The effect of surgical margins on outcomes for low grade MPNSTs and atypical neurofibroma

Bernthal, Nicholas M.; Putnam, Angelica R.; Jones, Kevin B.; Viskochil, David; Randall, R. Lor

doi:10.1002/jso.23736

Cited by 45 publications

(30 citation statements)

References 16 publications

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“…3,6 Moreover, data from the literature also support our finding of a strong association between malignant histology and worse prognosis. 11,13,14,19,24,29,35,39,59 PNNST includes different entities, both benign and malignant, that, in our series, showed a higher risk of recurrence compared with BPNST.…”

Section: Discussionsupporting

confidence: 83%

“…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. When a recurrence is diagnosed at follow-up MRI, our initial approach is "wait and see," performing an MRI study every 3-6 months to detect a lesion increase.…”

Section: Discussionmentioning

confidence: 69%

See 1 more Smart Citation

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: Discussionsupporting

confidence: 83%

Section: Discussionmentioning

confidence: 69%

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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“…6,21 Select studies have demonstrated more favorable outcomes in low-grade MPNSTs compared to high grade MPNSTs; therefore, we reviewed patients with low-grade MPNSTs independently ( n =12). 2,22 Patients in the high-grade cohort who did not receive surgical intervention as part of their primary treatment ( n = 22) or who received subtotal R2 resection ( n = 23) were evaluated independently owing to their anticipated poor prognosis. Only patients who had primary tumors in the absence of synchronous metastasis at diagnosis were included in the outcomes analysis (Figure 1).…”

Section: Methodsmentioning

confidence: 99%

“…1,7,19,23,25 However, some small studies have demonstrated superior survival in patients with low-grade variants of MPNST, suggesting a benign natural history in these rare lesions compared to their high-grade counterparts. 2,22 Whether NF1 is adversely associated with survival remains controversial. 14,19,23 Additionally, studies comparing outcomes of radiation-associated (RT-associated) MPNST to other subtypes are sparse as a consequence of their rarity.…”

Section: Introductionmentioning

confidence: 99%

Patterns of recurrence and survival in sporadic, neurofibromatosis Type 1–associated, and radiation-associated malignant peripheral nerve sheath tumors

Watson¹,

Sannaa

Kivlin

et al. 2017

JNS

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Structured Abstract Objective MPNSTs are an aggressive group of soft tissue sarcomas that can arise sporadically, in the context of NF1, or at a site of prior irradiation. Large series profiling the features and outcomes of sporadic, neurofibromatosis type 1 (NF1)-associated, and radiation (RT)-associated malignant peripheral nerve sheath tumor (MPNST) are limited. The goal of this study was to elucidate differences between MPNST etiologies in a large single-institution retrospective study. Methods Patients (n = 317) were identified through our institutional tumor registry. Clinicopathologic features were retrospectively collected. Features were compared among MPNST subtypes for patients who had sufficient clinical history (n = 289), and clinicopathologic features were used to identify adverse predictors of recurrence and survival outcomes. Results Five-year local recurrence-free survival (LRFS), distant recurrence-free survival (DRFS), and disease-specific survival (DSS) estimates were 56.6%, 49.6%, and 53.6% for the high-grade MPNST cohort, respectively. Five-year DSS was lower in NF1-associated and RT-associated compared to sporadic MPNST (48.7%, 40.9%, and 63.0%, respectively; p = 0.140). RT-associated MPNST had worse LRFS than sporadic and NF1-associated subtypes (p = 0.047). Truncally located tumors, positive surgical margins, local recurrence, and metastasis were predictors of adverse DSS in multivariate analysis. Conclusion RT-associated MPNSTs demonstrate poorer local recurrence-free and disease-specific survival than sporadic and NF1-associated tumors. NF1-associated MPNSTs may have worse survival outcomes owing to large tumor size, compromising truncal location, and lower rate of negative resection margins compared to sporadic tumors.

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“…Bernthal et al published a series of 23 patients in which the disease-specific free survival was 100% regardless of surgical margin[19]. What is unique about our cases is that they demonstrated an exclusive pattern of leptomeningeal spread without involvement of any other organs, a rare occurrence with Schwann cell neoplasms.…”

Section: Discussionmentioning

confidence: 75%

Low-grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings

et al. 2017

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Leptomeningeal dissemination of low grade Schwann cell neoplasms is an exceptionally rare occurrence, and has not been well documented in the literature. We encountered two cases of leptomeningeal dissemination of low grade Schwann cell neoplasms. Patient 1 was a 63-year-old woman with NF1 and a progressive low grade MPNST developing from a diffuse/plexiform orbital neurofibroma that arose in childhood. The neoplasm demonstrated local and leptomeningeal dissemination intracranially leading to the patient’s death. There was partial loss of H3K27 tri-methylation, p16 and collagen IV. Patient 2 was a 60-year-old man without NF1 who presented with cranial nerve symptoms and a disseminated neoplasm with a Schwann cell phenotype. The neoplasm stabilized after irradiation and chemotherapy but the patient died of medical complications. Autopsy findings documented disseminated leptomeningeal disease in the intracranial and spinal compartment. H3K27M tri-methylation was preserved. The clinicopathologic and autopsy findings are studied and presented, and the literature is reviewed.

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The effect of surgical margins on outcomes for low grade MPNSTs and atypical neurofibroma

Cited by 45 publications

References 16 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

Patterns of recurrence and survival in sporadic, neurofibromatosis Type 1–associated, and radiation-associated malignant peripheral nerve sheath tumors

Low-grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings

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