Munir A

Background: Renal Cell carcinoma (RCC) possesses the ability to metastasize to distant places most commonly lungs, lymph nodes, liver, bone, and brain. While RCC can metastasize potentially to any organ, the gastrointestinal tract involvement is exceedingly rare.

Case Presentation: A 76-year-old male veteran presented with complain of hematuria and was diagnosed initially with stage 3 (pT3cN0M0) clear cell RCC of the right kidney. He underwent right radical nephrectomy with caval thrombectomy. He was then followed for surveillance during which, based on his radiological imaging, he was found to have a mesenteric mass inseparable from the transverse colon, multiple pulmonary nodules, and a large hypo density in the liver. He underwent subsequent biopsy of the mesenteric mass, which confirmed metastatic RCC (clear cell).

He was started on sunitinib 50mg based on NCCN guidelines. He did well on sunitinib for a while, but repeat CT chest, abdomen, and pelvis scans showed progression of his disease. He was started on nivolumab as a second line agent as per the NCCN guidelines. While on nivolumab, he presented in early 2019 with an episode of GI bleeding (melena). He underwent repeat radiological imaging as well as an endoscopy, which showed medium size friable soft tissue mass in the 2nd part of duodenum. Biopsy of that mass con rmed RCC eroding into the duodenal mucosa. His case was discussed at the tumor board, and it was recommended that palliative surgery and radiation were not an option for him. A recommendation for palliative and supportive treatment were made. The patient’s condition was discussed with him, and given that he was asymptomatic and at his general baseline health, he opted to continue with the immunotherapy.

Conclusion: RCC metastasis to GI tract is rare. The duodenum is reported to be the least involved segment of the small intestine. The number of cases reports in literature on duodenal metastasis from RCC is estimated to be around 20-25.Treatment of RCC with duodenal metastases depends upon the location and extent of the tumor as well as patient’s fitness for different modalities.

Background: Renal Cell carcinoma (RCC) possesses the ability to metastasize to distant places most commonly lungs, lymph nodes, liver, bone, and brain. While RCC can metastasize potentially to any organ, the gastrointestinal tract involvement is exceedingly rare.

Case Presentation: A 76-year-old male veteran presented with complain of hematuria and was diagnosed initially with stage 3 (pT3cN0M0) clear cell RCC of the right kidney. He underwent right radical nephrectomy with caval thrombectomy. He was then followed for surveillance during which, based on his radiological imaging, he was found to have a mesenteric mass inseparable from the transverse colon, multiple pulmonary nodules, and a large hypo density in the liver. He underwent subsequent biopsy of the mesenteric mass, which confirmed metastatic RCC (clear cell).

He was started on sunitinib 50mg based on NCCN guidelines. He did well on sunitinib for a while, but repeat CT chest, abdomen, and pelvis scans showed progression of his disease. He was started on nivolumab as a second line agent as per the NCCN guidelines. While on nivolumab, he presented in early 2019 with an episode of GI bleeding (melena). He underwent repeat radiological imaging as well as an endoscopy, which showed medium size friable soft tissue mass in the 2nd part of duodenum. Biopsy of that mass con rmed RCC eroding into the duodenal mucosa. His case was discussed at the tumor board, and it was recommended that palliative surgery and radiation were not an option for him. A recommendation for palliative and supportive treatment were made. The patient’s condition was discussed with him, and given that he was asymptomatic and at his general baseline health, he opted to continue with the immunotherapy.

Conclusion: RCC metastasis to GI tract is rare. The duodenum is reported to be the least involved segment of the small intestine. The number of cases reports in literature on duodenal metastasis from RCC is estimated to be around 20-25.Treatment of RCC with duodenal metastases depends upon the location and extent of the tumor as well as patient’s fitness for different modalities.

Background: Renal Cell carcinoma (RCC) possesses the ability to metastasize to distant places most commonly lungs, lymph nodes, liver, bone, and brain. While RCC can metastasize potentially to any organ, the gastrointestinal tract involvement is exceedingly rare.

Case Presentation: A 76-year-old male veteran presented with complain of hematuria and was diagnosed initially with stage 3 (pT3cN0M0) clear cell RCC of the right kidney. He underwent right radical nephrectomy with caval thrombectomy. He was then followed for surveillance during which, based on his radiological imaging, he was found to have a mesenteric mass inseparable from the transverse colon, multiple pulmonary nodules, and a large hypo density in the liver. He underwent subsequent biopsy of the mesenteric mass, which confirmed metastatic RCC (clear cell).

