NEUROENDOCRINE DIFFUSE PANCREAS TUMOR: AN UNUSUAL PRESENTATION

Presentation: Female, 54 years old, with abdominal pain with 2 months of progression, associated with transitory jaundice and weight loss. Previously she was cholecystectomized and a recent diabetes diagnosis. On physical exam, she was with jaundice and no abdominal mass was present. Laboratory examinations showed elevation of CA 19-9 (66,2) Gama GT (1.520) alkaline phosphatase (764) and total bilirrubins (2,3). The MRI of biliary tree demonstrated a mild distension of biliary tract, and the pancreas was entirely enlarged and wholly affected by heterogeneous diffuse neoplasic lesion. An echoendoscopy showed a infiltrative lesion affecting all the pancreas, with a point of choledochal obstruction. A pancreatic biopsy was proceed, and the histopathological findings were a well differentiated neuroendocrine tumor, envolving all the pancreas. Toward the global involvement of the pancreas, a total pancreatectomy, splenectomy and lymphadenectomy was proceed. One lymph node was affected. The Immunohistochemistry revealed a positive pattern for chromogranin and synaptophysine and a Ki-67 of 4,3%. Discussion: pancreatic neuroendocrine tumors usually are focal and its treatment is resection: from enucleation to Whipple surgery. Untypically , in this case, a total pancreatectomy was need for a R0 ressection. Final comments: the surgical approach of pancreatic neuroendocrine tumors is the core of curative treatment in this kind of lesion. Specifically in this patient we faced a challenge: the need of a total pancreatectomy.

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Artikel online veröffentlicht:
23. Oktober 2019