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
A 72-year-old male complained of chronic diarrhea and weight loss of 10 kg in 1 month. He took a colonoscopy examination at a private clinic showing multiple colonic polyps of varying sizes. He was referred to the Gastrointestinal Department of Presbyterian Medical Center.\nThere were no abnormalities in his past medical and family history such as gastrointestinal polyposis or colorectal cancer. He had been drinking about 1.5 bottles of Soju (Korean liquor) every day for 20 years.\nHe was showing chronic signs of illness, but his vital signs were relatively stable. He was 164.3 cm tall and weighed 47 kg (body mass index, 17.4). His physical examination revealed mild hair loss and hair pull test showed that more than 10 hairs were pulled out (). Black discolorations in both hands and feet were observed in limb examination (). Also, all fingernails of both hands were dry, cracked, and transformed ().\nA peripheral blood test showed a hemoglobin level of 13.1 g/dL, hematocrit 38.2%, and evidence of megalocytes with mean corpuscular volume of 105.2 fL, mean corpuscular hemoglobin of 36.1 pg, and mean corpuscular hemoglobin concentration of 34.3%. The white blood cell count was 5,600/mm3 and platelet count was 209,000/mm3. Biochemical examinations showed aspartate aminotransferase of 35 IU/L, an alanine aminotransferase of 24 IU/L, total protein level of 5.5 g/dL, albumin level of 3.4 g/dL, total bilirubin level of 0.6 mg/dL, calcium level of 8.6 mg/dL, blood urea nitrogen (BUN) level of 10 mg/dL, creatinine level of 0.8 mg/dL, Na/K/Cl/Co2 of 139/4.3/104/27 mEq/L, and serum carcinoembryonic antigen level of 5.8 ng/mL.\nThe esophagus was normal in gastroscopy, but several hundred strawberry-like polyps of varying sizes (0.5 to 2.5 cm) were observed in the stomach (). The number and sizes of the polyps increased in the distal stomach than in the proximal stomach. They were diagnosed as hamartomatous polyps from the biopsy. Rapid urease test (Campylobacter-like organism, CLO test) showed positive result and the stool Helicobacter pylori Ag were also positive. The duodenum was covered with multiple small hyperemic polypoid lesions and numerous tiny polyps on whole small bowel were also observed by capsule endoscopy, but these were not confirmed histologically (). Thousands of little grape-like polyps that were less than 10 mm in size and some colon polyps of more than 1 cm in size were observed during colonoscopy. Small scale-like hyperemic mucosa under the size of 1 mm was observed without normal mucosa in the terminal ileum. These colon polyps were resected by endoscopic method, and the pathologic results confirmed colon cancer (adenocarcinoma in situ) () and serrated adenoma ().\nColonoscopic polypectomy was performed on multiple polyps that were larger than 1 cm. Most of them were inflammatory polyps containing lamina propria, and proliferated tortuous and cystic dilated glands were observed (). However, adenocarcinoma in situ with background low grade tubular adenomas was confirmed in one of them (). Also, another polyp showed serrated architecture and cytological dysplasia of the crypts, corresponding to serrated adenoma ().\nThe patient was given a nutritional therapy under the diagnosis of CCS. Prednisolone of 40 mg/day was administered, and the dosage was reduced by 5 mg every week. H. pylori eradication (proton pump inhibitor, amoxicillin, and metronidazole combination therapy for 2 weeks) was performed and the follow-up CLO test was negative.\nAt 4 months follow-up, although the patient appeared to have improved, hundreds of 0.5 to 2.5 cm-sized hyperplastic gastric polyps were not improved compared to the previous endoscopic findings. In the follow-up colonoscopy, numerous colon polyps were still discovered in the whole colon. Additional colon polypectomy was performed and the pathologic results confirmed inflammatory polyps and tubular adenoma. The patient is under observation in the outpatient clinic.