He was started on sunitinib 50mg based on NCCN guidelines. He did well on sunitinib for a while, but repeat CT chest, abdomen, and pelvis scans showed progression of his disease. He was started on nivolumab as a second line agent as per the NCCN guidelines. While on nivolumab, he presented in early 2019 with an episode of GI bleeding (melena). He underwent repeat radiological imaging as well as an endoscopy, which showed medium size friable soft tissue mass in the 2nd part of duodenum. Biopsy of that mass con rmed RCC eroding into the duodenal mucosa. His case was discussed at the tumor board, and it was recommended that palliative surgery and radiation were not an option for him. A recommendation for palliative and supportive treatment were made. The patient’s condition was discussed with him, and given that he was asymptomatic and at his general baseline health, he opted to continue with the immunotherapy.

Conclusion: RCC metastasis to GI tract is rare. The duodenum is reported to be the least involved segment of the small intestine. The number of cases reports in literature on duodenal metastasis from RCC is estimated to be around 20-25.Treatment of RCC with duodenal metastases depends upon the location and extent of the tumor as well as patient’s fitness for different modalities.

Background: In 2018, a male aged 62 years with a history of squamous cell tonsillar cancer, which was successfully treated by concurrent chemoradiation and selective neck dissection in 2014, was diagnosed with primary right sided lung adenocarcinoma (Stage IV, cT4c cN3 cM1a) after presenting with post-obstructive pneumonia. Molecular analysis was about 100% positive for PDL-1 and negative for EGFR, ALK, BRAF, and ROS. The patient was started on Pembrolizumab. After 5 cycles, he developed worsening dyspnea. A CT scan of his chest revealed a large pericardial effusion and decrease in size of right upper lobe lesion. An echocardiogram confirmed cardiac tamponade physiology. 400 ml of fluid was removed by emergent pericardial window and surgical drain was placed. Cytopathological analysis of pericardial fluid returned negative for malignant cells. The patient was treated with high dose prednisone with tapering, and pembrolizumab was discontinued. Patient responded very well. A repeat CT and ECHO after 3 months of treatment confirmed nearresolution of effusion.

Discussion: Distinguishing immune mediated pericardial effusion from malignant effusion is vital. Development of pericardial effusion with concurrent improvement or stability of malignant lesion, negative cytology and brisk response to corticosteroids indicates an immune etiology.

Pericardiocentesis or pericardial window is the key intervention in acute management. The critical step in immune mediated pericardial effusions is initiation of high-dose corticosteroids (1-2 mg/kg of Prednisone) with tapering of at least 4 weeks in severe cases. In unresponsive cases, corticosteroids at transplant rejection dose (1 g methylprednisone daily) and addition of mycophenolate mofetil, in iximab or antithymocyte globulin should be considered. As per ‘ASCO Practice Guideline’, any grade of cardiotoxicity above grade 1 warrants holding or permanently discontinuing the immunotherapy. For pericardial effusions secondary to pseudoprogression, immunotherapy can be continued.

Based on our literature review, concurrence of other immune related adverse events and recurrence of effusions with discontinuation of corticosteroids is reported with immune mediated pericardial effusions.

Conclusion: Early identification of immune related adverse events is very important. In immune mediated pericardial effusion, pericardial fluid should be drained, and corticosteroids should be started promptly. Permanent discontinuation of immunotherapy is recommended for grade 3 and 4 cardiotoxicity. Re-challenge of immunotherapy after treating the adverse event is a subject that needs further research.

Background: In 2018, a male aged 62 years with a history of squamous cell tonsillar cancer, which was successfully treated by concurrent chemoradiation and selective neck dissection in 2014, was diagnosed with primary right sided lung adenocarcinoma (Stage IV, cT4c cN3 cM1a) after presenting with post-obstructive pneumonia. Molecular analysis was about 100% positive for PDL-1 and negative for EGFR, ALK, BRAF, and ROS. The patient was started on Pembrolizumab. After 5 cycles, he developed worsening dyspnea. A CT scan of his chest revealed a large pericardial effusion and decrease in size of right upper lobe lesion. An echocardiogram confirmed cardiac tamponade physiology. 400 ml of fluid was removed by emergent pericardial window and surgical drain was placed. Cytopathological analysis of pericardial fluid returned negative for malignant cells. The patient was treated with high dose prednisone with tapering, and pembrolizumab was discontinued. Patient responded very well. A repeat CT and ECHO after 3 months of treatment confirmed nearresolution of effusion.

