Skip to main content
Log in

Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1

Report of 4 cases

  • Case Report
  • Published:
Pathology Oncology Research

Abstract

We report 4 cases of malignant peripheral nerve sheath tumors (MPNST) with neurofibromatosis type 1 (NF1). Mean age was 29.5. Two of them had a family history. Three of them were male. All of them had enlarging mass and pain in the background of neurofibromas. Locations were popliteal, thigh and forearm. The masses were greater than 5 cm in diameter in each case. In two cases the mass was showing continuity with a nerve. One patient had a nonossifying fibroma as well as a MPNST. Wide excision and radiotherapy were applied to three of the patients. One of them did not take any therapy after surgical resection. Two of the patients died of lung metastases after a mean period of 12.5 months. In a majority of NF1 patients MPNST emerges from a preexisting neurofibroma. The patients with NF1 are at greatest risk for developing sarcomas, so they should be followed closely.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Blatt J, Jaffe R, Deutsch M, et al: Neurofibromatosis and childhood tumors. Cancer 57: 1225–1229, 1986

    Article  PubMed  CAS  Google Scholar 

  2. D’Agostina AN, Soule EH, Miller RH: Sarcomas of the peripheral nerves and soft tissues associated with multiple neurofi-bromatosis (Von Recklinghausen’s Disease). Cancer 16: 1015–1027, 1963

    Article  Google Scholar 

  3. Ducatman BS, Scheithauer BW: Malignant peripheral nerve sheath tumors with divergent differentiation. Cancer 54: 1049–1057, 1984

    Article  PubMed  CAS  Google Scholar 

  4. Ducatman BS, Scheithauer BW, Piepgras DG, et al: Malignant peripheral nerve sheath tumors a clinicopathologic study of 120 cases. Cancer 57: 2006–2011, 1986

    Article  PubMed  CAS  Google Scholar 

  5. Ferner RE, Lucas JD, O’Doherty MJ, et al: Evaluation of 18 Fluorodeoxyglucose positron emission tomography (18 FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry 68: 353–357, 2000

    Article  PubMed  CAS  Google Scholar 

  6. Halliday AL, Sobel RA, Martuza RL: Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2. J Neurosurg 74: 248–253, 1991

    Article  PubMed  CAS  Google Scholar 

  7. Hajdu SI: Peripheral nerve sheath tumors, histogenesis, classification and prognosis. Cancer 72: 3549–3552, 1993

    Article  PubMed  CAS  Google Scholar 

  8. Hruban RH, Shiu MH, Senie RT, et al: Malignant peripheral nerve sheath tumors of the buttock and lower extremity, A study of 43 Cases. Cancer 66: 1253–1265, 1990

    Article  PubMed  CAS  Google Scholar 

  9. King AA, DeBaun MR, Riccardi VM, et al: Malignant peripheral nerve sheath tumors in neurofibromatosis 1. Am J Med Genet 93: 388–392, 2000

    Article  PubMed  CAS  Google Scholar 

  10. Nussbaum RL, McInnes RR, Willard HF: Genetics In Medicine. Genetics and Cancer, W. B. Saunders Company: Philadelphia, 2001.

    Google Scholar 

  11. Rasmussen SA, Overman J, Thomson SAM, et al: Chromosome 17 loss-of-heterozygosity studies in benign and malignant tumors in neurofibromatosis type 1. Genes, Chromosomes, Cancer 28:425–431, 2000

    CAS  Google Scholar 

  12. Schmidt H, Würl P, Taubert H, et al: Genomic imbalances of 7p and 17q in malignant peripheral nerve sheath tumors are clinically relevant. Genes, Chromosomes, Cancer 25: 205–211, 1999

    CAS  Google Scholar 

  13. Topsakal CD, Akdemir I, Tiftikci M, et al: Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1. Neurol Med Chir (Tokyo) 41: 551–555, 2001

    Article  CAS  Google Scholar 

  14. Wanebo JE, Malik JM, VandenBerg SR, et al: Malignant peripheral nerve sheath tumors, A clinicopathologic study of 28 cases. Cancer 71: 1247–1253, 1993

    Article  PubMed  CAS  Google Scholar 

  15. Weiss SW, Goldblum JR: Benign tumors of the peripheral nerves. Malignant tumors of the peripheral nerves. Enzinger and Weiss’s Soft Tissue Tumors. Mosby, St Louis, 2001.

    Google Scholar 

  16. Wick MR, Swanson PE, Scheithauer BW, et al: Malignant peripheral nerve sheath tumor. An immunohistochemical study of 62 cases. Am J Clin Pathol 87: 425–433, 1987

    PubMed  CAS  Google Scholar 

  17. Woodruff JM, Christensen WN: Glandular peripheral nerve sheath tumors. Cancer 72: 3618–3628, 1993

    Article  PubMed  CAS  Google Scholar 

  18. Woodruff JM: Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet (Semin Med Genet) 89:23–30, 1999

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Lora Esberk Ates.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bilgic, B., Ates, L.E., Demiryont, M. et al. Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1. Pathol. Oncol. Res. 9, 201–205 (2003). https://doi.org/10.1007/BF03033740

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03033740

Keywords

Navigation