The patient described here is a 64-year-old Japanese male with a past history of low anterior resection for rectal cancer at age 55, and a polypectomy for a colonic polyp at age 58. Family history was unremarkable. The patient has smoked 10 cigarettes per day and consumed 4 go (0.72 L) of shochu (a Japanese distilled spirit, 25% alcohol by volume) per day for 40 years. The patient has presented symptoms of muddy diarrhea since approximately January 2011, and visited a local doctor upon losing 4 kg of weight. A blood test revealed anemia and hypoproteinemia (hemoglobin: 6.7 g/dL, albumin (Alb): 3.2 g/dL), while a gastrointestinal (GI) endoscopy found multiple polyps in the stomach, duodenum, and large intestine. The patient therefore began attending the Digestive Disease Center at our hospital from March 2011.
Multiple polyps found by lower GI endoscopy, combined with the presence of associated symptoms (alopecia, onychatrophia, pigmentation, and dysgeusia), resulted in the diagnosis of CCS (Figure 1). A histopathological examination revealed the gastric lesions to be hyperplastic polyps, and the small and large intestine lesions to be inflammatory polyp. The base of the polyp and adjacent mucosa showed extensive edema and increased eosinophils in the lamina propria mucosa and dilated glands. These are typical histological features of CCS polyps. Hypoproteinemia caused due to liver cirrhosis and nephrotic syndrome was excluded because liver dysfunction, ascites, and proteinuria were not observed. Steroid treatment was not conducted due to the following factors: the patient was a heavy drinker, there was a possibility that the hypoproteinemia had not been corrected, and the patient had previously tested positive for tuberculosis with the QuantiFERON  test, which put him at risk for recurrence. During observation, the patient was therefore administered H2 blocker orally. Regular follow-up examinations revealed multiple gastric cancers and multiple colon adenomas, at which point the patient was admitted to our hospital for additional treatment in January 2012. A blood test revealed hypoproteinemia and undernutrition (total protein (T.P.): 5.08 g/dL, Alb: 3.08 g/dL, cholinesterase (CHE): 102 U/L), as well as abnormalities in trace elements (Fe: 18 g/dL, Zn: 60 g/dL, Ca: 8.12 mg/dL, P: 2.32 mg/dL). Immunoglobulin values, IgG (887 mg/dL) and IgE (1150 IU/mL) were high, while IgA (189 mg/dL) and IgM (82 mg/dL) were within normal limits. Carcinoembryonic antigen (CEA), a tumor marker, increased slightly from 5.0 to 7.6 ng/mL. An upper GI series found a dense growth in the stomach of small protrusions 10 mm or less, including 10-25 mm type 0-I lesions primarily in the gastric antrum, and type 0-IIc+III lesions in the gastric angle in the lesser curvature (Figure 2). Upper GI endoscopy found no abnormalities in the esophageal mucosa; however, large and small diffuse inflammatory polyps were found from the antrum cardiacum to the descending limb of the duodenum. Several 12-20 mm large protruding lesions indicative of type 0-I lesions were also found in the gastric antrum in the greater curvature, the posterior wall, and the pylorus in the posterior wall, which led us to suspect well-differentiated adenocarcinomas (Figure 3). Endoscopy of the small intestine revealed large and small inflammatory, partially villous polyps accompanied by oblong gland ducts with low duct density for 100 cm, from Bauhin's valve to the oral end. No polyps were found in the jejunum. Endoscopy of the large intestine revealed approximately 50 large and small inflammatory polyps with rugged surfaces predominantly in the right side of the colon (Figure 4).
Polypectomy was performed on 4 lesions that were diagnosed as adenomas in a biopsy taken at the time of the large intestine endoscopy. Of these 4 lesions, 2 were at the location of gland duct growth indicating neoplastic change on part of the mucosal epithelium, which in turn indicates hyperplastic change; these were diagnosed as low-grade tubular adenomas (Figure 5). A total gastrectomy was performed on the gastric lesions, because there was a possibility of cancer lurking in other polyps, and if the polyps were not removed, they may have become cancerous. Intraoperative findings included scattered melanoid pigmentation on the mesentery and the small intestinal wall. Large and small polyps were present in the surgical margin of the duodenum. Blinding the surgical margin would have resulted in the possibility of an anastomotic leak, thus making regular observation of duodenal polyps difficult. Therefore, we used the double tract method for reconstruction (Figure 6). The postoperative pathological report found atypical gland duct growth, inflammatory cell infiltration primarily into lymphocytes, and 4 gastric cancer lesions. All of these lesions were well-differentiated adenocarcinomas. The main lesion was well-differentiated tubular adenocarcinoma, intermediate type, 10x20 mm in size, invading submucosal layer (sm2), positive lymphatic invasion (ly1) and negative vascular invasion (v0). The tumor showed expanding growth and a distinct border with the surrounding tissue (Infiltration Alpha). No metastasis to the lymph nodes was observed (n0/52). Tumor cells were positive through full thickness for p53 and Ki67 and partially positive for MUC5AC and MUC2 (Figures 7 and 8). In addition, pathological examination detected Helicobacter pylori infection. The patient was discharged from the hospital 18 days after surgery with no postoperative complications.