Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review
Abstract Background Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature. Methods We retrospec...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-10-01
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| Series: | World Journal of Surgical Oncology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12957-023-03227-y |
| _version_ | 1797452478213521408 |
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| author | Ajmain Chowdhury Juan Vivanco-Suarez Nahom Teferi Alex Belzer Hend Al-Kaylani Meron Challa Sarah Lee John M. Buatti Patrick Hitchon |
| author_facet | Ajmain Chowdhury Juan Vivanco-Suarez Nahom Teferi Alex Belzer Hend Al-Kaylani Meron Challa Sarah Lee John M. Buatti Patrick Hitchon |
| author_sort | Ajmain Chowdhury |
| collection | DOAJ |
| description | Abstract Background Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature. Methods We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan–Meier survival analyses were performed. Results Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15–67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence. Conclusions Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival. |
| first_indexed | 2024-03-09T15:09:16Z |
| format | Article |
| id | doaj.art-dcd9cc788e10478192d07a60ddf5d650 |
| institution | Directory Open Access Journal |
| issn | 1477-7819 |
| language | English |
| last_indexed | 2024-03-09T15:09:16Z |
| publishDate | 2023-10-01 |
| publisher | BMC |
| record_format | Article |
| series | World Journal of Surgical Oncology |
| spelling | doaj.art-dcd9cc788e10478192d07a60ddf5d6502023-11-26T13:31:09ZengBMCWorld Journal of Surgical Oncology1477-78192023-10-0121111010.1186/s12957-023-03227-ySurgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature reviewAjmain Chowdhury0Juan Vivanco-Suarez1Nahom Teferi2Alex Belzer3Hend Al-Kaylani4Meron Challa5Sarah Lee6John M. Buatti7Patrick Hitchon8Carver College of Medicine, University of IowaDepartment of Neurology, University of IowaNeurosurgery and Biomedical Engineering, Department of Neurosurgery, University of Iowa Hospitals and ClinicsCarver College of Medicine, University of IowaCarver College of Medicine, University of IowaCarver College of Medicine, University of IowaNeurosurgery and Biomedical Engineering, Department of Neurosurgery, University of Iowa Hospitals and ClinicsDepartment of Radiation Oncology, University of IowaNeurosurgery and Biomedical Engineering, Department of Neurosurgery, University of Iowa Hospitals and ClinicsAbstract Background Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature. Methods We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan–Meier survival analyses were performed. Results Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15–67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence. Conclusions Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival.https://doi.org/10.1186/s12957-023-03227-yMalignant peripheral nerve sheath tumorsMPNSTMesenchymal tumorsCraniospinal axisMalignant Triton tumor |
| spellingShingle | Ajmain Chowdhury Juan Vivanco-Suarez Nahom Teferi Alex Belzer Hend Al-Kaylani Meron Challa Sarah Lee John M. Buatti Patrick Hitchon Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review World Journal of Surgical Oncology Malignant peripheral nerve sheath tumors MPNST Mesenchymal tumors Craniospinal axis Malignant Triton tumor |
| title | Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review |
| title_full | Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review |
| title_fullStr | Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review |
| title_full_unstemmed | Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review |
| title_short | Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review |
| title_sort | surgical management of craniospinal axis malignant peripheral nerve sheath tumors a single institution experience and literature review |
| topic | Malignant peripheral nerve sheath tumors MPNST Mesenchymal tumors Craniospinal axis Malignant Triton tumor |
| url | https://doi.org/10.1186/s12957-023-03227-y |
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