TY - JOUR
T1 - Cronkhite-Canada syndrome six decades on
T2 - The many faces of an enigmatic disease
AU - Slavik, Tomas
AU - Montgomery, Elizabeth Anne
PY - 2014/10/1
Y1 - 2014/10/1
N2 - Cronkhite-Canada syndrome is a rare gastro-enterocolopathy 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.
AB - Cronkhite-Canada syndrome is a rare gastro-enterocolopathy 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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U2 - 10.1136/jclinpath-2014-202488
DO - 10.1136/jclinpath-2014-202488
M3 - Review article
C2 - 25004941
AN - SCOPUS:84907249290
SN - 0021-9746
VL - 67
SP - 891
EP - 897
JO - Journal of clinical pathology
JF - Journal of clinical pathology
IS - 10
ER -