Spontaneous Regression of Polyposis following Abdominal Colectomy and Helicobacter pylori Eradication for Cronkhite-Canada Syndrome

Kato, Kimitoshi; Ishii, Yoshinori; Mazaki, Takerou; Uehara, Toshiki; Nakamura, Hitomoi; Kikuchi, Hiroshi; Yanagimoto, Hiroaki; Sato, Hideki; Mizuno, Shigeaki; Soma, Masayoshi; Henmi, Akihiro; Masuda, Hideki; Moriyama, Mitsuhiko; Tanaka, Masanori

doi:10.1159/000350321

Cited by 18 publications

(17 citation statements)

References 18 publications

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“…Numerous treatment approaches, including aggressive nutritional support,14 100 101 antibiotics,8 42 histamine receptor antagonists,14 cromolyn sodium,14 immune suppression (corticosteroids, azathioprine and cyclosporine),13 20 102 103 and surgery89 104 105—often in different combinations—have had variable success 4 6 100 102 106. Apart from nutritional support, corticosteroids are currently recommended as the mainstay of medical treatment,26 with azathioprine used for its steroid-sparing effect 13.…”

Section: Therapy and Outcomementioning

confidence: 99%

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Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

Slavík¹,

Montgomery

2014

J Clin Pathol

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Cronkhite–Canada syndrome is a rare gastro-entero-colopathy of uncertain aetiology first described almost 60 years ago. It is characterised by diffuse gastrointestinal polyposis sparing only the oesophagus, ectodermal abnormalities and an unpredictable but often fatal clinical course. The disease may demonstrate extremely diverse clinical and endoscopic features, which often leads to a delay in diagnosis. A high index of suspicion and recognition of the characteristic histological findings frequently facilitate a correct diagnosis, but the distribution of the gastrointestinal pathology and its microscopic features may be atypical. The pathologist thus requires a thorough knowledge of both the typical and many atypical faces of this disease, for which various documented therapies often still prove ineffective. Close correlation with clinical findings, including any pertinent ectodermal abnormalities, and careful examination of biopsies derived from polypoid and endoscopically spared mucosa will ensure a timely and correct diagnosis in patients with this enigmatic syndrome.

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Section: Therapy and Outcomementioning

confidence: 99%

“…CCS has an extremely variable clinical course, ranging from fulminant cases to those with initial remission of GI as well as systemic manifestations (occurring spontaneously or after therapy) 42 92 103 106 108–113. Less than 5% of patients have a complete remission, however, and overall outcome remains poor 114.…”

Section: Therapy and Outcomementioning

confidence: 99%

Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

Slavík¹,

Montgomery

2014

J Clin Pathol

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“…In addition, Goto et al [5] reported that mental stress and physical fatigue were the important risk factors for CCS. HP might also another factor since eradication of HP could reduce some symptoms in CCS patients [14,15]. There is very limited information in our study.…”

Section: Discussionmentioning

confidence: 78%

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

Huang

Wang

et al. 2020

Preprint

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Background: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal polyposis and ectodermal dysplasia. Its etiology still remains indefinite to us. Currently, the diagnosis is mainly based on typical clinical manifestations and endoscopic features. However, there is a lack of these information from Chinese CCS patients. Our study was to investigate the clinical and endoscopic characteristics of Chinese CCS patients. Methods: This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literature 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 gastrointestinal (GI) manifestations were observed in all the patients, and the most common symptoms were weight loss (98.80%) and loss of appetite (93.94%). Almost all the patients had at least one symptom of ectodermal dysplasias. 3) All CCS patients presented multiple polyps in the GI tract except esophagus. The size of polyps varied from 0.5 cm (88.64%) to 0.6-1.0 cm (81.82%) . The polyps were various in shapes, the most of them were nodular polyps (58.14%) ,what’s more,the sessile polyps were the most common(90.24%). 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%). There is only 5.97% cancer reported. Conclusions: middle-aged and elderly people are the high-risk group; various gastrointestinal symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps; these gastrointestinal polyps has a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the CCS patients in China.

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“…Treatment responses to corticosteroids or azathioprine also suggest possible underlying autoimmune mechanism. H. pylori infection was also reported to be related, and eradication of H. pylori seems to improve symptoms in some cases [9] [10].…”

Section: Discussionmentioning

confidence: 99%

Cronkhite-Canada Syndrome: A Case Report and Literature Review of Gastrointestinal Polyposis Syndrome

Shen¹,

Husson²,

Lipshutz³

2014

CRCM

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Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.

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Spontaneous Regression of Polyposis following Abdominal Colectomy and Helicobacter pylori Eradication for Cronkhite-Canada Syndrome

Cited by 18 publications

References 18 publications

Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

Cronkhite–Canada syndrome six decades on: the many faces of an enigmatic disease

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

Cronkhite-Canada Syndrome: A Case Report and Literature Review of Gastrointestinal Polyposis Syndrome

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