Extraosseous Ewing's Sarcoma of the Pancreas: An Uncommon but Treatable Disease

Abstract

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare but aggressive and malignant tumor, and has been reported in various sites such as the lungs, biliary tract, kidney, prostate, stomach, esophagus, oral cavity, salivary glands, urinary bladder, uterus, cervix, gonads, and vagina. However, the pancreas is considered to be an extremely uncommon site and only a handful of cases have been published to date. We present here another case of a pancreatic ES/PNET. Our case intensifies the importance to recognize this rare type of tumor in the pancreas as there is a broad spectrum of tumors with a similar morphology that includes sheets of small, round blue cells. As observed in our present case, this problem is markedly challenged when the tumor site of origin is uncertain.

Keywords: cytogenetics; ewing's sarcoma; neuroectodermal tumor; pancreas; sarcomatoid carcinoma.

Figure 1. Computed tomography (CT) scan of the abdomen showing a big mass in the body and tail of the pancreas

CT scan examination showed an 8 x 10 cm mass in the body and part of the tail of the pancreas with hypodense non-enhancing areas suggestive of necrosis or cystic change.