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.

Endoscopic management of biliary leaks: a systematic review with meta-analysis.

BACKGROUND: The first-line approach to the treatment of biliary leaks is endoscopic retrograde cholangiopancreatography. A variety of techniques can be used, including sphincterotomy, stenting, a combination of both techniques, or nasobiliary drainage. We performed a systematic review with meta-analysis to define the optimal strategy. METHODS: We searched MEDLINE/PubMed, EMBASE, CENTRAL, Scopus, Google Scholar, and Web of Science until January 2018 for randomized clinical trials, case-control studies, and prospective cohort studies. Data on procedure, success, and complication rate were extracted. Risk of bias was assessed. A network meta-analysis was performed to compare sphincterotomy alone vs. stenting alone vs. combination treatment. Stenting was further stratified into leak-bridging and short stenting. RESULTS: 11 studies out of 5085 references were included. Compared with sphincterotomy alone, the combination of sphincterotomy with leak-bridging stenting had the highest success rate (risk ratio [RR] 1.15, 95 % confidence interval [CI] 0.97 - 1.50), followed by leak-bridging stenting alone (RR 1.10, 95 %CI 0.84 - 1.44). For nonbridgeable leaks, stenting alone had a higher success rate than sphincterotomy alone (RR 1.07, 95 %CI 0.72 - 1.40). The combination of short stents with sphincterotomy had no added benefit (RR 0.94, 95 %CI 0.49 - 1.29). Overall quality of the included studies was considered to be moderate. CONCLUSIONS: We recommend sphincterotomy with stenting if the biliary leak can be bridged. If not, stenting alone with a short stent may be preferred in order to avoid sphincterotomy-related complications. More and larger studies are needed to confirm these findings.