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Everything you need to know about Computed Tomography (CT) & CT Scanning

Colon: Polyposis Syndromes Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Colon ❯ Polyposis Syndromes

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  • “Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disease characterized by diffuse gastrointestinal hyperplastic polypoid lesions and ectodermal symptoms such as pigmentation, alopecia and onychatrophia. Since Cronkhite and Canada first reported about this syndrome in 1955, more than 500 cases have been reported worldwide, the majority of which were from Japan. The etiology of CCS is currently unknown, and the association between gastrointestinal polyposis and ectodermal symptoms is undetermined.”
    “Cronkhite-Canada Syndrome Successfully Treated by Corticosteroids before Presenting Typical Ectodermal Symptoms.”  
    Murata, Kazumoto et al.  
    Case reports in gastroenterology vol. 14,3 561-569. 30 Oct. 2020
  • “Cronkhite-Canada syndrome (CCS) is a noninherited condition associated with high morbidity and characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea.”
    Cronkhite-Canada syndrome: an acquired condition of gastrointestinal polyposis and dermatologic abnormalities.
    Sweetser S, Boardman LA.
    Gastroenterol Hepatol (N Y). 2012 Mar;8(3):201-3. 
  • “Polyps in CCS patients can develop throughout the gastrointestinal tract (except for the esophagus) and are non-neoplastic hamartomas. Nevertheless, there is concern that CCS polyps may possess malignant potential, as evidenced by the dysplastic intestinal changes noted by Seshadri and coworkers and by reports of gastric, colon, and rectal cancers in patients with CCS.”
    Cronkhite-Canada syndrome: an acquired condition of gastrointestinal polyposis and dermatologic abnormalities.
    Sweetser S, Boardman LA.
    Gastroenterol Hepatol (N Y). 2012 Mar;8(3):201-3. 
  • “The diagnosis of CCS should be considered in patients with gastrointestinal hamartomatous polyps, diarrhea, and the dermatologic triad of alopecia, hyperpigmentation, and onychodystro-phy. Malignant transformation of CCS polyps may occur, and the risk of colorectal cancer may warrant aggressive screening in CCS patients. Immunosuppression with corticosteroids or long-term azathioprine therapy may eradicate or lessen manifestations of CCS.”
    Cronkhite-Canada syndrome: an acquired condition of gastrointestinal polyposis and dermatologic abnormalities.
    Sweetser S, Boardman LA.
    Gastroenterol Hepatol (N Y). 2012 Mar;8(3):201-3. 
  • “FAP is the most common GI polyposis syndrome, characterized by the presence of more than 100 synchronous colorectal adenomas and a 100% lifetime risk of colorectal cancer. FAP is an autosomal dominant condition caused by germline mutations in the adenomatous polyposis coli (APC) gene on chromosome 5q21, although one-third of new cases are due to de novo mutations. APC is a tumor suppressor gene that functions in the Wnt pathway, and its loss leads to accumulation of b-catenin oncoprotein, with subsequent uncontrolled cellular proliferation.”
    Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management  
    Katabathina VS et al.
    RadioGraphics 2019; 39:1280–1301 
  • "Gastric fundic gland hamartomatous and hyperplastic polyps and duodenal adenomas are the most common extra- colonic GI findings in FAP; duodenal/periampul- lary cancer (4%–10%) is the most common cause of death in patients with FAP who have undergone prophylactic colectomy. Gastric carcinoma and small bowel polyps are not common in patients with FAP.”  
    Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management  
    Katabathina VS et al.
    RadioGraphics 2019; 39:1280–1301 
  • “Desmoids in patients with FAP have a predi- lection for surgical sites and mostly develop after prophylactic colectomy .The small bowel mesentery and musculoaponeurotic structures of the abdominal wall near prior surgical sites are the two most common locations for desmoids in FAP. Desmoids are locally aggressive and can lead to long-term and at times devastating complications, including bowel obstruction, mesenteric ischemia, ureteric obstruction, fistula, and abscess formation . Desmoids are the cause of death in up to 10% of patients with FAP.”
    Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management  
    Katabathina VS et al.
    RadioGraphics 2019; 39:1280–1301 
  • “JPS is an autosomal dominant condition characterized by the presence of multiple juvenile polyps in the colon and rectum (98%), the stomach (14%), and less commonly the duodenum, jejunum, and ileum. Up to 70% of colonic polyps develop in the proximal colon.The term juvenile refers to the histologic type of the polyps, not the age of clinical diagnosis or the age of onset of polyps.”
    Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management  
    Katabathina VS et al.
    RadioGraphics 2019; 39:1280–1301 
  • "Large polyps are seen as masslike lesions at CT and can prolapse and cause obstruction.The antropyloric region is the most common site for gastric polyps; rarely, diffuse gastric polyposis can be seen in the absence of colonic polyps and can be identified at upper GI contrast material study. Smaller gastric polyps can be seen as multiple small nodular masses carpeting the stomach or diffuse wall thickening at CT or MRI and can mimic Ménétrier disease or diffuse linitis plastica of diffuse gastric cancer.”
    Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management  
    Katabathina VS et al.
    RadioGraphics 2019; 39:1280–1301 
  • “PJS is characterized by histologically distinc- tive hamartomatous polyps of the GI tract, most frequently in the small bowel (96%), colorectum (25%–50%), and stomach (25%), along with characteristic mucocutaneous pigmentation in the perioral area (55). PJS is an autosomal domi- nant condition due to a mutation in the STK11 tumor suppressor gene (previously called LKB1), located on chromosome 19p13, which encodes a serine-threonine kinase involved in cell cycle activities, mTOR (mammalian target of rapamy- cin) regulation, chromatin remodeling, and Wnt pathway signaling (23,55). Ninety-four percent of affected PJS families demonstrate mutations of STK11; 25% of newly diagnosed PJS cases are sporadic with de novo mutations.”
    Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management  
    Katabathina VS et al.
    RadioGraphics 2019; 39:1280–1301 
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