A Case of Recurrent Cronkhite-Canada Syndrome Containing Colon Cancer

Zhu, Xi; Shi, Haiyun; Zhou, Xiaona; Zong, Ye; Wang, Jin; Xiao, Jing; Zhang, Yanning; Tian, Ye

doi:10.9738/intsurg-d-14-00003.1

Cited by 5 publications

(7 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…According to other studies during 2000–2013 in Japan, 41 cases of CCS associated with colorectal cancer were reported [ 19 ]. In addition, from 2013 to 2015, 2 new cases were reported; thus, combined with our case, overall, 78 cases of CCS associated with colorectal cancer have been reported [ 10 , 15 ]. Regarding the sum of these cases and that, so far, more than 500 cases of CCS have been reported, we can say that the incidence of cancer in this syndrome is 15.6%.…”

Section: Discussionsupporting

confidence: 66%

See 1 more Smart Citation

Cronkhite-Canada Syndrome Associated with Metastatic Colon Cancer

Haghighi

Noorali

Alizadeh

2018

Case Rep Gastroenterol

View full text Add to dashboard Cite

Cronkhite-Canada syndrome is characterized by gastrointestinal and ectodermal manifestations. In this paper, we describe a 64-year-old Iranian male, presenting with Cronkhite-Canada syndrome with metastatic colon cancer. The patient was suffering from hair loss, which occurred on the scalp at first and then, during 5 months, extended to the whole body. After that, his sense of taste was impaired, and 2 months later, gastrointestinal symptoms gradually started, with weight loss of 20 kg over 2 months with an initial weight of 100 kg. Finally, he was admitted to our center 10 months after the onset of symptoms. On skin examination, generalized hair loss and hyperpigmentation and dysmorphic nail changes were observed. Multiple polyps within the colon and sigmoid were observed on colonoscopy. According to biopsies, a serrated adenoma and an invasive adenocarcinoma were reported in the ascending colon and sigmoid, respectively. Other polyps were pseudopolyps, and their characteristics were not significant. Computed tomography of the lungs and abdomen showed multiple adenopathies. On biopsy, metastatic adenocarcinoma was reported. The patient underwent chemotherapy with FOLFIRI and ERBITUX. Finally, after 5 courses of chemotherapy, his regimen was changed to FOLFOX and Avastin because of evidence of progression on computed tomography. The etiology of Cronkhite-Canada syndrome is currently unknown, and the optimal therapy has not been reported so far. This syndrome has many complications; the major of them is malignancy, and the prognosis is poor with a mortality rate of 50%. Therefore, annual monitoring is necessary in these patients.

show abstract

Section: Discussionsupporting

confidence: 66%

“…The major problem of CCS is malignancy [ 3 ]. As another complication, osteoporotic fractures may result from malabsorption of calcium or prolonged glucocorticoid therapy or both [ 6 , 15 ]. The prognosis of CCS is poor with a mortality rate of 50%.…”

Section: Discussionmentioning

confidence: 99%

Cronkhite-Canada Syndrome Associated with Metastatic Colon Cancer

Haghighi

Noorali

Alizadeh

2018

Case Rep Gastroenterol

View full text Add to dashboard Cite

show abstract

“…The effectiveness of hormone therapy has not been studied in large-scale trials, but is, nonetheless, the preferred drug regimen. [ 4 ] After gastric or bowel resection, and endoscopic polypectomies, corticosteroid treatment can provide satisfactory effects. The mechanism by which corticosteroid therapy achieves its beneficial effects may involve decreasing inflammation in the GI tract, and suppressing the autoimmune response.…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 ] 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 [ 2 ] and colorectal carcinomas, [ 3 , 4 ] and esophageal cancers. [ 5 ] More than 450 cases of CCS have been described in the literature, and the incidence of associated adenomatous changes has risen from 40% to more than 70% between 2012 and 2015.…”

Section: Introductionmentioning

confidence: 99%

Cronkhite-Canada syndrome associated with colon cancer metastatic to liver

Wang

Zhao²,

Ma³

et al. 2017

Medicine

View full text Add to dashboard Cite

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.

show abstract

“…65,66 There have been reports that colonic carcinomas can arise in the CCS polyps of colon. 66,67 Gastric adenocarcinomas have also been reported in patients with CC polyps. [68][69][70] At present, there is still no widely accepted algorithm or specific criteria on the diagnosis of CCS.…”

Section: Cronkhite-canada Syndrome Polypsmentioning

confidence: 99%

Gastric Hamartomatous Polyps—Review and Update

Vyas

Yang

Zhang

2016

Clin Med Insights Gastroenterol

View full text Add to dashboard Cite

Gastric polyps are frequently encountered on endoscopic examinations. While many of these represent true epithelial lesions, some of the polyps may result from underlying stromal or lymphoid proliferations or even heterotopic tissue. Histologic examination is essential for accurate typing of the polyps to predict malignant potential and underlying possible genetic abnormalities. The focus of this review is on gastric hamartomatous polyps, which are relatively rare and diagnostically challenging. Though most of the gastric hamartomatous polyps are benign, certain types are associated with increased malignant potential. These include certain polyps associated with specific genetic familial polyposis syndromes and gastric inverted hamartomatous polyps. Identification of these polyps can result in the prevention or early diagnosis of gastric carcinoma and also help in the identification of family members with polyposis syndromes. The aim of this review is to categorize gastric hamartomatous polyps and aid in the identification of high-risk categories.

show abstract

A Case of Recurrent Cronkhite-Canada Syndrome Containing Colon Cancer

Cited by 5 publications

References 24 publications

Cronkhite-Canada Syndrome Associated with Metastatic Colon Cancer

Cronkhite-Canada Syndrome Associated with Metastatic Colon Cancer

Cronkhite-Canada syndrome associated with colon cancer metastatic to liver

Gastric Hamartomatous Polyps—Review and Update

Contact Info

Product

Resources

About