Cancer Family Syndrome

Cancer Family Syndrome

Article
Diseases & Conditions
Cancer & Benign Tumors
+1
Contributed byKrish Tangella MD, MBAMar 29, 2018

The topic Cancer Family Syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition Lynch Syndrome.

Quick Summary:

  • Lynch Syndrome is an autosomal dominant disorder caused by DNA mismatch repair defect. It is an inherited syndrome wherein there are increased malignancy of the gastrointestinal tract, particularly in the colon and rectum
  • Based upon histology as described by the pathology reports, 3 major types of the microsatellite instability malignancies have been described. They include:
    • Right-sided colon poorly differentiated cancers
    • Right-sided colon mucinous cancers
    • Adenocarcinomas of colon in any colonic location showing intraepithelial lymphocyte (IEL) response
  • In addition to colorectal cancers, individuals with Lynch Syndrome (Hereditary Non-Polyposis Colorectal Carcinoma-HNPCC) have an increased risk of cancers of following sites:
    • Stomach
    • Ovary
    • Endometrium
    • Small intestine in the duodenum and jejunum
    • Liver
    • Gallbladder ducts
    • Upper urinary tract transitional carcinoma of the ureter and renal pelvis
    • Brain glioblastoma
    • Skin
    • Prostate
  • Individuals with Lynch Syndrome have a 80% lifetime risk of developing colon cancer; most of which occur in the right side of colon

In order to identify high-risk individuals for Lynch Syndrome, two Amsterdam criteria are proposed. They are as follows:

  • Amsterdam criteria:
    • Three or more family members with a confirmed diagnosis of colorectal cancer, one of whom is a first degree (parent, child, sibling) relative of the other two
    • Two successive affected generations
    • One or more colon cancers diagnosed under age 50 years
    • Familial adenomatous polyposis (FAP) has been excluded
  • Amsterdam criteria II:
    • Three or more family members with HNPCC-related cancers, one of whom is a first degree relative of the other two
    • Two successive affected generations
    • One or more of the HNPCC-related cancers diagnosed under age 50 years
    • Familial adenomatous polyposis (FAP) has been excluded

Please find comprehensive information on Lynch Syndrome regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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