Discussion: Distinguishing immune mediated pericardial effusion from malignant effusion is vital. Development of pericardial effusion with concurrent improvement or stability of malignant lesion, negative cytology and brisk response to corticosteroids indicates an immune etiology.

Pericardiocentesis or pericardial window is the key intervention in acute management. The critical step in immune mediated pericardial effusions is initiation of high-dose corticosteroids (1-2 mg/kg of Prednisone) with tapering of at least 4 weeks in severe cases. In unresponsive cases, corticosteroids at transplant rejection dose (1 g methylprednisone daily) and addition of mycophenolate mofetil, in iximab or antithymocyte globulin should be considered. As per ‘ASCO Practice Guideline’, any grade of cardiotoxicity above grade 1 warrants holding or permanently discontinuing the immunotherapy. For pericardial effusions secondary to pseudoprogression, immunotherapy can be continued.

Based on our literature review, concurrence of other immune related adverse events and recurrence of effusions with discontinuation of corticosteroids is reported with immune mediated pericardial effusions.

Conclusion: Early identification of immune related adverse events is very important. In immune mediated pericardial effusion, pericardial fluid should be drained, and corticosteroids should be started promptly. Permanent discontinuation of immunotherapy is recommended for grade 3 and 4 cardiotoxicity. Re-challenge of immunotherapy after treating the adverse event is a subject that needs further research.

Background: In 2018, a male aged 62 years with a history of squamous cell tonsillar cancer, which was successfully treated by concurrent chemoradiation and selective neck dissection in 2014, was diagnosed with primary right sided lung adenocarcinoma (Stage IV, cT4c cN3 cM1a) after presenting with post-obstructive pneumonia. Molecular analysis was about 100% positive for PDL-1 and negative for EGFR, ALK, BRAF, and ROS. The patient was started on Pembrolizumab. After 5 cycles, he developed worsening dyspnea. A CT scan of his chest revealed a large pericardial effusion and decrease in size of right upper lobe lesion. An echocardiogram confirmed cardiac tamponade physiology. 400 ml of fluid was removed by emergent pericardial window and surgical drain was placed. Cytopathological analysis of pericardial fluid returned negative for malignant cells. The patient was treated with high dose prednisone with tapering, and pembrolizumab was discontinued. Patient responded very well. A repeat CT and ECHO after 3 months of treatment confirmed nearresolution of effusion.

Discussion: Distinguishing immune mediated pericardial effusion from malignant effusion is vital. Development of pericardial effusion with concurrent improvement or stability of malignant lesion, negative cytology and brisk response to corticosteroids indicates an immune etiology.

Pericardiocentesis or pericardial window is the key intervention in acute management. The critical step in immune mediated pericardial effusions is initiation of high-dose corticosteroids (1-2 mg/kg of Prednisone) with tapering of at least 4 weeks in severe cases. In unresponsive cases, corticosteroids at transplant rejection dose (1 g methylprednisone daily) and addition of mycophenolate mofetil, in iximab or antithymocyte globulin should be considered. As per ‘ASCO Practice Guideline’, any grade of cardiotoxicity above grade 1 warrants holding or permanently discontinuing the immunotherapy. For pericardial effusions secondary to pseudoprogression, immunotherapy can be continued.

Based on our literature review, concurrence of other immune related adverse events and recurrence of effusions with discontinuation of corticosteroids is reported with immune mediated pericardial effusions.

Conclusion: Early identification of immune related adverse events is very important. In immune mediated pericardial effusion, pericardial fluid should be drained, and corticosteroids should be started promptly. Permanent discontinuation of immunotherapy is recommended for grade 3 and 4 cardiotoxicity. Re-challenge of immunotherapy after treating the adverse event is a subject that needs further research.

Background: The translocation t(11;14)(q13;q32) typically considered a hallmark of mantle cell lymphoma(MCL), has also been implicated in some cases of non-MCL lymphoproliferative disorders. Although uncommon, it has been reported in 2-5% of chronic lymphocytic leukemia (CLL) cases. Most of the cases identified have been observed mostly in relapsed CLL. This genetic aberration can be considered a significant prognostic indicator for CLL. t(11;14) positive CLL at the time of diagnosis has been rarely reported. We describe a case of a patient
diagnosed with CLL who was positive for this genetic abnormality.

