Neuroendocrine pancreatic carcinoma after initial diagnosis of acute postpartal coeliac disease in a 37-year old woman - fatal coincidence or result of a neglected disease?
An acute presentation after pregnancy of coeliac disease (CD) in the puerperium is a rare condition which has been described mostly in primigravidae in patients highly suspicious of latent CD. We report the case of a 37-year-old woman who was referred to our Hospital because of refractory watery diarrhea and malnutrition syndrome. Endoscopy of the upper gastrointestinal tract revealed the classic visual features of CD and in addition, some duodenal ulcers negative for Helicobacter pylori, which seems to be another clinical feature in patients with CD. The diagnosis of acute onset of fulminant postpartal CD (Marsh score stage 3c) was confirmed histologically. Remarkably, simultaneous well-differentiated neuroendocrine non-functioning pancreatic neuroendocrine carcinoma (PNET) was diagnosed on radiological abdominal imaging which was performed since serum gastrin was remarkably high, treated by distal pancreatectomy and splenectomy. This report is, to our knowledge, the first description of the two entities, CD and PNET occurring together. Since results of antral histological studies showed diffuse hyperplasia of G-cells, probably in response to hypergastrinaemia, enterochromaffin cell carcinogenesis might have served as a possible link between both diseases.
Department of Gastroenterology, Hepatology and Gastrointestinal Oncology, Bogenhausen Academic Teaching Hospital, Technical University of Munich, Englschalkinger Straße 77, 81925 Munich, Germany. Gastroenterologie@kh-bogenhausen.de.
Felix
F
Gundling
Andreas
A
Nerlich
Wolf
W
Heitland
Wolfgang
W
Schepp
D000328:Adult; D018278:Carcinoma, Neuroendocrine / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D002446:Celiac Disease / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D049590:Postpartum Period
Coeliac disease; pancreatic neuroendocrine carcinoma; puerperium