New models for MPNST: establishment and comprehensive characterization of two tumor cell lines.

ABSTRACT:

Background

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, invasive, and aggressive soft tissue sarcomas arising from peripheral nerves. They may occur sporadically or in association with Neurofibromatosis type 1 (NF1), in which they are the leading cause of mortality. Currently, there are no effective therapies other than surgery. Therefore, tumor-derived cell lines are essential for testing new therapeutic strategies, especially when used in parallel with in vivo models. In this study, we present two new MPNST cell lines and two patient-derived orthotopic xenograft (PDOX) models from a sporadic (SP-10) and an NF1-related (NF1-18B) MPNST patient to increase the number of available preclinical models for in vitro and in vivo drug testing.

Methods

The cell lines w

SUBMITTER: Ortega-Bertran S 

PROVIDER: S-EPMC12273359 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature