The Association of Metastasis Pattern and Management of Metastatic Disease with Oncological Outcomes in Patients with Malignant Peripheral Nerve Sheath Tumors: A Multicenter Cohort Study

Purpose: This multicenter cohort study aimed to identify clinicopathologic and treatment-related factors associated with the development of distant metastasis (DM) and with overall survival (OS) after DM diagnosis in patients with malignant peripheral nerve sheath tumors (MPNST). Methods: All patients diagnosed with primary MPNST from 1988 to 2019 who were surgically treated for the primary tumor were included. Multivariable Cox regression analyses were performed to identify factors associated with DM and OS after DM diagnosis. Results: A total of 383 patients were included in this analysis, of which 150 developed metastatic disease. No differences in clinicopathologic characteristics and clinical outcome were found between patients with synchronous and metachronous DM. Neurofibromatosis type 1 (NF1), high grade, tumor size, triton and R2 resections were independent risk factors for the development of DM. NF1 and more than two metastasis sites were independently associated with worse OS after DM diagnosis. Metastasectomy, chemotherapy and the metastatic site category ‘other’ were associated with prolonged survival after DM diagnosis. Conclusion: This analysis provides important insights into clinicopathologic and treatment factors associated with outcomes in metastatic MPNST. Moreover, NF1-status is associated with a higher risk of DM; it is also independently associated with worse survival in metastatic MPNST.

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SURG-04. THE CLINICAL COURSE AND ROLE OF SURGERY IN PEDIATRIC MALIGNANT PERIPHERAL NERVE SHEATH TUMORS: A DATABASE STUDY

Neuro-Oncology ◽

10.1093/neuonc/noab196.774 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi195-vi195

Author(s):

Victor Lu ◽

Shelly Wang ◽

David Daniels ◽

Robert Spinner ◽

Allan Levi ◽

...

Keyword(s):

Overall Survival ◽

Radiation Therapy ◽

Peripheral Nerve ◽

Cox Regression ◽

Clinical Course ◽

The Body ◽

National Database ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Abstract BACKGROUND Malignant peripheral nerve sheath tumors (MPNST) are rare tumors found throughout the body, with their clinical course in children still to be completely understood. Correspondingly, this study aimed to determine survival outcomes and specific clinical predictors of survival in this demographic from a large national database. METHODS All MPNST patients aged ≤18 year in the U.S. National Cancer Database (NCDB) between 2005-2016 were retrospectively reviewed. Data were summarized and overall survival (OS) was modeled using Kaplan-Meier and Cox regression analyses. RESULTS A total of 251 pediatric MPNST cases were identified. Overall, mean age at diagnosis was 13.1 years (range, 1-18) with there being 132 (53%) females and 119 (47%) males. There were 84 (33%) MPNSTs located in extremities, 127 (51%) less than 1cm in size, and 22 (9%) have metastasis at diagnosis. In terms of treatment, surgery was pursued in 187 (74%) patients, chemotherapy in 116 (46%) patients, and radiation therapy in 129 (61%) patients. Five-year overall survival was estimated to be 52% (95% CI, 45-59%), and with median survival of 64 months (range, 36-136). Multivariate regression revealed older age (HR 1.10, P< 0.01), with metastases at time of diagnosis (HR 2.14, P=0.01), and biopsy only (HR 2.98, P< 0.01) all significantly and independently predicted shorter overall survival. Chemotherapy and radiation therapy were not statistically significant. CONCLUSIONS Pediatric MPNSTs are rare malignant entities, and only approximately half of patients will survive to five years. There remains a clear survival benefit in pursuing maximal safe resection in these patients. As such, judicious workup with meticulous surgical resection by an expert team should then be considered the standard of care for these tumors in children.

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Neurofibromatosis‐associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study

Pediatric Blood & Cancer ◽

10.1002/pbc.28138 ◽

2019 ◽

Vol 67 (4) ◽

Cited By ~ 5

Author(s):

Enrico Martin ◽

J. Henk Coert ◽

Uta E. Flucke ◽

Willem‐Bart M. Slooff ◽

Michiel A.J. de Sande ◽

...

Keyword(s):

Cohort Study ◽

Peripheral Nerve ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Worse Prognosis

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Need for arthrodesis following facetectomy for spinal peripheral nerve sheath tumors: an institutional experience and review of the current literature

Journal of Neurosurgery Spine ◽

10.3171/2019.1.spine181057 ◽

2019 ◽

Vol 31 (1) ◽

pp. 112-122 ◽

Cited By ~ 1

Author(s):

Mohammed Adeeb Sebai ◽

Panagiotis Kerezoudis ◽

Mohammed Ali Alvi ◽

Jang Won Yoon ◽

Robert J. Spinner ◽

...

