Breast cancer in neurofibromatosis 1: survival and risk of contralateral breast cancer in a five country cohort study.

ABSTRACT:

Purpose

Neurofibromatosis 1 (NF1) is an autosomal dominant condition caused by pathogenic variants of the NF1 gene. A markedly increased risk of breast cancer is associated with NF1. We have determined the breast cancer survival and risk of contralateral breast cancer in NF1.

Methods

We included 142 women with NF1 and breast cancer from five cohorts in Europe and 335 women without NF1 screened for other familial breast cancers. Risk of contralateral breast cancer and death were assessed by Kaplan-Meier analysis with delayed entry.

Results

One hundred forty-two women with NF1 were diagnosed for breast cancer at a median age of 46.9 years (range 27.0-84.3 years) and then followed up for 1235 person-years (mean = 8.70 years). Twelve women had contralateral breast cancer

SUBMITTER: Evans DGR 

PROVIDER: S-EPMC7000349 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature