Abstract

Abstract A 72-year-old male patient who presented with a painless right sided scrotal swelling for few months. History of stage A Chronic Lymphocytic Leukemia (CLL) since 2015- under surveillance by hematology team. On examination; 10 X 10 cm hard non-tender right-sided scrotal mass, normal left testicle, no peripheral lymphadenopathy or organomegaly. WBC count = 17.5 × 109 /L with neutrophils of 4.39 × 109 /L & lymphocytes of 11.16 × 109 /L. Testicular tumour marker; within normal CT chest, abdomen, and pelvis (CAP); some borderline enlarged left para-aortic and aorto-caval lymph nodes and unremarkable abdominal viscera. Right inguinal orchiectomy Histopathology and immunohistochemistry profile were consistent with a high-grade Richter’s transformation to diffuse large B cell lymphoma. MDT plan with hematologist: 6 cycles of R-CHOP and IV as well as intrathecal Methotraxate for CNS prophylaxis. Patient was given the option testicular radiotherapy vs orchidectomy and he opted for orchidectomy. After more than 24 months follow up, the patient had no relapse. Richter’s syndrome (RS) is the transformation of CLL to an aggressive form of lymphoma mostly represented by diffuse large B cell lymphoma (DLBCL) in 2 -10% of CLL patients. RS is clinically characterized by development of B symptoms (fever, night sweats and weight loss), rapid clinical deterioration and a rapid increase in the size of lymphadenopathy and /or splenomegaly. RS commonly arises in lymph nodes or bone marrow and disseminates to other organs. Presenting as a testicular mass, as in this case, is quite rare and our patient represents the 4th case reported in the literature.

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