CHARACTERIZATION AND CHEMOSENSITIVITY OF 2 HUMAN-MALIGNANT PERIPHERAL-NERVE SHEATH TUMOR-CELL LINES DERIVED FROM A PATIENT WITH NEUROFIBROMATOSIS TYPE-1

Two new cell lines, designated NMS-2 and NMS-2PC, were established in vitro from a malignant peripheral nerve sheath tumour (MPNST) in the r ight thigh and a retroperitoneal lesion of a 30-year-old man with neur ofibromatosis type 1 (NF1). The NMS-2 cell line was derived from the f irst tumour, and the NMS-2PC cell line from a retroperitoneal metastat ic tumour detected 9 months later. Cultured NMS-2 cells showed epithel ioid features, while NMS-2PC cells showed fibroblast-like features. Ho wever, both cell lines were strongly positive for S-100 protein. The t ransplanted NMS-2 and NMS-2PC tumours showed the same histological fea tures typical of MPNST. Chromosomal analysis revealed that only the NM S-2 cells had a t (1;2) chromosomal translocation. Chemosensitivity te sts demonstrated that NMS-2PC cells were far more sensitive than NMS-2 cells to Adriamycin and etoposide, which had been used clinically. Al l-trans-retinoic acid induced a morphological change in NMS-2PC cells so that they were no longer fibroblast-like, but epithelioid cells. We believe the epitheloid components in the MPNST were derived from typi cal spindle cells.