Familial adenomatous polyposis: Associations, variants and more

Suvendu Maji, Makhan Lal Saha, Kamal Singh Kanwar

Astrocyte 2016 3(2):118-120

Familial adenomatous polyposis (FAP) is the most common adenomatous polyposis syndrome. It affects all age groups and all races. Almost all patients with FAP develop colorectal cancers in the absence of surgery. A number of variants of FAP have been described, out of which Gardner syndrome is one subtype. Indian data on FAP or its rarer variants is scarce and limited to isolated case reports due to its low incidence. [1] We herein report five cases of FAP treated in our hospital and illustrate challenges in the management. The case series also highlights the interesting case of the fifth female who presented with synchronous duodenal adenocarcinoma and colonic adenocarcinoma in the background of multiple polyps in the entire colon. The case amply highlights the need of increased awareness of the syndrome and its variants while treating patients with polyposis-related conditions along with a center of referral where facilities for screening, counseling, genetic tests, surgery, and tumor banking and multidisciplinary facilities are available.

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