Minhaz Ahmad, MD1, Nazif Chowdhury, MD2, Nasrin A. Chowdhury1, Atif Saleem, MD1; 1UHS Wilson Hospital, Johnson City, NY; 2United Health Services, Johnson City, NY
Introduction: Primitive neuroectodermal tumors (PNETs) are small round cell tumors belonging to the Ewing’s sarcoma family of tumors. They usually occur between the age of 6-25 years and mostly in soft tissues or bones. PNETs may rarely arise from solid organs like pancreas, only 26 cases have been reported so far. We are presenting the 27th case of PNET of pancreas in a 61-year-old male.
Methods: A 61-year-old male with history of active smoking, alcohol dependence, GERD presented to the ER with worsening abdominal pain associated with nausea, loss of appetite, early satiety and unintentional weight loss of 10 lbs for one month. Abdomen was nontender, distended with hypoactive bowel sound and a large mass was evident on physical exam. Lab showed WBC 14K/UL, AST 32 IU/L, ALT 38 IU/L, ALP 141 IU/L, Ca 10.6 MG/DL, Lipase 162 U/L, PT 15.1 sec, INR 1.29, CEA 2.1 NG/ML, CA 19-9 2 U/ML. CT abdomen with contrast showed a 18.5x11x20 cm cystic mass in the head of the pancreas. Upper GI endoscopy and endoscopic ultrasound revealed severe extrinsic compression by 15x12 cm, septated pancreatic cyst in the gastric body, antrum and prepyloric region causing gastric outlet obstruction. FNAC of the cyst showed no malignancy. With progressive worsening symptoms, he underwent excisional debridement of necrotic pancreatic cyst. Microscopy and immunohistochemistry of the specimen confirmed PNET of pancreas. With mild improvement initially, he continued to have persistent abdominal distention. Repeat CT abdomen showed enlarged cyst to the extent of previous level. Exploratory laparotomy revealed incorporation of cyst cavity into the distal part of the stomach, proximal duodenum, liver and gallbladder. He underwent excision of the pancreatic cyst, cholecystectomy, distal gastrectomy and Roux-en-Y gastrojejunostomy. Pathology revealed invasion of PNET to stomach, duodenum, liver capsule and gallbladder. Repeat CT abdomen showed multiple liver lesions. He was scheduled for outpatient PET scan and oncology follow up. Discussion: The PNETs are aggressive tumors with almost inevitable recurrence and metastasis. Metastasis to the bone, bone marrow, lymph nodes, lung, liver, and other organs have been reported. Currently, the standard treatment of PNETs is complete surgical resection with an adequate margin with systemic chemotherapy and/or radiotherapy. It is difficult to achieve complete resection of PNETs of pancreas because of the adjacent unresectable organs.
CT Abdomen with contrast: Pancreatic cyst
Upper GI Endoscopy: Extrinsic compression
PNET 40x, FLI1 and CD 99
Disclosures: Minhaz Ahmad indicated no relevant financial relationships. Nazif Chowdhury indicated no relevant financial relationships. Nasrin Chowdhury indicated no relevant financial relationships. Atif Saleem indicated no relevant financial relationships.