Malignant peripheral nerve sheath tumors (MPNST): A SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population

Bates, James E.; Peterson, Carl R.; Dhakal, Sughosh; Giampoli, Ellen; Constine, Louis S.

doi:10.1002/pbc.25149

Cited by 61 publications

(48 citation statements)

References 34 publications

(56 reference statements)

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“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Montano¹,

D’Alessandris²,

D’Ercole³

et al. 2016

JNS

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abbreviatioNs BPNST = benign PNST; MPNST = malignant PNST; NF = neurofibromatosis; OS = overall survival; PFS = progression-free survival; PNNST = peripheral non-neural sheath tumor; PNST = peripheral nerve sheath tumor; VAS = visual analog scale. patients (with 173 surgically treated tumors) had adequate follow-up data available for analysis. Pain was assessed using a visual analog scale (VAS), and motor and sensory function were assessed by means of the Louisiana State University grading system. They also analyzed the relationship between tumor recurrence and patient sex, patient age, diagnosis of neurofibromatosis (NF), tumor histopathology, tumor size, tumor location, and extent of resection (subtotal vs gross-total resection), using univariate and multivariate analyses. results There was a statistically significant improvement in the mean VAS pain score (preoperative 3.96 ± 2.41 vs postoperative 0.95 ± 1.6, p = 0.0001). Motor strength and sensory function were significantly improved after resection of tumors involving the brachial plexus (p = 0.0457 and p = 0.0043, respectively), tumors involving the upper limb (p = 0.0016 and p = 0.0016, respectively), BPNSTs (p = 0.0011 and p < 0.0001, respectively), and tumors with dimensions less than 5 cm (motor strength: p = 0.0187 and p = 0.0021 for ≤ 3 cm and 3-5 cm tumors, respectively; sensory function: p = 0.0003 and p = 0.0001 for ≤ 3 cm and 3-5 cm tumors, respectively). Sensory function showed a statistically significant improvement also in patients who had undergone resection of tumors involving the lower limb (p = 0.0118). Total resection was associated with statistically significant improvement of motor strength (p = 0.0251) and sensory function (p < 0.0001). In univariate analysis, a history of NF (p = 0.0034), a diagnosis of MPNST or PNNST (p < 0.0001), and subtotal resection (p = 0.0042) were associated with higher risk of tumor recurrence. In multivariate analysis (logistic regression analysis), a history of NF (OR 9.28%, 95% CI 1.62-52.94, p = 0.0121) and a diagnosis of MPNST (OR 0.03%, 95% CI 0.002-0.429, p = 0.0098) or PNNST (OR 0.081%, 95% CI 0.013-0.509, p = 0.0077) emerged as independent prognostic factors for tumor recurrence. coNclusioNs A total resection should be attempted in all cases of peripheral nervous system tumors (irrespective of the supposed diagnosis and tumor dimensions) because it is associated with better prognosis in term of functional outcome and overall survival. Moreover, a total resection predicts a lower risk of tumor recurrence. Patients with a history of NF and tumors with malignant histology remain a challenge both for neurosurgeons and oncologists due to higher recurrence rates and the lack of standardized adjuvant therapies.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Montano¹,

D’Alessandris²,

D’Ercole³

et al. 2016

JNS

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“…MPNST is a rare cancer with an incidence of less than 1 in 1 000 000 [26]. The low number of patients also limits the statistical power of the few clinical trials that have been initiated.…”

Section: Discussionmentioning

confidence: 99%

Adjuvant Treatment For Patients With Malignant Peripheral Nerve Sheath Tumours

Kolberg¹,

Smeland²,

Lothe³

2015

Chemotherapy (Los Angel)

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“…Considering the rarity of this tumor, there are few published reports available that describe its clinical management and treatment, particularly in pediatric age …”

Section: Introductionmentioning

confidence: 99%

Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

Bergamaschi

Bisogno

Manzitti

et al. 2017

Pediatric Blood & Cancer

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Our study confirmed the unsatisfactory prognosis for pediatric patients with relapsing MPNST and pointed to a risk-adapted stratification model for the purposes of deciding second-line treatments. For the time being, an aggressive surgical approach seems to be the only effective salvage treatment and should be recommended. New therapeutic approaches are under evaluation with a view to improving current outcomes.

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Malignant peripheral nerve sheath tumors (MPNST): A SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population

Cited by 61 publications

References 34 publications

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

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

Adjuvant Treatment For Patients With Malignant Peripheral Nerve Sheath Tumours

Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

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