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

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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Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1

Case Reports in Oncological Medicine ◽

10.1155/2014/843749 ◽

2014 ◽

Vol 2014 ◽

pp. 1-6 ◽

Cited By ~ 4

Author(s):

Apostolos Pourtsidis ◽

Dimitrios Doganis ◽

Margarita Baka ◽

Despina Bouhoutsou ◽

Maria Varvoutsi ◽

...

Keyword(s):

Radiation Therapy ◽

Peripheral Nerve ◽

Neurofibromatosis Type 1 ◽

Soft Tissue Sarcomas ◽

Neurofibromatosis Type ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Increased Risk

Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department.Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them.Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear.

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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

Cancers ◽

10.3390/cancers13205115 ◽

2021 ◽

Vol 13 (20) ◽

pp. 5115

Author(s):

Ibtissam Acem ◽

Enrico Martin ◽

Winan van Houdt ◽

Michiel van de Sande ◽

Dirk Grünhagen ◽

...

Keyword(s):

Cohort Study ◽

Peripheral Nerve ◽

Metastatic Disease ◽

Cox Regression ◽

Multicenter Cohort Study ◽

Factors Associated ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Multicenter Cohort ◽

Nerve Sheath

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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Malignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient

Neurosurgery ◽

10.1093/neuros/nyx368 ◽

2017 ◽

Vol 83 (1) ◽

pp. 38-42 ◽

Cited By ~ 9

Author(s):

Andrew T King ◽

Scott A Rutherford ◽

Charlotte Hammerbeck-Ward ◽

Simon K Lloyd ◽

Simon R Freeman ◽

...

Keyword(s):

Peripheral Nerve ◽

Vestibular Schwannoma ◽

Neurofibromatosis Type ◽

Neurofibromatosis Type 2 ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Increased Risk

Abstract BACKGROUND The published literature suggests that malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in neurofibromatosis type 2 (NF2). A recent review based on incidence data in North America showed that 1 per 1000 cerebellopontine angle nerve sheath tumors were malignant. OBJECTIVE To determine whether MPNST occurred spontaneously in NF2 by reviewing our NF2 database. METHODS The prospective database consists of 1253 patients with NF2. One thousand and nine are known to be alive at last follow-up. The presence and laterality/pathology of vestibular schwannoma at diagnosis and last follow-up was sought. RESULTS There were no cases of spontaneous MPNST with 2114 proven (n = 1150) and presumed benign (n = 964) vestibular schwannomas found. Two patients had developed MPNST (1 presumed) after having previously undergone stereotactic radiosurgery for a vestibular schwannoma. CONCLUSION In this series, and from the literature, malignant transformation of a vestibular schwannoma was not a feature of NF2 in the unirradiated patient. NF2 patients should not be told that they have an increased risk of malignant change in a vestibular schwannoma unless they undergo radiation treatment. However, very much larger datasets are required before it can be determined whether there is any association between NF2 and MPNST in the unirradiated patient.

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The Surgical Management of Symptomatic Peripheral Nerve Sheath Tumors

Neurosurgery ◽

10.1227/01.neu.0000367636.91555.70 ◽

2010 ◽

Vol 66 (4) ◽

pp. 833-840 ◽

Cited By ~ 45

Author(s):

Allan D. Levi ◽

Andrew L. Ross ◽

Esteban Cuartas ◽

Rabah Qadir ◽

H. Thomas Temple

Keyword(s):

Peripheral Nerve ◽

Surgical Management ◽

Tumor Size ◽

Motor Deficit ◽

Benign Tumors ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Neurologic Deficits ◽

Increased Risk

Abstract OBJECTIVE To determine the clinical presentation and morbidity of the surgical management of peripheral nerve sheath tumors (PNSTs). METHODS We performed a retrospective chart review of surgically treated PNSTs at the University of Miami between 1991 and 2008. RESULTS There were a total of 140 cases, including 87 schwannomas, 34 neurofibromas, and 19 malignant peripheral nerve sheath tumors (MPNSTs). The average age of the total study group was 49.0 years; it was significantly lower for patients with neurofibroma. There was a high correlation between neurofibroma tumors and neurofibromatosis-1. Most patients with benign tumors presented with a painful mass, paresthesias, or numbness without significant weakness. Patients who had previously undergone attempted resections and preoperative biopsy had a significantly increased risk (41%) for developing postoperative neurologic deficits when compared with patients who presented with de novo tumors (15%). Intraoperative monitoring appeared to reduce the risk of postoperative motor deficit, particularly in neurofibromas. Most MPNSTs (>80%) were diagnosed at stage IIB or higher and had a combined mortality rate of 31.6% at 78 months. Tumor size was the best predictor of adverse outcome, as all MPNST mortalities occurred in patients with a tumor size of more than 7 cm. CONCLUSION PNSTs are a heterogeneous group of lesions. Benign tumors respond well to marginal excision, whereas MPNSTs are aggressive sarcomas that require multimodal management. There was a significantly increased risk of postoperative neurologic deficits in patients who had undergone a previous biopsy, and thus tertiary referral without biopsy is recommended when a PNST is suspected.

