Cronkhite-Canada Syndrome Patients Research Articles

Endoscopic features help to identify the histopathological patterns of colorectal polyps in Cronkhite-Canada syndrome.

Cronkhite-Canada syndrome (CCS) is a rare nonhereditary gastrointestinal hamartomatous polyposis syndrome with a high risk of colorectal cancerogenesis. It is challenging to discriminate adenomas from nonneoplastic colorectal polyps macroscopically. This study aimed to explore the endoscopic features of different histopathological patterns of colorectal polyps in CCS. Sixty-seven lesions from 23 CCS patients were prospectively biopsied or resected during the colonoscopic examinationfor histopathological analysis. The Fisher's exact test and multivariate logistical analysis were conducted to reveal the predictive endoscopic features of CCS polyps with low-grade dysplasia (LGD) and adenomas. There were seven (10.4%) adenomas, 20 (29.9%) CCS-LGD, and 40 (59.7%) nonneoplastic CCS polyps. Polyps were large (>20 mm) in none of the adenomas, 30.0% of CCS-LGD polyps, and 2.5% of nonneoplastic CCS polyps (P < 0.001). The color of the polyps was whitish for 71.4% of adenomas, 10.0% of CCS-LGD polyps, and 15.0% of nonneoplastic CCS polyps (P = 0.004). Pedunculated polyps were detected in 42.9% of adenomas, 45.0% of CCS-LGD polyps, and 5.0% of nonneoplastic CCS polyps (P < 0.001). The proportions of types IV and VI in the Kudo classification were 42.9%, 95.0%, and 35.0% in adenomatous, CCS-LGD, and nonneoplastic CCS polyps, respectively (P = 0.002). The endoscopic activity was in remission for 71.4% of adenomas, 5.0% of CCS-LGD polyps, and 10.0% of nonneoplastic CCS polyps (P < 0.001). Endoscopic features, including the size, color, sessility, Kudo's pit pattern classification of polyps, and endoscopic activity, help identify the histopathological patterns of colorectal polyps in CCS.

Cronkhite-Canada syndrome: a retrospective analysis of four cases at a single medical center

Objective Cronkhite-Canada syndrome (CCS) is a rare disease that is characterized by multiple gastrointestinal polyps and ectodermal abnormalities. This study aimed to improve the understanding of CCS by presenting our patient data. Methods Clinical features, treatment, and outcomes of four CCS patients at a single medical center were retrospectively analyzed. Results The age of the patients ranged from 32 to 61 years (mean: 49.5 years), including three men and one woman. All the patients presented with gastrointestinal symptoms, ectodermal abnormalities, and multiple gastrointestinal polyps. Two patients showed abnormal immune indices. Three patients underwent magnetic resonance enterography, and the typical manifestations of small intestine involvement were diffuse wall thickening, high signal intensity on diffusion-weighted imaging, obvious enhancement, and multiple small nodular enhancements of the small intestine. The main histological manifestations were chronic inflammation and hyperplastic, adenomatoid, and hamartomatoid polyps. Eosinophilic infiltration was observed in two patients. One patient had rectal adenocarcinoma at the time of diagnosis. All the four patients received prednisone at a dose of 0.75–1 mg/kg/day, and had their gastrointestinal symptoms gradually resolved (including two with ectodermal abnormality and endoscopic remission). Two patients are currently receiving low-dose prednisone (2.5–5 mg/day) with no recurrence after a 1.5- and 6-year follow-up periods, respectively. Conclusion Magnetic resonance enterography has the potential to evaluate small-intestinal lesions in CCSs. Long-term therapy with low doses of prednisone may be beneficial in maintaining remission.

Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases

Introduction: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal (GI) polyposis and ectodermal dysplasia. So far, most of CCS related literatures are published as single case report or reviewed with limited case numbers. Our study was to update the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literatures and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. Results: (1) In Chinese population, people aged 50–70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. (2) The diverse range of GI manifestations was observed in all the patients, and almost all the patients had at least 1 symptom of ectodermal dysplasias. (3) All CCS patients presented multiple polyps in the GI tract except esophagus, and the size and appearance of polyps were diverse. Congestion, edema, and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%). (4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). The prevalence of cancer was 5.97% in Chinese CCS patients. Conclusions: Middle-aged and elderly people are the high-risk group. Various GI symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps. These GI polyps have a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients.

