PURPOSE:

Neuroendocrine tumors of pancreas (PNET) are rare pancreatic neoplasms comprising 1-2% of all pancreatic tumors. The overall prognosis and long-term survival for PNET patients is far better than for patients with exocrine pancreatic cancer. PNETs are classified as functional or nonfunctional based on the presence or absence of a specific clinical syndrome associated with hormone oversecretion.

METHODS:

We present the case of a 36-year-old female with epigastric and right upper quadrant abdominal pain for 3 months associated with decreased appetite, early satiety and a 20-lb weight loss. On examination, she was cachectic with hepatomegaly.

RESULTS:

Laboratory assays showed elevated liver and pancreatic enzymes. On computed tomography (CT) scan of the abdomen and pelvis, there was a low-attenuation mass in the distal pancreatic tail measuring 4.7 × 2.4 cm with multiple liver masses, omental implants, left ovarian mass, and a small amount of ascites. CT-guided liver biopsy on pathology was consistent with a well-differentiated pancreatic neuroendocrine carcinoma with metastasis to the liver. Assays for biomarkers of pancreatic neuroendocrine tumors showed an elevated chromogranin A with normal to non-specific elevations of the rest.

CONCLUSIONS:

The patient and her family declined palliative chemoembolization of the liver lesions or palliative chemotherapy and desired home hospice. We describe here the presentation and course of the case as well as a literature review of PNET with particular emphasis on nonfunctioning PNETs.