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

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 adenom...

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Autores principales: Wang, Jing, Zhao, Lei, Ma, Nina, Che, Juanjuan, Li, Huihui, Cao, Bangwei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5617684/
https://www.ncbi.nlm.nih.gov/pubmed/28930817
http://dx.doi.org/10.1097/MD.0000000000007466
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author Wang, Jing
Zhao, Lei
Ma, Nina
Che, Juanjuan
Li, Huihui
Cao, Bangwei
author_facet Wang, Jing
Zhao, Lei
Ma, Nina
Che, Juanjuan
Li, Huihui
Cao, Bangwei
author_sort Wang, Jing
collection PubMed
description 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.
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spelling pubmed-56176842017-10-13 Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report Wang, Jing Zhao, Lei Ma, Nina Che, Juanjuan Li, Huihui Cao, Bangwei Medicine (Baltimore) 5700 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. Wolters Kluwer Health 2017-09-22 /pmc/articles/PMC5617684/ /pubmed/28930817 http://dx.doi.org/10.1097/MD.0000000000007466 Text en Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nd/4.0 This is an open access article distributed under the Creative Commons Attribution-NoDerivatives License 4.0, which allows for redistribution, commercial and noncommercial, as long as it is passed along unchanged and in whole, with credit to the author. http://creativecommons.org/licenses/by-nd/4.0
spellingShingle 5700
Wang, Jing
Zhao, Lei
Ma, Nina
Che, Juanjuan
Li, Huihui
Cao, Bangwei
Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report
title Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report
title_full Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report
title_fullStr Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report
title_full_unstemmed Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report
title_short Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report
title_sort cronkhite-canada syndrome associated with colon cancer metastatic to liver: a case report
topic 5700
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5617684/
https://www.ncbi.nlm.nih.gov/pubmed/28930817
http://dx.doi.org/10.1097/MD.0000000000007466
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