Characterization and chemosensitivity of two human malignant peripheral nerve sheath tumour cell lines derived from a patient with neurofibromatosis type 1

Virchows Arch. 1998 Nov;433(5):435-41. doi: 10.1007/s004280050271.


Two new cell lines, designated NMS-2 and NMS-2PC, were established in vitro from a malignant peripheral nerve sheath tumour (MPNST) in the right thigh and a retroperitoneal lesion of a 30-year-old man with neurofibromatosis type 1 (NF1). The NMS-2 cell line was derived from the first tumour, and the NMS-2PC cell line from a retroperitoneal metastatic tumour detected 9 months later. Cultured NMS-2 cells showed epithelioid features, while NMS-2PC cells showed fibroblast-like features. However, both cell lines were strongly positive for S-100 protein. The transplanted NMS-2 and NMS-2PC tumours showed the same histological features typical of MPNST. Chromosomal analysis revealed that only the NMS-2 cells had a t (1;2) chromosomal translocation. Chemosensitivity tests demonstrated that NMS-2PC cells were far more sensitive than NMS-2 cells to Adriamycin and etoposide, which had been used clinically. All-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 typical spindle cells.

MeSH terms

  • Adult
  • Animals
  • DNA Primers / chemistry
  • Doxorubicin / pharmacology*
  • Drug Screening Assays, Antitumor
  • Etoposide / pharmacology*
  • Female
  • Humans
  • Immunoenzyme Techniques
  • Karyotyping
  • Male
  • Mice
  • Mice, Inbred BALB C
  • Mice, Nude
  • Neoplasm Transplantation
  • Neurofibromatosis 1 / drug therapy
  • Neurofibromatosis 1 / genetics
  • Neurofibromatosis 1 / metabolism
  • Neurofibromatosis 1 / pathology*
  • Peripheral Nervous System Neoplasms / drug therapy
  • Peripheral Nervous System Neoplasms / genetics
  • Peripheral Nervous System Neoplasms / metabolism
  • Peripheral Nervous System Neoplasms / pathology*
  • Polymerase Chain Reaction
  • Retroperitoneal Neoplasms / drug therapy
  • Retroperitoneal Neoplasms / genetics
  • Retroperitoneal Neoplasms / metabolism
  • Retroperitoneal Neoplasms / pathology*
  • S100 Proteins / metabolism
  • Translocation, Genetic / genetics
  • Tretinoin / pharmacology
  • Tumor Cells, Cultured


  • DNA Primers
  • S100 Proteins
  • Tretinoin
  • Etoposide
  • Doxorubicin