» Articles » PMID: 3082508

Malignant Peripheral Nerve Sheath Tumors. A Clinicopathologic Study of 120 Cases

Overview
Journal Cancer
Publisher Wiley
Specialty Oncology
Date 1986 May 15
PMID 3082508
Citations 405
Authors
Affiliations
Soon will be listed here.
Abstract

A review was done of 120 cases of malignant peripheral nerve sheath tumor (MPNST) seen during a 71-year period. Of the 120 patients, 52 were males and 68 were females with a mean age at diagnosis of 35.3 years; 12 patients were younger than 20 years. The series included 62 (52%) patients with neurofibromatosis, 13 (11%) with postradiation sarcomas, and 19 (16%) with metaplastic foci. The incidence of MPNST arising in neurofibromatosis was 4.6% in the current series and 0.001% in the general clinic population. Tumors greater than 5 cm and the presence of neurofibromatosis adversely affected the prognosis (P less than 0.05). When both features were present, survival was greatly decreased. Patients with tumor in the extremities did better than those with head or neck lesions. Metaplastic foci or previous radiation at the tumor site did not alter the prognosis. Each tumor was graded 1 to 4 on the basis of cellularity, pleomorphism, mitotic index, and necrosis. No significant correlation was noted between survival and either grade or mitotic rate. Survival was improved when total rather than subtotal resection was done. This was most marked in patients with a small lesion, which may reflect the difficulty in adequately excising large tumors. Adjuvant radiation or chemotherapy did not appear to affect survival. The MPNST is an aggressive uncommon neoplasm, and large tumor size, the presence of neurofibromatosis, and total resection are the most important prognostic indicators.

Citing Articles

Surgical treatment of rare peripheral nerve lesions: long-term outcomes and quality of life.

Savic A, Lepic M, Grujic J, Micic A, Stojiljkovic A, Putra G Front Oncol. 2025; 14:1476019.

PMID: 40078480 PMC: 11897479. DOI: 10.3389/fonc.2024.1476019.


Adulthood consequences of spinal deformity surgeries in neurofibromatosis type 1 patients.

Tsukamoto A, Uno K, Suzuki T, Ito M, Nakashima K, Kakutani K BMC Musculoskelet Disord. 2025; 26(1):113.

PMID: 39905398 PMC: 11792234. DOI: 10.1186/s12891-024-08218-y.


A Sequencing Overview of Malignant Peripheral Nerve Sheath Tumors: Findings and Implications for Treatment.

Xiao K, Yang K, Hirbe A Cancers (Basel). 2025; 17(2).

PMID: 39857962 PMC: 11763529. DOI: 10.3390/cancers17020180.


Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review.

Skorka P, Kordykiewicz D, Ilkow A, Ptaszynski K, Wojcik J, Skorka W Life (Basel). 2025; 14(12.

PMID: 39768355 PMC: 11680011. DOI: 10.3390/life14121648.


Extraneural Soft Tissue Perineurioma: A Report of a Rare Case of Peripheral Nerve Sheath Tumor.

Arunachalam Ganesh R, Selvaraj K, Chandran S, Yesuvadiyan J Cureus. 2024; 16(11):e73169.

PMID: 39651020 PMC: 11624966. DOI: 10.7759/cureus.73169.