Pancreas malignant rhabdoid tumour in an adult from southern of Mato Grosso state, Brazil: case report

Abstract

Malignant rhabdoid tumour is a very rare neoplasm with a high lethality rate. Its neoplastic cells present rhabdomyoblastic features, although those cells must be differentiated from epithelioid sarcoma, melanoma, and rhabdomyosarcoma. Therefore, this is a case report of a 21-year-old man who presented a Malignantrhabdoid tumour in the head of the pancreas. Patient was submitted to partial pancreatomy and gastrectomy in block, splenectomy, and retroperitoneal lymphadenectomy. Anatomopathological analysis revealed invasive malignant neoplasm of the pancreas, which was classified as malignant epithelioid neoplasm according to the Department of Pathology, Emory University (Atlanta, United States of América). Systemic chemotherapy protocol consisted in ifosfamide, mesna and doxorrubicin, although after the first cycle of chemotherapy, patient died due to disease complications. According to literature only seven pancreatic neoplasms were described with rhabdoid features. Extra renal Malignant rhabdoid tumours diagnosis is challenging because of immunohistochemistry characteristics. Thus, this case report may contribute to medical literature, as recording and describing anatomo pathological and immunochemistry features of one more case of this rare neoplasm, which in this case was described in an uncommon age range and organ.