Cronkhite-Canada Syndrome Associated with Serrated Adenoma and Malignant Polyp: A Case Report and a Literature Review of 13 Cronkhite-Canada Syndrome Cases in Korea

Abstract

Cronkhite-Canada syndrome (CCS) is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances both in the gastrointestinal tract and in the epidermis. The pathologic finding of the polyp is usually a hamartomatous polyp of the juvenile type; however, the possibility of serrated adenoma associated malignant neoplasm was reported in some Japanese cases. Up till now in South Korea, 13 CCS cases have been reported, but there was no case accompanied by the colon cancer. We report the first case of CCS associated with malignant colon polyp and serrated adenoma in Korea. A 72-year-old male patient who complained of diarrhea and weight loss was presented with both hands and feet nail dystrophy, hyperpigmentation, and alopecia. Endoscopic examination showed numerous hamartomatous polyps from the stomach to the colon. The pathologic results confirmed colon cancer and serrated adenoma. Helicobacter pylori eradication and prednisolone was used. Thus, the authors report this case along with a literature review.

Cronkhite-Canada syndrome: A case report

“…These changes often occur later during the disease progression, usually several weeks or months subsequent to the GI symptoms (8). Consistent with this, in the present case study, the patient initially experienced diarrhea and hematochezia, followed by abdominal pain, nail and toenail loss, onychatrophy and hyperpigmentation.…”

“…CCS is a rare, acquired, nonhereditary syndrome with diffuse GI polyposis associated with ectodermal changes, including hyperpigmentation, alopecia and onychatrophy (8,9). Additionally, it has been reported that the CCS has been associated with poor prognosis and life-threatening malignant complications (7).…”

“…The prognosis of CCS is poor, with a 5-year mortality rate of 55% and the majority of mortality being associated with malnutrition, hypoalbuminemia, repetitive infection, sepsis, heart failure and GI bleeding (8). The natural history of CCS appears to be substantially improved owing to the sufficient dose and duration of corticosteroid therapy accompanied by nutritional support and periodic endoscopic surveillance (7).…”

Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

“…Carcinomas of the colorectum (most often distal) and stomach have been documented in 15–25% of patients with CCS at diagnosis and may be multiple 5 26 76–92. Additionally, up to 40% of patients with CCS have been found to harbour colorectal adenomas and adenomatous change is sometimes seen in close proximity to CCS polyps and carcinoma 12 79 82 86 93–95.…”

Cronkhite-Canada Syndrome (CCS)—A Rare Case Report

“…The index patient was a 68-year-old male with characteristic manifestations. Yun et al, reported a case of CCS in a 72-year-old male patient who presented with diarrhoea and weight loss along with both hand and feet nail dystrophy, hyperpigmentation, and alopecia [7]. Kao et al, mentioned a case of a 39-year-old Filipino woman who initially presented with diarrhoea but later developed significant amount of hair loss as well as hyperpigmentation of her palms and soles [8].…”