K. Yamaguchi et al., Cronkhite-Canada syndrome associated with advanced rectal cancer treated by a subtotal colectomy: Report of a case, SURG TODAY, 31(6), 2001, pp. 521-526
A 41-year-old man with Cronkhite-Canada syndrome presented with multiple ju
venile polyps with hyperplastic and adenomatous changes throughout his stom
ach and entire colorectum. Dysgeusia was recognized and the degree of hypop
roteinemia was remarkable. A barium enema study and colonofiberscopy also r
evealed an advanced cancer in the rectum. Chronic hepatitis B and membranou
s glomerulonephritis were: also present. It was difficult to design a conse
rvative protocol using steroids for the treatment of protein loosing entero
pathy because the patient was a hepatitis B virus carrier. As a result, a s
ubtotal colectomy while preserving the cecum with cecorectal anastomosis wa
s performed. Pathologically, the ulcerated rectal tumor was a moderately di
fferentiated adenocarcinoma with invasion into the muscularis propria. Most
polyps showed cystically dilated glands without dysplasia or edematous str
oma with inflammatory cell infiltration. A, few polyps were juvenile-type p
olyps with adenoma components. Although no remarkable improvement was obser
ved in the hypoproteinemia postoperatively, an alpha1-antitrypsin clearance
test showed a significant decrease in protein loss from the gastrointestin
al tract, which was only about one third of the loss seen preoperatively. T
hese findings lead us to conclude that when improvement using conservative
treatment can be nei ther obtained nor is expected, then the use of surgery
should be considered when treating patients with Cronkhite-Canada syndrome
.