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Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report.
Medicine (Baltimore). 2017 Sep; 96(38):e7466.M

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.

Authors+Show Affiliations

Department of Oncology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

28930817

Citation

Wang, Jing, et al. "Cronkhite-Canada Syndrome Associated With Colon Cancer Metastatic to Liver: a Case Report." Medicine, vol. 96, no. 38, 2017, pp. e7466.
Wang J, Zhao L, Ma N, et al. Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report. Medicine (Baltimore). 2017;96(38):e7466.
Wang, J., Zhao, L., Ma, N., Che, J., Li, H., & Cao, B. (2017). Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report. Medicine, 96(38), e7466. https://doi.org/10.1097/MD.0000000000007466
Wang J, et al. Cronkhite-Canada Syndrome Associated With Colon Cancer Metastatic to Liver: a Case Report. Medicine (Baltimore). 2017;96(38):e7466. PubMed PMID: 28930817.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report. AU - Wang,Jing, AU - Zhao,Lei, AU - Ma,Nina, AU - Che,Juanjuan, AU - Li,Huihui, AU - Cao,Bangwei, PY - 2017/9/21/entrez PY - 2017/9/21/pubmed PY - 2017/9/29/medline SP - e7466 EP - e7466 JF - Medicine JO - Medicine (Baltimore) VL - 96 IS - 38 N2 - 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. SN - 1536-5964 UR - https://www.unboundmedicine.com/medline/citation/28930817/Cronkhite_Canada_syndrome_associated_with_colon_cancer_metastatic_to_liver:_A_case_report_ L2 - http://dx.doi.org/10.1097/MD.0000000000007466 DB - PRIME DP - Unbound Medicine ER -