A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature
Primitive neuroectodermal tumors (PNETs) are small round cell malignant tumors classified as part of the Ewing’s sarcoma family of neoplasms, which represents approximately 1% of all sarcomas1. Predominantly occurring in soft tissues along the extremities, they have also been reported in a variety of organs such as kidney, urinary bladder, testis, ovary, uterus, heart and lung2, 3. Pancreatic PNETs (PPNET) are extremely rare and need to be distinguished from neuroendocrine carcinomas, small cell undifferentiated carcinoma, other childhood small round cell tumors, pancreatoblastomas, and pancreatic tumors. Knowledge about PPNET is scarce; only 17 reports can be found in the literature. Here is reported the case of a 25 year old woman with a solid-cystic mass at the pancreatic head that later revealed to be a PPNET.