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Clinical, radiological, and pathological features of 26 intracranial and intraspinal malignant peripheral nerve sheath tumors

Journal of Neurosurgery ◽

10.3171/2013.5.jns122119 ◽

2013 ◽

Vol 119 (3) ◽

pp. 695-708 ◽

Cited By ~ 11

Author(s):

Xiaohui Ren ◽

Junmei Wang ◽

Mengqing Hu ◽

Haihui Jiang ◽

Jun Yang ◽

...

Keyword(s):

Peripheral Nerve ◽

Tumor Recurrence ◽

Univariate Analysis ◽

Progression Free Survival ◽

Pathological Features ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Low Incidence ◽

Total Tumor Removal

Object Intracranial and intraspinal malignant peripheral nerve sheath tumors (MPNSTs) are rarely reported because of their extremely low incidence. Knowledge about these tumors is poor. In this study the authors aimed to analyze the incidence and clinical, radiological, and pathological features of intracranial and intraspinal MPNSTs. Methods Among 4000 cases of intracranial and intraspinal PNSTs surgically treated between 2004 and 2011 at Beijing Tiantan Hospital, cases of MPNST were chosen for analysis and were retrospectively reviewed. To determine which parameters were associated with longer progression-free survival (PFS) and overall survival (OS), statistical analysis was performed. Results Malignant PNSTs accounted for 0.65% of the entire series of intracranial and intraspinal PNSTs. Twenty-four (92.3%) of these 26 MPNSTs were primary. Radiologically, 26.9% (7 of 26) of the MPNSTs were misdiagnosed as nonschwannoma diseases. Twenty-one patients were followed up for 1.5 to 102 months after surgery. Twelve patients experienced tumor recurrence, and median PFS was 15.0 months. The 2- and 3-year PFS rates were 47.7% and 32.7%, respectively. Five patients died of tumor recurrence, and median OS was not available. The 2- and 3-year OS rates were 74.7% and 64.0%, respectively. Univariate analysis revealed that female sex, total tumor removal, and primary MPNSTs were significantly associated with a better prognosis. Multivariate analysis revealed that only total removal was an independent prognostic factor for both PFS and OS. Conclusions Malignant PNST within the skull or spinal canal is a rare neoplasm and is seldom caused by benign schwannomas. Radiologically, intracranial or intraspinal MPNST should be differentiated from meningioma, chordoma, fibrous dysplasia of bone, and ear cancer. Total resection whenever possible is necessary for the prolonged survival of patients, especially males.

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Survival and NF1 Analysis in a Cohort of Orthopedics Patients with Malignant Peripheral Nerve Sheath Tumors

Sarcoma ◽

10.1155/2021/9386823 ◽

2021 ◽

Vol 2021 ◽

pp. 1-6

Author(s):

Daniel K. Knewitz ◽

Colin J. Anderson ◽

William T. Presley ◽

MaryBeth Horodyski ◽

Mark T. Scarborough ◽

...

Keyword(s):

Peripheral Nerve ◽

Survival Rates ◽

Treatment Guideline ◽

Neurofibromatosis Type ◽

Adjuvant Radiation ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Large Deletions ◽

Nerve Sheath ◽

Increased Risk

Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor syndrome in which benign plexiform neurofibromas are at risk of transforming into malignant peripheral nerve sheath tumors (MPNSTs), a very rare soft-tissue sarcoma. The prognosis of patients with MPNSTs is poor, with most studies reporting <50% survival at five years. However, studies evaluating MPNSTs are limited and report heterogeneous results. Because no MPNST-specific evidence-based treatment guideline exists, individual institutional experiences are very informative to the field. The main objective of this study was to investigate and report MPNST prognostic clinical and genetic biomarkers from our institution’s Orthopedics service experience treating 20 cases from 1992 to 2017. Most patients were treated with resection and adjuvant radiation. Extended follow-up, averaging 11.4 years (ranging 1.1 to 25.1), revealed excellent five-year survival rates: 70% for overall and 60% for metastatic disease. An S100 B immunonegative tumor phenotype was associated with a significantly worse outcome than MPNSTs with positive S100 B stain. In addition, NF1 gene mutation analysis was performed on 27 families with NF1 in which at least one affected family member developed MPNSTs. Of the 27 NF1 germline mutations, five were large deletions spanning (or nearly spanning) the gene (18.5%), substantially more than such deletions in NF1 in general, consistent with increased risk of MPNSTs in such cases.

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