Hepatoblastoma in patients with molecularly proven familial adenomatous polyposis: Clinical characteristics and rationale for surveillance screening.

Abstract:

:Familial adenomatous polyposis (FAP) due to APC mutation is associated with an increased risk of hepatoblastoma. All cases of hepatoblastoma in patients with FAP reported in the literature were reviewed. One hundred and nine patients were identified. Thirty-five patients (of 49 with data) were diagnosed with hepatoblastoma prior to a later diagnosis of FAP (often in association with advanced colorectal carcinoma), emphasizing a need to identify patients earlier with germline APC mutations for early colorectal carcinoma screening. Hepatoblastoma may present at birth, and screening for hepatoblastoma in infancy in families with FAP prior to APC mutation testing results may be warranted.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Trobaugh-Lotrario AD,López-Terrada D,Li P,Feusner JH

doi

10.1002/pbc.27103

subject

Has Abstract

pub_date

2018-08-01 00:00:00

pages

e27103

issue

8

eissn

1545-5009

issn

1545-5017

journal_volume

65

pub_type

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