Abstracts
PO46
Triple Primary Malignancy: A case of Primary Cutaneous Marginal Zone Lymphoma transforming to a CD5+ B-cell Lymphoma in a patient with Invasive Ductal Carcinoma and Colon Adenocarcinoma
Introduction/Background/Significance: Primary cutaneous B-cell lymphomas (PCBCL) account for 25% of all primary cutaneous lymphomas. Primary Cutaneous Marginal Zone Lymphoma (PCMZL) is a rare, indolent subtype representing roughly 10-11% of all marginal zone lymphomas. Periorbital and facial presentations are considered atypical anatomical sites. Triple primary malignancies (three or more metachronous cancers) are statistically rare, occurring in less than 0.8% of cases. We present a case of colon and breast cancer with a subsequent lymphoma.
Materials and Methods/Case Presentation/Objective: An 84-year-old female was diagnosed with invasive ductal carcinoma 30 years prior and subsequently achieved remission. She was cancer-free until she was diagnosed with a Stage I well-differentiated adenocarcinoma arising from a tubulovillous adenoma of the cecum (pT2N0M0) 3 years prior, for which she underwent resection of the tumor. She later presented to the clinic with macular lesions on her face, and a biopsy revealed primary cutaneous marginal zone lymphoma. She was given treatment based on guidelines; however, in the interim, she noted progression of the skin lesions, which became more pruritic and erythematous. This progression was associated with painful cervical and inguinal lymph nodes. A workup showed a panhypercellular bone marrow with panhyperplasia, and a PET scan revealed a hypermetabolic mass at the left breast along with active skin lesions. A repeat biopsy of the skin lesions showed positive staining for CD5, favoring a transformation of the lymphoma. Cyclin D1 staining was negative, and a repeat Ki-67 was still at 15-25%. At this time, the patient is currently being treated with the Bendamustine-Rituximab (BR) regimen and Zanubrutinib for the lymphoma, with subsequent ongoing management for her recurrent breast cancer.
Results/Description/Main Outcome Measure(s): The most significant contribution of our case is the documentation of the presence of 3 primary malignancies, Invasive ductal carcinoma, colon adenocarcinoma and B-cell lymphoma which all occurred subsequently. This phenomenon occurs only in 0.8% of all oncology patients, which is considered an exceptionally rare phenomenon. The development of these primaries raises the question regarding genetic disposition such as germline mutations in tumor suppressor genes, or the long-term oncogenic effects of the patient's previous chemotherapy and radiotherapy. The late recurrence of breast cancer after three decades of remission, demonstrates the risk of dormant micro metastases. The management of these malignancies requires a balance, Anastrazole for ER/PR positive breast cancer, while lymphoma required a pivot to Zanabrutinib and Bendamustine-rituximab once standard Anti-CD20 therapy failed. The presence of CD5 expression is rare and is an ominous finding in cutaneous B-cell lymphoproliferative disorder.1,3,8 The patient's transition coincided with a shift from slow-growing patches to rapidly growing exophytic periorbital tumors. The transformation reclassified the tumor into a high-grade B-cell lymphoma which is a traditionally systemic and aggressive type of lymphoma.
Conclusion(s).: In conclusion, the case highlights the challenge of managing a patient with three concurrent malignancies. Carefully tailored systemic chemo-immunotherapy, guided by serial biopsies and multidisciplinary team is vital to managing triple primary malignancies. This case also highlights that sudden development of rapidly enlarging exophytic lesions from an indolent lymphoma should warrant immediate investigation and consider a more aggressive transformation
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Materials and Methods/Case Presentation/Objective: An 84-year-old female was diagnosed with invasive ductal carcinoma 30 years prior and subsequently achieved remission. She was cancer-free until she was diagnosed with a Stage I well-differentiated adenocarcinoma arising from a tubulovillous adenoma of the cecum (pT2N0M0) 3 years prior, for which she underwent resection of the tumor. She later presented to the clinic with macular lesions on her face, and a biopsy revealed primary cutaneous marginal zone lymphoma. She was given treatment based on guidelines; however, in the interim, she noted progression of the skin lesions, which became more pruritic and erythematous. This progression was associated with painful cervical and inguinal lymph nodes. A workup showed a panhypercellular bone marrow with panhyperplasia, and a PET scan revealed a hypermetabolic mass at the left breast along with active skin lesions. A repeat biopsy of the skin lesions showed positive staining for CD5, favoring a transformation of the lymphoma. Cyclin D1 staining was negative, and a repeat Ki-67 was still at 15-25%. At this time, the patient is currently being treated with the Bendamustine-Rituximab (BR) regimen and Zanubrutinib for the lymphoma, with subsequent ongoing management for her recurrent breast cancer.
Results/Description/Main Outcome Measure(s): The most significant contribution of our case is the documentation of the presence of 3 primary malignancies, Invasive ductal carcinoma, colon adenocarcinoma and B-cell lymphoma which all occurred subsequently. This phenomenon occurs only in 0.8% of all oncology patients, which is considered an exceptionally rare phenomenon. The development of these primaries raises the question regarding genetic disposition such as germline mutations in tumor suppressor genes, or the long-term oncogenic effects of the patient's previous chemotherapy and radiotherapy. The late recurrence of breast cancer after three decades of remission, demonstrates the risk of dormant micro metastases. The management of these malignancies requires a balance, Anastrazole for ER/PR positive breast cancer, while lymphoma required a pivot to Zanabrutinib and Bendamustine-rituximab once standard Anti-CD20 therapy failed. The presence of CD5 expression is rare and is an ominous finding in cutaneous B-cell lymphoproliferative disorder.1,3,8 The patient's transition coincided with a shift from slow-growing patches to rapidly growing exophytic periorbital tumors. The transformation reclassified the tumor into a high-grade B-cell lymphoma which is a traditionally systemic and aggressive type of lymphoma.
Conclusion(s).: In conclusion, the case highlights the challenge of managing a patient with three concurrent malignancies. Carefully tailored systemic chemo-immunotherapy, guided by serial biopsies and multidisciplinary team is vital to managing triple primary malignancies. This case also highlights that sudden development of rapidly enlarging exophytic lesions from an indolent lymphoma should warrant immediate investigation and consider a more aggressive transformation
Trademarked Items:
References:
