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

Abstract

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.

Keywords:

• Cronkhite-Canada syndrome
• clinical characteristics
• imaging features
• treatment
• prognosis

Acknowledgment

The authors thank Dr. Wanzun Lin, Shanghai Proton and Heavy Ion Center, Fudan University Cancer Hospital, for his editorial assistance.

Ethical approval

Informed consent was obtained from each patient according to research proposals approved by the local ethics committee of Fujian Medical University.

Disclosure statement

The authors report no conflicts of interest concerning the materials or methods used in this study or the findings specified in this paper.

Additional information

Funding

This work was supported by the Startup Fund for Scientific Research, Fujian Medical University [Grant 2017XQ1058 and 2016QH054], the Youth Fund for Scientific Research, Fujian Provincial Health Commission [Grant 2017-1-56], the Provincial University Project of Fujian Education Department [Grant JK2017020], and the National Natural Science Foundation of China [Grant 81901243].