High Grade Malignant Peripheral Nerve Sheath Tumors: Outcome of 62 Patients with Localized Disease and Review of the Literature

Abstract

Malignant peripheral nerve sheath tumours (MPNST) are rare sarcomas with one of the poorest prognoses of all the soft tissue sarcomas. Information about adjuvant treatment is scarce and not homogeneous for this diagnosis. We analyzed retrospectively the outcome of patients with localized high grade MPNST admitted to our institute from 1969 to 2008. A review of the literature is also reported. Of 62 evaluable patients, 23 were females and 39 males, median age 39 years (17-71), 22/62 had neurofibromatosis type I. Median follow-up was 54 months (range 12-194). A total of 22/62 are alive; 26 patients had surgery alone, 18 received radiation therapy, 12 received radiation therapy and chemotherapy, and 6 received only adjuvant chemotherapy. The 5-year disease-free survival was 30% and 5-year overall survival was 38%. A positive trend for adjuvant radiation, but not for chemotherapy was observed according to univariate analysis only for disease-free survival and overall survival. Multivariate analysis indicated that primary site, size and surgical margins remained significant for disease-free survival and only site and size were significant for overall survival. New drugs employed successfully in advanced mpNSt should be employed also in the adjuvant setting.

Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1

“…Parts of the patient data have been presented in previous publications. 24,32–34 The biobanks and projects were approved according to national legislation. …”

Expression profiling of 519 kinase genes in matched malignant peripheral nerve sheath tumor/plexiform neurofibroma samples is discriminatory and identifies mitotic regulators BUB1B, PBK and NEK2 as overexpressed with transformation

“…1,3,8,15,16 For these neurofibromatosis type 1 patients, this often becomes a life-limiting aspect of their condition as the 5-year survival rate of malignant peripheral nerve sheath tumor is B40%. 10,17,18 The biological factors underlying this transformation are still poorly understood. 3 Which of a patient's often numerous plexiform neurofibromas, if any, will transform into a malignant peripheral nerve sheath tumor cannot be predicted.…”

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

“…Ten studies [13,14,17,22,24,25,[29][30][31]38] evaluated tumor size (the large vs. the small) as a risk factor for OS. Values of I 2 = 44.4% and p= 0.063 were obtained after the HR values of OS were merged, indicating that heterogeneity existed.…”

“…Radical therapy surgery combined with adjuvant therapy have been applied past decades. However, the prognosis for MPNST patients remains truly dissatisfactory, with 5-year overall survival (OS) rate of 15%-66% [1,[4][5][6][7][8][9][10], 5-year event free survival (EFS) of 24%-53% [6,[11][12][13][14][15][16][17], and local recurrence (LR) rate of 20% to 85.7% [2,4,[18][19][20]. It was obvious that the reported rates varied widely in different literatures.…”