Gender dimorphism and age of onset in malignant peripheral nerve sheath tumor preclinical models and human patients.

ABSTRACT: Gender-based differences in disease onset in murine models of malignant peripheral nerve sheath tumor (MPNST) and in patients with Neurofibromatosis type-1-(NF-1)-associated or spontaneous MPNST has not been well studied.Forty-three mGFAP-Cre+;Ptenloxp/+;LSL-K-rasG12D/+ mice were observed for tumor development and evaluated for gender disparity in age of MPNST onset. Patient data from the prospectively collected UCLA sarcoma database (1974-2011, n?=?113 MPNST patients) and 39 published studies on MPNST patients (n?=?916) were analyzed for age of onset differences between sexes and between NF-1 and spontaneous MPNST patients.Our murine model showed gender-based differences in MPNST onset, with males developing MPNST significantly earlier than females (142 vs. 162 days, p?=?0.015). In the UCLA patient population, males also developed MPNST earlier than females (median age 35 vs. 39.5 years, p?=?0.048). Patients with NF-1-associated MPNST had significantly earlier age of onset compared to spontaneous MPNST (median age 33 vs. 39 years, p?=?0.007). However, expanded analysis of 916 published MPNST cases revealed no significant age difference in MPNST onset between males and females. Similar to the UCLA dataset, patients with NF-1 developed MPNST at a significantly younger age than spontaneous MPNST patients (p?

SUBMITTER: Shurell E 

PROVIDER: S-EPMC4237782 | biostudies-other | 2014 Nov

REPOSITORIES: biostudies-other