{"id":2673,"date":"2010-02-10T20:39:16","date_gmt":"2010-02-11T01:39:16","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2673"},"modified":"2010-02-10T20:45:12","modified_gmt":"2010-02-11T01:45:12","slug":"turcots-syndrome","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2010\/02\/turcots-syndrome\/","title":{"rendered":"Turcot’s Syndrome"},"content":{"rendered":"


\nPathophysiology<\/strong><\/p>\n

familial polyposis syndrome with association of large intestine adenomatous polyps and brain tumors<\/p>\n

Histology\/Gross Pathology<\/strong><\/p>\n

1) polyps are adenomas 2) polyps are found uniformly throughout colon and rectum (no skip areas)<\/p>\n

Associated Conditions<\/strong><\/p>\n

colorectal adenocarcinoma develops in virtually 100% of patients by age 40 years<\/p>\n

Biochemistry<\/strong><\/p>\n

impaired DNA repair occurs<\/p>\n

Inheritance\/Epidemiology<\/strong><\/p>\n

1) polyps almost always present after puberty 2) associated with deletion in long arm of chromosome 5 that codes for tumor suppressor gene APC 3) autosomal dominant<\/p>\n

Treatment<\/strong><\/p>\n

1) total colectomy with ileoanal anastomosis 2) screening test in offspring (who have 50% risk of disease) is flexible sigmoidoscopy (if disease occurs, there is always rectal involvement<\/p>\n

Tips for USMLE<\/strong><\/p>\n

in any patient who has both colon cancer and any type of brain tumor, think Turcot’s
\n
\n<\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology familial polyposis syndrome with association of large intestine adenomatous polyps and brain tumors Histology\/Gross Pathology 1) polyps are adenomas 2) polyps are found uniformly throughout colon and rectum (no skip areas) Associated Conditions colorectal adenocarcinoma develops in virtually 100% of patients by age 40 years Biochemistry impaired DNA repair occurs Inheritance\/Epidemiology 1) polyps almost […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[63],"tags":[1212,1208,1210,1209,1211],"yoast_head":"\nTurcot's Syndrome - 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