Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report

Abstract

Rationale: Cronkhite-Canada Syndrome (CCS) is an idiopathic, nonhereditary syndrome haracterized by gastrointestinal (GI) polyposis and ectodermal changes including alopecia, onychatrophia, and pigmentation. CCS colon polyps were previously considered to be benign neoplasms. However, serrated adenoma was reported to be associated with malignant neoplasms in some cases of gastric and colorectal carcinomas, and esophageal cancers. Although malignant colon and gastric cancer have been reported in CCS, reports of distant metastasis have been rare in CCS.

Patient concerns: A 58-year-old male was referred from a nearby hospital with diarrhea and weight loss. The patient was hypoproteinemia (17.9 g/L), and multiple polyps were observed in the large intestine. He also had alopecia, onychatrophia, and dysgeusia.

Diagnoses: The presence of multiple polyps and associated symptoms of alopecia, onychatrophia, pigmentation, and dysgeusia informed the diagnosis of CCS.

Interventions: He was treated with 20mg dexamethasone acetate per day for about 3 months, 10 mg for about 9 month, 5 mg for about 1 year, and then maintained on 5 mg daily. Three years after starting treatment, colonoscopy revealed colon cancer and colon adenomas. A sigmoidectomy revealed 4 well-differentiated adenocarcinomas of the ulcerating type in the sigmoid colon, and tubularadenomas throughout the rest of the large intestine. He was treated with FOLFOX6 for 6 months. At this stage liver metastasis was found. A right hepatectomy was performed confirming hepatic metastasis of colonic adenocarcinoma, which was GPC-3(-), CD34(-), CK20(+), CDX-2(+), Hep(-), CK19(+), and CK8(+).The patient received 3 courses of hepatic arterial infusion chemotherapy.

Outcomes: The patient's status has been stable for more than 2 years, and there was no tumor recurrence or metastasis occurred.

Lessons: CCS is a rare cause of multiple polyposis most often treated with hormone therapy. Regular follow-ups are very important to ensure discovery of malignant tumors at an early stage. Studies with longer-term observations and larger sample sizes will be required to confirm these observations. However, characterization of molecular markers for the early detection of malignant transformation that might allow less invasive and more cost-effective surveillance of colon cancer is urgently sought.

Figure 1

Physical characteristics of patient with CCS. Clinical presentation, cutaneous symptoms: (A) Alopecia (sparse head hair), (B) Milphosis (sparse eyebrows), and (C) Nail atrophy.

Figure 2

At colonoscopy, polyps of various sizes with surface hyperemia and edema were observed. A–C, Numerous colonic polyps. D, The largest colonic polyp was 14 mm × 4 mm.

Figure 3

A, Focal low-level mucosal neoplasia. Tubular adenoma with mild hyperplasia. (HE stain ×10). B, Gastric polyps exhibiting millet fundic glands and pyloric glandular stomach mucosa, chronic inflammation with epithelial hyperplasia. (HE stain ×10).

Figure 4

Ulcerated, well-differentiated colonic adenocarcinoma with invasion of the muscularis and serosa. (HE stain ×10).

Figure 5

Tumor marker levels over time. A, AFP, (B) CA125, (C) CA199, and (D) CEA. The red line indicates the date of colon cancer diagnosis; the blue line indicates the date of liver metastasis discovery.

Figure 6

A, Double air-barium contrast examination revealed a narrow lumen with irregular contours, intestinal wall rigidity, and thickened mucosal folds (a–b). The abdomen CT scan revealed bowel wall thickening suggesting tumor invasion (c–d). B, The abdomen CT scan at the time of colon resection revealed an incisional hernia (a–d).

Figure 7

The abdomen CT scan revealed low density liver metastases with irregular margins. A, Focal uneven liver metastases (8.8 × 4.5 cm) before chemotherapy. B, Liver metastases (5.2 × 3.7 cm), after 12 cycles of FOLFOX6 chemotherapy: (oxaliplatin 175 mg d1+ fluorouracil 800 mg d1, fluorouracil 4000 mg (civ), and calcium folinate 800 mg d1).