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GIGANTIC HYPERPLASTIC POLYPOSIS OF THE STOMACH WITH G CELL HYPERPLASIA IN A PATIENT WITH JUVENILE POLYPOSIS SYNDROME: A CASE REPORT
K Papay1, V Falck2, J Rehfeld3, R Panaccione1, M Storr1
1Department of Medicine, Division of Gastroenterology; 2Department of Pathology and Laboratory Medicine, University of Calgary, Calgary, Alberta; 3Department of Clinical Biochemistry, Rigshospitalet, Coppenhagen, Denmark
Aims: Juvenile Polyposis Syndrome (JPS) of the upper GI tract involves the development of hyperplastic-type polyps in the stomach. It has never been described in association with G cell hyperplasia and hypergastrinemia.
Methods: We present a 38 year-old woman with a previous diagnosis of Crohn's proctocolitis and juvenile polyposis of the colon diagnosed as an adolescent. At age 35, she ultimately required proctocolectomy for medically refractory Crohn's disease (CD). She presented in adulthood with recurrent and frequent nausea, vomiting and hematemesis.
Results: The esophagastroduodenoscopy (EGD) revealed massive carpeting of the corpus and fundus of the stomach with multiple giant villous polyps (3-5 cm) associated with superficial ulcerations, generalized oozing of blood, and causing gastric outlet obstruction. The antrum was covered with minor polyps. Histology diagnosed hyperplastic polyps. Treatment consisted of aggressive acid suppression with proton-pump inhibitor (PPI) therapy and repetitive endoscopic debulking of the mass of polyps to relieve obstruction. Despite this the patient continued to experience repeated episodes of gastric outlet obstruction and bleeding from the upper GI tract.
Over the next 3 years her symptoms progressed to complete gastric obstruction, and EGD and CT showed that the stomach was completely filled with a mass of polyps. Labwork was unremarkable except a gastrin of 5250 ng/L. Progastrin and its biosynthetic products (amidated gastrins, glycine-extended gastrins) were equably elevated. However, there was no detectable gastrinoma as evaluated by MIBG, octreotide-scintigraphy, PET, CT, MRI or EUS. Genetic testing for JPS (MADH4, BMPR1A) was negative.
The patient's condition continued to deteriorate with intractable post-prandial vomiting, persistent hypoalbuminema, anemia requiring blood transfusions and signs of severe malnutrition. Despite TPN she required total gastrectomy. Post-operative histopathology revealed hyperplastic-type polyposis consistent with JPS of the stomach and G cell hyperplasia in the epithelium of the polyps.
Conclusions: We describe a severe presentation of juvenile polyposis of the stomach, and for the first time present a case of JPS of the stomach associated with stomach G cell hyperplasia and hypergastrinemia.