. 2002;28:959-961.

8. Kang SK, Chang SE, Choi JH, et al. A case of congenital infantile digital fibromatosis. Pediatr Dermatol. 2002;19:462-463.

9. Rimareix F, Bardot J, Andrac L, et al. Infantile digital fibroma—report on eleven cases. Eur J Pediatr Surg. 1997;7:345-348.

References

1. Heymann WR. Infantile digital fibromatosis. J Am Acad Dermatol. 2008;59:122-123.

2. Niamba P, Léauté-Labrèze C, Boralevi F, et al. Further documentation of spontaneous regression of infantile digital fibromatosis. Pediatr Dermatol. 2007;24:280-284.

3. Failla V, Wauters O, Nikkels-Tassoudji N, et al. Congenital infantile digital fibromatosis: a case report and review of the literature. Rare Tumors. 2009;1:e47.

4. Oh CK, Son HS, Kwon YW, et al. Intralesional fluorouracil injection in infantile digital fibromatosis. Arch Dermatol. 2005;141:549-550

5. Holmes WJ, Mishra A, McArthur P. Intra-lesional steroid for the management of symptomatic infantile digital fibromatosis. J Plast Reconstr Aesthet Surg. 2011;64:632-637.

6. Campbell LB, Petrick MG. Mohs micrographic surgery for a problematic infantile digital fibroma. Dermatol Surg. 2007;33:385-387.

7. Albertini JG, Welsch MJ, Conger LA, et al. Infantile digital fibroma treated with Mohs micrographic surgery. Dermatol Surg. 2002;28:959-961.

8. Kang SK, Chang SE, Choi JH, et al. A case of congenital infantile digital fibromatosis. Pediatr Dermatol. 2002;19:462-463.

9. Rimareix F, Bardot J, Andrac L, et al. Infantile digital fibroma—report on eleven cases. Eur J Pediatr Surg. 1997;7:345-348.

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Nodule on the Second Toe in an Infant
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Nodule on the Second Toe in an Infant
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A 6-month-old male infant presented with a 1×0.5-cm, flesh-colored nodule on the dorsolateral aspect of the left second toe. The persistent, slowly enlarging, painless lesion was first noticed at 3 months of age and did not cause functional impairment. There was no preceding trauma and the patient’s medical history was otherwise noncontributory.
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