Neuroendocrine tumor with diarrhea: not always the usual suspects - a case report of metastatic calcitoninoma with literature review

We describe a case of a 59-year-old man without relevant past medical history, presenting with chronic diarrhea and weight loss. Extensive laboratory analysis, stool cultures and gastro- and ileocolonoscopy could not identify a diagnosis. Abdominal imaging revealed a mass in the uncinate process of the pancreas with mesenteric adenopathies and liver metastases. Fine needle aspiration was compatible with a pancreatic neuroendocrine tumor with low proliferative capacity (Ki-67 <1%). Immunohistochemical staining was positive for calcitonin and serum calcitonin levels were clearly elevated. Surprisingly, ¹⁸FDG PET-CT scan was positive, but no tracer uptake was seen on ⁶⁸Gallium-DOTATOC PET-CT scan. Treatment with somatostatin analogues was not successful, but long-term tumor control could be obtained with Everolimus. However, no significant effect was seen on stool frequency despite additional treatment with multiple symptomatic therapies, liver-directed therapy with radio- and chemoembolization and additional external radiotherapy to the primary pancreatic tumor. Ondansetron, eventually, seems to be the only therapy, until now, causing a decrease in stool frequency.Functioning pancreatic calcitoninomas are considered to be a rare disease entity with few literature on optimal (nuclear) imaging and treatment. We discuss molecular insights regarding these aspects that can be of great interest to nuclear medicine physicians, pathologists, endocrinologists and gastroenterologists.