Case Report: A 64-year-old white male presented with absolute lymphocytosis of 7 years. Lymphocyte immunophenotype detected a CD5(+) CD10(-) CD23(+) CD38(-) CD43(+) FMC7(partial dim) kappa-restricted B-cell population consistent with CLL. CT chest, abdomen, pelvis showed mildly prominent mediastinal and hilar lymph nodes only and was thus classified as Rai stage I. Peripheral FISH came back positive for t(11:14), cyclin D1-IgH translocation. His EPO, Jak2 and BCR-ABL mutation were all negative (done for mild erythrocytosis). Immunoglobulin and SPEP were negative. UPEP showed high Kappa/Lambda ratio. Although this tumor carries t(11;14) (q13;q32) translocation, immunostaining for BCL-1 was negative. It is possible that the gene is not expressed. Based on the staining and the clinical presentation, MCL was excluded. Per NCCN guidelines, patient is receiving clinical monitoring for stage I CLL.

Conclusions: Translocations involving the immunoglobulin genes are commonly identified. Uncommon genomic abnormalities in CLL should be recognized as significant independent predictors of disease progression and survival. It is important to recognize cases of CLL with t(11;14) translocation to achieve risk-adapted treatment strategies, which might be required to treat such patients.

Background: The translocation t(11;14)(q13;q32) typically considered a hallmark of mantle cell lymphoma(MCL), has also been implicated in some cases of non-MCL lymphoproliferative disorders. Although uncommon, it has been reported in 2-5% of chronic lymphocytic leukemia (CLL) cases. Most of the cases identified have been observed mostly in relapsed CLL. This genetic aberration can be considered a significant prognostic indicator for CLL. t(11;14) positive CLL at the time of diagnosis has been rarely reported. We describe a case of a patient
diagnosed with CLL who was positive for this genetic abnormality.

Case Report: A 64-year-old white male presented with absolute lymphocytosis of 7 years. Lymphocyte immunophenotype detected a CD5(+) CD10(-) CD23(+) CD38(-) CD43(+) FMC7(partial dim) kappa-restricted B-cell population consistent with CLL. CT chest, abdomen, pelvis showed mildly prominent mediastinal and hilar lymph nodes only and was thus classified as Rai stage I. Peripheral FISH came back positive for t(11:14), cyclin D1-IgH translocation. His EPO, Jak2 and BCR-ABL mutation were all negative (done for mild erythrocytosis). Immunoglobulin and SPEP were negative. UPEP showed high Kappa/Lambda ratio. Although this tumor carries t(11;14) (q13;q32) translocation, immunostaining for BCL-1 was negative. It is possible that the gene is not expressed. Based on the staining and the clinical presentation, MCL was excluded. Per NCCN guidelines, patient is receiving clinical monitoring for stage I CLL.

Conclusions: Translocations involving the immunoglobulin genes are commonly identified. Uncommon genomic abnormalities in CLL should be recognized as significant independent predictors of disease progression and survival. It is important to recognize cases of CLL with t(11;14) translocation to achieve risk-adapted treatment strategies, which might be required to treat such patients.

Background: The translocation t(11;14)(q13;q32) typically considered a hallmark of mantle cell lymphoma(MCL), has also been implicated in some cases of non-MCL lymphoproliferative disorders. Although uncommon, it has been reported in 2-5% of chronic lymphocytic leukemia (CLL) cases. Most of the cases identified have been observed mostly in relapsed CLL. This genetic aberration can be considered a significant prognostic indicator for CLL. t(11;14) positive CLL at the time of diagnosis has been rarely reported. We describe a case of a patient
diagnosed with CLL who was positive for this genetic abnormality.

Case Report: A 64-year-old white male presented with absolute lymphocytosis of 7 years. Lymphocyte immunophenotype detected a CD5(+) CD10(-) CD23(+) CD38(-) CD43(+) FMC7(partial dim) kappa-restricted B-cell population consistent with CLL. CT chest, abdomen, pelvis showed mildly prominent mediastinal and hilar lymph nodes only and was thus classified as Rai stage I. Peripheral FISH came back positive for t(11:14), cyclin D1-IgH translocation. His EPO, Jak2 and BCR-ABL mutation were all negative (done for mild erythrocytosis). Immunoglobulin and SPEP were negative. UPEP showed high Kappa/Lambda ratio. Although this tumor carries t(11;14) (q13;q32) translocation, immunostaining for BCL-1 was negative. It is possible that the gene is not expressed. Based on the staining and the clinical presentation, MCL was excluded. Per NCCN guidelines, patient is receiving clinical monitoring for stage I CLL.

Conclusions: Translocations involving the immunoglobulin genes are commonly identified. Uncommon genomic abnormalities in CLL should be recognized as significant independent predictors of disease progression and survival. It is important to recognize cases of CLL with t(11;14) translocation to achieve risk-adapted treatment strategies, which might be required to treat such patients.