Endoscopic features and clinical correlation analysis of 24 patients with Cronkhite-Canada syndrome

Objective: To clarify the endoscopic changes prior to corticosteroid therapy in Cronkhite-Canada syndrome (CCS) patients and to explore the correlation between endoscopic features and clinical characteristics. Methods: A total of 24 CCS patients who were hospitalized in Peking Union Medical College Hospital from January 1999 to June 2019 and underwent gastroscopy and colonoscopy before corticosteroid therapy were retrospectively enrolled. The endoscopic images were re-interpreted. The demographic characteristics, clinical manifestations, laboratory tests and histopathological data were collected and analyzed. Results: Of all 24 patients, 15 (62.5%) were male and 9(37.5%) were female, with an average age of (59±10) years and disease course of 6 (1~36) months. Based on the endoscopic findings, the percentages of stomach, colon, duodenum, rectum and terminal ileum involvement were 100%, 100%, 95.7%, 66.7% and 50.0% respectively. Gastric involvement was more severe in the lower part of the body and the antrum of the stomach, while the cardia and the fundus were spared in 5 (20.8%) cases. Colonic involvement was more severe in the right colon. No patient showed remarkable esophageal involvement. The typical appearance under endoscopy were diffuse mucosal hyperemia and edema with polyps or nodular changes. The lesions may have mulberry-like or imbricate changes in severe cases. Lymphatic dilation in the duodenum was found in 47.8% patients. Most of the polyps were pedunculated or sub-pedunculated, with occasionally seen sessile polyps in the colon. The pit patterns of the 12 resected colon polyps in 11 patients could be classified as Kudo type Ⅲ(S), Ⅲ(L), Ⅳ and Ⅴ(I), among which 2 tubular adenomas had the Kudo type Ⅲ(L). Other resected polyps were hyperplastic polyps or CCS polyps. The disease duration prior to diagnosis was positively correlated with the maximum diameter of colon polyps (r=0.625, P=0.006). Serum albumin levels in patients with whole stomach involvement were significantly lower than those in patients with cardia spared [(29±8) g/L vs (37±5) g/L, P=0.034]. Conclusions: The typical initial endoscopic finding of CCS is multiple polyps or nodular changes on the background of diffuse hyperemia and edema lining the gastric, duodenal and colonic mucosa. Lymphatic dilatation in the duodenum could also be found. Some endoscopic features are correlated to clinical characteristics.

Cronkhite-Canada syndrome: A case report

Cronkhite-Canada syndrome (CCS) is a non-inherited, sporadic disorder characterized by generalized gastrointestinal polyps (hamartomas), cutaneous pigmentation, alopecia and onychodystrophy. More than 500 CCS patients have been reported, mostly from Asian countries. Patients with CCS have a propensity to develop colonic traditional serrated adenomas (TSA). Some authors found increased frequency of colonic carcinomas in CCS patients. In the present communication, we report a patient with CCS in whom a colonoscopic examination failed to disclose a coexistent TSA.A 73-year-old female had a history of alopecia and nail atrophy. Because of iron deficiency anemia and occult gastrointestinal bleeding, she underwent a colonoscopic examination.Colonoscopy revealed multiple broad-based polyps. Due to continuous bleedings, a coloproctectomy was performed four months after colonoscopy. Pathology disclosed 50 hamartomas and, unexpectedly, a TSA with high-grade dysplasia in the cecum.The TSA was either overlooked at colonoscopy or not interpreted as different from the other colonic polyps by the endoscopist. CCS cases are very rare in Western countries. Given this circumstance, it is suggested that, when confronting the next CCS case, endoscopists should perform a comprehensive colonoscopic examination, including chromoscopy and directed biopsies from irregular polyps, to rule out a TSA, an adenoma prone to evolve into invasive carcinoma.

Cronkhite‐Canada Syndrome with Complete Remission after Four Months of Prednisolone Therapy and Polypectomy

The etiology of and optimal treatments for Cronkhite‐Canada syndrome (CCS) are still undetermined. Fewer than 5% of CCS patients experience complete remission and 15% of CCS patients develop malignancies. Here, we present a case of CCS involving autoimmune disorders, in which rapid diagnosis, rapid commencement of therapy, and complete remission were achieved after 4 months of prednisolone therapy and polypectomy, although biopsy specimens from all residual colonic polyps revealed adenocarcinoma in one polyp 4 months after prednisolone therapy. It is important to identify gastrointestinal or colonic polyps harboring malignant changes and to examine the involvement of autoimmune mechanisms for better outcomes in CCS patients.