Keyword(s):

Peripheral Nerve ◽

Care Center ◽

Univariate Analysis ◽

Tertiary Care ◽

Multivariable Analysis ◽

Factors Associated ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Increased Risk

OBJECTIVESpinal peripheral nerve sheath tumors (PNSTs) are a group of rare tumors originating from the nerve and its supporting structures. Standard surgical management typically entails laminectomy with or without facetectomy to gain adequate tumor exposure. Arthrodesis is occasionally performed to maintain spinal stability and mitigate the risk of postoperative deformity, pain, or neurological deficit. However, the factors associated with the need for instrumentation in addition to PNST resection in the same setting remain unclear.METHODSAn institutional tumor registry at a tertiary care center was queried for patients treated surgically for a primary diagnosis of spinal PNST between 2002 and 2016. An analysis focused on patients in whom a facetectomy was performed during the resection. The addition of arthrodesis at the index procedure comprised the primary outcome. The authors also recorded baseline demographics, tumor characteristics, and surgery-related variables. Logistic regression was used to identify factors associated with increased risk of fusion surgery.RESULTSA total of 163 patients were identified, of which 56 (32 had facetectomy with fusion, 24 had facetectomy alone) were analyzed. The median age was 48 years, and 50% of the cohort was female. Age, sex, and race, as well as tumor histology and size, were evenly distributed between patients who received facetectomy alone and those who had facetectomy and fusion. On univariate analysis, total versus subtotal facetectomy (OR 9.0, 95% CI 2.01–64.2; p = 0.009) and cervicothoracic versus other spinal region (OR 9.0, 95% CI 1.51–172.9; p = 0.048) were significantly associated with increased odds of performing immediate fusion. On multivariable analysis, only the effect of total facetectomy remained statistically significant (OR 6.75, 95% CI 1.47–48.8; p = 0.025).CONCLUSIONSThe authors found that total facetectomy and cervicothoracic involvement may be highly associated with the need for concomitant arthrodesis at the time of index surgery. These findings may help surgeons to determine the best surgical planning for patients with PNST.

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Treatment and survival differences across tumor sites in malignant peripheral nerve sheath tumors: a SEER database analysis and review of the literature

Neuro-Oncology Practice ◽

10.1093/nop/npy025 ◽

2018 ◽

Vol 6 (2) ◽

pp. 134-143 ◽

Cited By ~ 3

Author(s):

Enrico Martin ◽

Ivo S Muskens ◽

J H Coert ◽

Timothy R Smith ◽

Marike L D Broekman

Keyword(s):

Peripheral Nerve ◽

Cox Regression ◽

Treatment Modalities ◽

Spinal Tumors ◽

Seer Database ◽

Intracranial Tumors ◽

Large Tumor ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

AbstractBackgroundCurrently, literature is scarce on differences across all possible tumor sites in malignant peripheral nerve sheath tumors (MPNSTs). To determine differences in treatment and survival across tumor sites and assess possible predictors for survival, we used the Surveillance, Epidemiology, and End Results (SEER) database.MethodsMPNST cases were obtained from the SEER database. Tumor sites were recoded into: intracranial, spinal, head and neck (H&N), limbs, core (thorax/abdomen/pelvis), and unknown site of origin. Patient and tumor characteristics, treatment modalities, and survival were extracted. Overall survival (OS) was assessed using univariable and multivariable Cox regression hazard models. Kaplan-Meier survival curves were constructed per tumor site for OS and disease-specific survival (DSS).ResultsA total of 3267 MPNST patients were registered from 1973 to 2013; 167 intracranial (5.1%), 119 spinal (3.6%), 449 H&N (13.7%), 1022 limb (31.3%), 1307 core (40.0%), and 203 unknown (6.2%). The largest tumors were found in core sites (80.0 mm, interquartile range [IQR]: 60.0-115.0 mm) and the smallest were intracranial (37.4 mm, IQR: 17.3-43.5 mm). Intracranial tumors were least frequently resected (58.1%), whereas spinal tumors were most often resected (83.0%). Radiation was administered in 35.5% to 41.8%. Independent factors associated with decreased survival were: older age, male sex, black race, no surgery, partial resection, large tumor size, high tumor grade, H&N site, and core site (all P < .05). Intracranial and pediatric tumors show superior survival (both P < .05). Intracranial tumors show superior OS and DSS curves, whereas core tumors have the worst (P < .001).ConclusionSuperior survival is seen in intracranial and pediatric MPNSTs. Core and H&N tumors have a worse prognosis.

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