Neuroendocrine pancreatic carcinoma after initial diagnosis of acute postpartal celiac disease in a 37-year old woman – fatal coincidence or result of a neglected disease?

An acute presentation of celiac disease (CD) after a pregnancy 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 diarrhoea 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 celiac patients. The diagnosis of acute onset of fulminant postpartal CD (Marsh score stage 3 c) could be confirmed histologically. Remarkably, simultaneous well-differentiated neuroendocrine non-functioning pancreatic neuroendocrine carcinoma (PNET) could be diagnosed due to radiological abdominal imaging which was performed since serum gastrin was remarkably high which was removed by distal pancreatectomy and splenectomy. This report is, to our knowledge, the first description of the 2 entities CD and PNET. Since results of antral histological studies showed diffuse hyperplasia of G-cells, probably in response to hypergastrinemia, ECL cell carcinogenesis might have served as a possible link between both diseases.