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      • Disorders of Small and Large Intestine : Two Cases Of Cronkhite-Canada Syndrome With Gastrointestinal Cancer And Membranous Glomerulonephritis

        ( Eiki Nomura ),( Yoshitaka Kinouchi ),( Tooru Shimosegawa ),( Sho Takagi ),( Hisashi Shiga ),( Nobuya Obana ),( Katsuya Endo ),( Nobuo Ueki ),( Kenichi Negoro ),( Seichi Takahashi ) 대한소화기학회 2007 SIDDS Vol.9 No.-

        Background/Aims: Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disorder with gastrointestinal (GI) polyposis, associated with ectodermal changes. GI polyps are generally accepted to be nonneoplastic in nature. However, GI malignancy have been described in some CCS patients. Methods: Two cases of CCS associated with GI cancer and membranous glomerulonephritis (MGN) were examined about clinicopathological features. Results: 〔Case 1〕 A 71-year-old man had GI polyposis. The skin of his legs showed brown pigmentation and his fingernails and toenails were atrophic. He had proteinuria and the biopsied specimen of the kidney revealed MGN. We made a diagnosis of CCS associated with MGN. Treatment with methylprednisolone and cyclosporine ameliorated the symptoms and the GI polyposis Colonoscopy after the treatment showed the regression of the polyposis and the histological examination of the remnant polyp after endoscopic resection showed well differentiated adenocarcinoma. Twelve months after the resection, he achieved remission and no recurrence of the polyposis was found. 〔Case 2〕 A 58-year-old man was admitted our hospital for the examination of the progression of anemia. HE was diagnosed as MGN at 24 years old and was under medical treatment. He had poor nutrition, with the typical clinical features of CCS. He had also taste disturbance. Radiologic, endoseopic and histological examinations confirmed CCS polyposis in the stomach, small intestine and colon. He was treated with prednisolone, oral zinc and tranexamic acid and was improved of the symptoms. Six months after the diagnosis, follow-up upper GI scopy showed no change of the diffuse polyposis of the stomach, whereas the histological findings of the biopsied specimens from the lower body polyp revealed to be an adenocarcinoma. He was performed a total gastrectomy. Conclusions: Although the frequency of malignant transformation is considered to be low in CCS, it has definite malignant potential and a close follow-up examination is recommended.

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