Giant Stomach Secondary to Juvenile Polyposis Syndrome.
Summary of "Giant Stomach Secondary to Juvenile Polyposis Syndrome."
Juvenile polyposis syndrome (JPS) is a rare, autosomal dominant condition. The polyps predominate in the colon but may be seen less commonly in the stomach or small intestine. We report an unusual case of JPS associated with massive gastric polyposis, resulting in a giant stomach, severe anemia, hematemesis, protein-losing enteropathy, and gastric outlet obstruction. Progressive complications ultimately necessitated a total gastrectomy.
A 27-year-old woman presented in 2005 with severe anemia. Gastroscopy revealed severe gastric polyposis. Histopathology confirmed juvenile polyposis. Gastrectomy was initially declined. Progressive hematemesis, hypoproteinemia, and gastric outlet obstruction, however, resulted in a total gastrectomy 5 years following initial presentation. Massive gastric polyposis resulted in severe gastromegaly.
Department of Surgery, Mount Sinai Hospital, 600 University Avenue, Suite 1225, Toronto, ON, M5G 1X5, Canada.
This article was published in the following journal.
Name: Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22005895
- DOI: http://dx.doi.org/10.1007/s11605-011-1714-4
Medical and Biotech [MESH] Definitions
Adenomatous Polyposis Coli
A polyposis syndrome due to an autosomal dominant mutation of the APC genes (GENES, APC) on CHROMOSOME 5. The syndrome is characterized by the development of hundreds of ADENOMATOUS POLYPS in the COLON and RECTUM of affected individuals by early adulthood. The lifetime risk of colorectal cancer in these patients reaches 100 percent by age 60.
Adenomatous Polyposis Coli Protein
A negative regulator of beta-catenin signaling which is mutant in ADENOMATOUS POLYPOSIS COLI and GARDNER SYNDROME.
Tumor suppressor genes located in the 5q21 region on the long arm of human chromosome 5. The mutation of these genes is associated with familial adenomatous polyposis (ADENOMATOUS POLYPOSIS COLI) and GARDNER SYNDROME, as well as some sporadic colorectal cancers.
A variant of ADENOMATOUS POLYPOSIS COLI caused by mutation in the APC gene (GENES, APC) on CHROMOSOME 5. It is characterized by not only the presence of multiple colonic polyposis but also extracolonic ADENOMATOUS POLYPS in the UPPER GASTROINTESTINAL TRACT; the EYE; the SKIN; the SKULL; and the FACIAL BONES; as well as malignancy in organs other than the GI tract.
Giant Cells, Langhans
Multinucleated cells (fused macrophages) seen in granulomatous inflammations such as tuberculosis, syphilis, sarcoidosis, and deep fungal infections. They resemble foreign-body giant cells (GIANT CELLS, FOREIGN BODY) but Langhans giant cells contain less chromatin and their nuclei are arranged peripherally in a horseshoe-shaped pattern. Langhans giant cells occur frequently in delayed hypersensitivity.
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