Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype

Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin

Gonadal metastases in neuroblastoma: A Sequel of prolonged chemotherapy?

Background: A large majority of neuroblastoma continue to present with advanced disease with widespread dissemination. However, metastasis to the ovary or testis is infrequent with sparse literature even from the major neuroblastoma study groups. The aim of this study was to determine the group of patients in whom such a rare entity has occurred and evaluate any common factor and clinical implication. Materials and methods: We retrieved records of all patients with abdominal neuroblastoma operated from January 2008 to August 2013. We selected the cases with overt gonadal (ovarian or testicular) metastasis at presentation or relapse and noted the details. Results: Of the 186 cases of neuroblastoma four patients were having metastasis to the gonads (testis = 2, ovary = 2). All the four cases had extensive abdominal disease and received prolonged chemotherapy before the institution of local surgical treatment. Conclusion: Protracted chemotherapy without timely and adequate local treatment may contribute to the metastases seen in unusual sites such as the gonads. However, the therapeutic implication of identifying gonadal metastases is uncertain in patients already having disseminated disease

Intracranial extramedullary hematopoiesis masquerading as progressive metastasis in a child with stage 4 neuroblastoma: Utility of sulfur colloid scan

Extramedullary hematopoiesis is production of blood cells outside bone marrow which occurs in a variety of disorders including myelofibrosis. We present a 15 month old child with stage 4 neuroblastoma with secondary myelofibrosis which resulted in intracranial extramedullary hematopoiesis, posing a diagnostic and therapeutic dilemma. This was resolved with a sulfur colloid isotope scan which confirmed extramedullary hematopoiesis and not a progressing metastasis. This helped us continue therapy with curative intent. To the best of our knowledge we did not come across any report of neuroblastoma with myelofibrosis and intracranial extramedullary hematopoiesis mimicking progression of metastasis