Cronkhite-Canada syndrome associated with colon cancer metastatic to liver

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.

Cronkhite-Canada Syndrome: A Case Report

“… 8 However, some studies have reported the presence of few cases with normal levels of these markers and the case in this study is one of such. 9 Also, this case had comorbidity of hypothyroidism and pulmonary hypertension. Some studies have depicted an association of CCS with hypothyroidism and various autoimmune originated diseases such as systemic lupus erythematosus, rheumatoid arthritis, and scleroderma.…”

Cronkhite‑Canada syndrome: A case report

“…CCS is also known as polyposis pigmentation-alopecia-onycholrophia syndrome, and GI polyposis and ectodermal changes are its two main features ( 16 ). For patients with CCS, diarrhea is the most common initial symptom, which may develop to substantially watery diarrhea, followed with symptoms of malabsorption, including weakness, anemia, weight loss, edema and dysgeusia ( 17 ).…”

Cronkhite-Canada syndrome: A case report

“…CCS is also known as polyposis pigmentation-alopeciaonycholrophia syndrome, and GI polyposis and ectodermal changes are its two main features (16). For patients with CCS, diarrhea is the most common initial symptom, which may develop to substantially watery diarrhea, followed with symptoms of malabsorption, including weakness, anemia, weight loss, edema and dysgeusia (17).…”