Cronkhite-Canada Syndrome Associated with Advanced Rectal Cancer Treated by a Subtotal Colectomy: Report of a Case

Abstract

A 41-year-old man with Cronkhite-Canada syndrome presented with multiple juvenile polyps with hyperplastic and adenomatous changes throughout his stomach and entire colorectum. Dysgeusia was recognized and the degree of hypoproteinemia was remarkable. A barium enema study and colonofiberscopy also revealed an advanced cancer in the rectum. Chronic hepatitis B and membranous glomerulonephritis were also present. It was difficult to design a conservative protocol using steroids for the treatment of protein-loosing enteropathy because the patient was a hepatitis B virus carrier. As a result, a subtotal colectomy while preserving the cecum with cecorectal anastomosis was performed. Pathologically, the ulcerated rectal tumor was a moderately differentiated adenocarcinoma with invasion into the muscularis propria. Most polyps showed cystically dilated glands without dysplasia or edematous stroma with inflammatory cell infiltration. A few polyps were juvenile-type polyps with adenoma components. Although no remarkable improvement was observed in the hypoproteinemia postoperatively, an alpha1-antitrypsin clearance test showed a significant decrease in protein loss from the gastrointestinal tract, which was only about one third of the loss seen preoperatively. These findings lead us to conclude that when improvement using conservative treatment can be neither obtained nor is expected, then the use of surgery should be considered when treating patients with Cronkhite-Canada syndrome.

A Case of Recurrent Cronkhite-Canada Syndrome Containing Colon Cancer

“…According to a summary of the reported treatments of CronkhiteCanada syndrome, 22 when improvement using conservative treatment can be neither obtained nor is expected, then the use of surgery should be considered when treating patients with CCS. 23,24 Whatever the outcome it is very important to perform a strict surveillance examination for CCS even in the remission stage and even when more research of this disease is necessary.…”

Clinicopathologic Features and Treatment Outcomes in Cronkhite–Canada Syndrome: Support for Autoimmunity

“…Although neoplastic (or malignant) transformation has not been convincingly linked to CCS, many recent reports describe the development of adenomas and carcinomas in affected patients [26][27][28][29][30][31][32][33][34][35][36][37]. Serrated adenomas have also been described [38].…”

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide survey

“…CCS is sometimes difficult to diagnose for clinicians and pathologists alike, owing to the considerable overlap of histologic and clinical features shared among CCS-related, juvenile, and inflammatory polyps [3, 12–14]. Its clinical course is characterized by progressive disease with occasional spontaneous remissions and frequent relapses [15–18], with rare progression to malignancy [1, 19, 20]. Concomitant cancers occurring in the gastrointestinal tract have been reported, although no systemic investigation of the cancer risk and prognosis has been published since the 1980s [11, 19–23].…”