A case of Cronkhite-Canada syndrome with gastrointestinal cancer

Abstract

A 52-year-old woman was admitted to our hospital for further evaluation of intractable bloody diarrhea and a weight loss of 10 kg in two months. She had onychodystrophy, alopecia, hyperpigmentation of the skin, and laboratory examination revealed severe hypoproteinemia (serum total protein 3.8 g/dl) and hypokalemia (1.7 mmol/l). Endoscopy showed diffuse reddish sessile polyposis with edematous inflammatory mucosa in the stomach, small intestine and large intestine. Histological analysis of endoscopically-obtained biopsies demonstrated cystic dilatation of mucosal glands and invasion by inflammatory cells─compatible with Cronkhite-Canada syndrome (CCS)─and one of the pedunculated polyps from the transverse colon was diagnosed as non-invasive adenocarcinoma. Eradication therapy for H. pylori and mesalazine administration was performed as an initial treatment, but no symptomatic improvement was achieved. At that point, prednisolone therapy was initiated at 60 mg/day, tapering in combination with mercaptopurine (6-MP) administration. This treatment gradually improved the patient’s symptoms and nutritional status. The malignant colonic polyp was resected endoscopically. CCS is a rare disorder with nonhereditary gastrointestinal polyposis, and has recently been considered to be a high-risk factor for gastrointestinal carcinoma. Careful examination should be undertaken to ensure gastrointestinal carcinoma is not overlooked in cases of CCS.