Merkel Cell Carcinoma in the Setting of Chronic Lymphocytic Leukemia and Diffuse Large B-Cell Lymphoma

Alkhouri, Samaa; Afify, Omar; Alkhouri, Faris; Boalbanat, Hashem; Patel, Pragnesh

Merkel cell carcinoma (MCC) is a rare, rapidly growing, and highly malignant cutaneous tumor that typically presents in elderly males as an erythematous or violaceous plaque or nodule in sun-exposed areas. Risk factors include long-term ultraviolet (UV) exposure, Merkel cell polyomavirus (MCV) infection, immunosuppression, and lymphoproliferative disorders such as chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Given the aggressive nature of this tumor, patients may present with nodal and distal metastasis. Locoregional disease can be managed with definitive radiotherapy or surgery with or without adjuvant radiotherapy, depending on the case. Disseminated disease, on the other hand, often requires a multidisciplinary tumor board consultation to individually tailor the treatment. Possible treatments include systemic therapy with chemotherapy or immunotherapy, radiotherapy, and surgery. Here we report a case of a patient with a medical history significant for chronic lymphocytic leukemia and diffuse large B-cell lymphoma who presented with a rapidly growing lesion that contained neighboring MCC and CLL/SLL on biopsy. Management included immunotherapy with pembrolizumab and radiotherapy to limit the tumor's growth and spread. To the best of our knowledge, the coexistence of all three malignancies in a person is rare and has not been reported previously.