Intestinal tumors in neurofibromatosis 1 with special reference to fatal gastrointestinal stromal tumors (GIST).

ABSTRACT:

Background

Type 1 neurofibromatosis (NF1) is a genetic tumor predisposing Rasopathy. NF1 patients have an increased risk for developing benign and malignant tumors, but the occurrence of intestinal tumors has not been investigated at the population level.

Methods

In this retrospective register-based total population study, diagnoses of gastrointestinal tract tumors were retrieved from the Finnish Care Register for Health Care for 1,410 NF1 patients and 14,030 reference persons. We also reviewed the death certificates of 232 NF1 patients who died during years 1987-2013, and specifically searched for diagnosis of gastrointestinal stromal tumor (GIST).

Results

The register analysis revealed an increased overall hazard ratio (HR) of 2.6 (95% CI 1.9-3.6) for intestinal tum

SUBMITTER: Yla-Outinen H 

PROVIDER: S-EPMC6732307 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature