Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor

Abstract

With this, the largest documented Chinese cohort, our data supply powerful Chinese evidence of the prognostic role of tumor size and NF1 status in MPNST.

Tumors of the peripheral nervous system: analysis of prognostic factors in a series with long-term follow-up and review of the literature

“…Moreover, we found that a subtotal resection was associated with a higher risk of tumor recurrence. These data are in agreement with previous observations that the extent of resection affects the duration of PFS and OS 2,10,[19][20][21]42,52,56 and the risk of recurrence. 6,9,37,39,40,57 Considering the risk of recurrence in the present series, the variables that emerged as independent prognostic factors were a history of NF and a diagnosis of MPNST or PNNST.…”

“…In our opinion, the main strengths of this study are the long follow-up, the large number of patients/tumors, and the use of multivariate analysis (regression analysis). In fact, when looking at the literature (see Tables 2 and 3) we found only 3 studies with a duration of follow-up longer than in our series, 10,21,40 3 papers with a greater number of patients and wider tumors than our study, 2,14,35 and only a few articles analyzing surgical results and possible prognostic factors using a multivariate analysis. 2,5,20,21,30,31,42,57 The main limit of this study is the retrospective nature of the data, which could affect the results despite the strict statistical analysis.…”

“…Although Wanebo et al 56 found that NF was associated with better PFS in patients with MPNSTs, data in the literature are otherwise consistent with the finding that a history of NF is associated with a worse prognosis in terms of PFS and OS, 3,21,40,48,56 and recurrence risk. 3,6 Moreover, data from the literature also support our finding of a strong association between malignant histology and worse prognosis.…”

“…The role of tumor size has been the object of extensive investigation in the literature and this factor has been found to be a good prognostic indicator for PFS and OS in various series. 2,5,10,21,30,44,56 Moreover, smaller tumor size has been found to be associated with a lower incidence of postoperative deficit, 36 local recurrence, 9,40 and possible metastasis in cases of MPNST. 57 The extent of resection is another factor frequently analyzed in previous series.…”

Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas

“…For individuals harboring a malignant peripheral nerve sheath tumor, despite adjuvant chemotherapy and radiation, the overall prognosis is poor, with 5-year disease-specific survival ranging between 35 and 50%. [2][3][4] In a large cohort, 41% of patients who initially presented with a localized malignant peripheral nerve sheath tumor developed distant metastasis in a median time of 12 months. 2 Cellular schwannoma initially described by Woodruff et al 5 is an uncommon, but wellrecognized, benign nerve sheath tumor that often presents diagnostic challenges.…”