Histopathological insights into hair loss in Cronkhite–Canada syndrome: Diffuse anagen‐telogen conversion precedes clinical hair loss progression

Cronkhite-Canada syndrome (CCS) is a rare disorder characterised by gastrointestinal polyposis and ectodermal changes, represented by extensive alopecia. Detailed histopathological investigations of alopecic lesions in two female CCS patients with severe hair loss revealed a marked increase in telogen hair follicles with no sign of loss or of the minaturisation or atrophy of hair follicle structures and the absence of inflammatory change, despite severe inflammation in the gastrointestinal tract. These findings suggested that hair regrowth can be expected without systemic corticosteroids, if they are not necessary for treatment of the gastrointestinal tract, and that anagen-telogen transition is an early event preceding clinical hair loss in CCS.

Clinicopathologic Features and Treatment Outcomes in Cronkhite–Canada Syndrome: Support for Autoimmunity

Cronkhite-Canada syndrome (CCS) is a noninherited condition, associated with high morbidity, and characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea. All features may respond to immunosuppressive therapy, but little is known about the etiology. An autoimmune origin has been suggested but not proved. From a retrospectively selected cohort, we evaluated clinicopathologic features, including immunostaining for IgG4 (an antibody associated with autoimmunity), and therapeutic outcomes in a cohort of CCS patients to provide further insights into this disease. Cases included 14 consecutive CCS patients seen at the Mayo Clinic on whom tissue and follow-up were available. All histology was reviewed by an expert gastrointestinal pathologist. Immunostaining for IgG4 was performed on 42 polyps from CCS cases and on control tissues, including 46 histologically similar hamartomas [from juvenile polyposis syndrome (JPS)] and 20 normal mucosae (six stomach, three small bowel, and 11 colon). Clinical features and treatment outcomes were descriptive. All CCS cases had both upper and lower gastrointestinal polyps; most had typical dermatologic features of alopecia, hyperpigmentation, and onychodystrophy; and most had evidence of protein-losing enteropathy. Ten patients (71%) had adenomatous polyps and 2 (14%) had colorectal cancer. IgG4 immunostaining was positive (>5 cells/HPF) in 52% of CCS polyps compared to 12% of JPS polyps (P = 0.001); IgG4 staining was negative in all other control tissues. Of 11 CCS patients treated with oral corticosteroids, 91% achieved remission. Relapse was common with steroid tapering. Five patients who initially responded to corticosteroids were maintained in remission on azathioprine (2 mg/kg/day) with no relapse after a median of 4.5 years. Immunostaining for the autoimmune-related IgG4 antibody is significantly increased in CCS polyps compared to disease and normal control tissues. Furthermore, immunosuppression by corticosteroids or long-term azathioprine may eradicate or lessen manifestations of CCS. These histologic findings and treatment responses are consistent with an autoimmune mechanism underlying CCS.

A case of Cronkhite-Canada syndrome complicated by McKittrick-Wheelock syndrome associated with advanced villous adenocarcinoma

F w Cronkhite-Canada syndrome (CCS) is a rare noninherited disease characterized by hyperplastic GI polyposis with various GI symptoms, alopecia, and onychodystrophy.1 Although the etiology remains unclear, a significant umber of CCS patients with GI malignancy, especially olorectal adenoma and/or cancer, have been reported.2,3 McKittrick-Wheelock syndrome (MWS) is an infrequent disorder that has the clinical features of depletion syndrome characterized by dehydration, watery diarrhea, and various symptoms caused by hyponatremia and hypokalemia4 as well as CCS. The most frequently reported leions causing MWS are villous adenomas situated primarly in the rectum and sigmoid colon.4,5 We report an extremely rare case of a patient with CCS complicated by MWS, presumably caused by a large villous adenocarcinoma of the rectum.

Clinical characteristics of Cronkhite-Canada syndrome in Chinese: meta-analysis of 35 cases

To investigate the clinical characteristics of Chinese patients with Cronkhite-Canada syndrome (CCS). Relevant data of Chinese CCS patients from 1985 to 2006 were retrieved from Medline and Chinese biomedical database and a meta-analysis was conducted. A total of 35 CCS cases had been reported by Chinese hospitals with the clinical characteristics of gastrointestinal polyposis with ectodermal trilogy, mainly manifested as diarrhea, bellyache, weight loss, anemia, and edema. Canceration was reported in 2 patients. Some patients had symptomatic response to combination therapy. There might be racial or regional differences in CCS susceptibility; however, such information was often neglected in the medical records at home and abroad. New techniques, such as (99)Tc(m)-HSA scan, double-balloon enteroscopy and wireless capsule endoscopy provided new information on CCS. The clinical features of Chinese CCS patients are similar to those of the European or Japanese patients. Novel appliance, case report standardization and sharing database may promote the understanding of this rare syndrome.