Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016

Abstract

ObjectiveTo investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors (MPNST).ResultsA total of 159 patients with MPNST were enrolled in the study. The ratio of male to female was 1.04 to 1. The median age was 40 (range: 5–76) years at the time of diagnosis. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. The median follow-up period was 31.0 (range: 2.0–199.0) months. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival (p < 0.05 for both). 3- and 5-year tumor-free survival rates for 140 completely resected patients were 40.0% and 34.0%, respectively. Multivariate analysis showed that AJCC stage, S-100 and Ki67 staining were independent factors of tumor-free survival (p < 0.05 for all).Materials and MethodsThe clinical data of MPNST patients who were treated at Cancer Institute and Hospital, Chinese Academy of Medical Science from January 1999 to January 2016 was retrospectively reviewed.ConclusionsMPSNT is a highly aggressive tumor with poor prognosis and this study may be useful for prognostic assessment and management decisions. This had been largest documented retrospective study of MPSNT among Chinese populations. Some characteristics were different from those of foreign populations which may suggest the specificity of Chinese patients.

Neurofibromatosis type 1‐associated malignant peripheral nerve sheath tumour carcinomatous pleurisy: an autopsy case

“…Some MPNST patients have no evidence of S‐100 expression, which was attributed to the differentiation of Schwann cells. Furthermore, the absence of S‐100 expression was reported to be an independent prognostic factor and was associated with a 3.24‐fold risk of recurrence or metastasis and a 5.62‐fold risk of mortality compared with positive S‐100 .…”

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.…”

“…Given the rarity of MPNST, there are sporadic published studies [14,16,[21][22][23] reporting the prognosis and related factors. Although poor survival may result from large tumor size [13,24,25], inadequate margin [13,15,24], high-level tumor grade [13][14][15], or presence of distant metastasis [13,25,26], prognostic factors have not reach an agreement in literatures. Besides, few prognostic factors were reported in each study, and the population of these studies was not enough to indicate more detail factors.…”

Integrative analysis identifies candidate tumor microenvironment and intracellular signaling pathways that define tumor heterogeneity in NF1

“…Neurofibromas that occur deeper in the body, including subcutaneous neurofibromas or plexiform neurofibromas (pNFs), occur in 40-60% of NF1 patients and can cause pain and disfigurement among other symptoms [1,4]. Patients with pNFs have a 10% lifetime risk of these tumors developing into malignant peripheral nerve sheath tumors (MPNSTs) which have a 5-year-survival rate of 40-50% [5,6].…”