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 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.

Keywords: Craniospinal axis; MPNST; Malignant Triton tumor; Malignant peripheral nerve sheath tumors; Mesenchymal tumors.

Fig. 1

Case 2. Sagittal (a) and axial (b) post-contrast T1-weighted images showing the tumor at the right side extending from T10 to the sacrum. Post-surgical sagittal (c) and axial (d) post-contrast T1-weighted images showing the resection cavity

Fig. 2

Case 5. Sagittal (a) and axial (b) post-contrast T1-weighted images showing the tumor at the left frontal lobe. Post-surgical sagittal (c) and axial (d) post-contrast T1-weighted images showing the resection cavity. Tumor pathologic sample (e) [hematoxylin–eosin 200 × magnification] showing hypercellularity, fascicles of hyperchromatic spindled cells with pale cytoplasm, and scattered rhabdomyoblastic cells (arrowheads). Immunohistochemical stains for desmin (f) and myogenin (g). Axial (h) post-contrast T1-weighted images showing final tumor progression before the patient passed

Fig. 3

Kaplan–Meier survival curves. Overall survival (a) in patients with a diagnosed MPNST. Survival by the following: extent of resection (b), presence of NF-1 mutation (c), tumor grade (d), age (e) and adjuvant chemotherapy/radiation (f). Abbreviations: extent of resection (EOR); gross total resection (GTR); neurofibromatosis type 1 (NF-1); radiotherapy (RT); subtotal resection (STR)