NCOG-03. SURGICAL MANAGEMENT OF CRANIOSPINAL AXIS MALIGNANT PERIPHERAL NERVE SHEATH TUMORS: A SINGLE-INSTITUTION EXPERIENCE AND LITERATURE REVIEW

Abstract

Abstract Malignant peripheral nerve sheath tumors (MPNSTs) are exceedingly rare and aggressive tumors with an overall incidence rate of 0.001%. The craniospinal axis is a rare location for primary MPNSTs with very few cases reported in the literature to date. Here-in, we present one of the larger series of single-institutional primary craniospinal MPNSTs, analyze patient outcomes, and extensively review the associated literature. All primary craniospinal axis MPNSTs managed at our institution between January 2005 to May 2023 were retrospectively reviewed. Patient demographics, tumor characteristics and radiographic features, treatment, and clinical outcomes were collected and analyzed. Descriptive statistics and multivariate logistic regression were performed. A total of 8 patients satisfied inclusion criteria (4 male and 4 female patients). Median age at presentation was 40 years (range 34-80 years). Most of these tumors were localized to the spine (6, 75%) and 3 patients (37%) had neurofibromatosis type 1(NF-1). Most common presenting symptoms were paresthesia/numbness in 50% of cases, and vision changes in 13%. Median tumor size was 3 cm (range 1-20), and most tumors were oval-shaped (4, 50%), with well-defined borders (6, 75%). One spinal tumor (11%) had an intramedullary extension. Most tumors were high grade (6, 75%), and gross-total resection was achieved in 5 (63%) patients with subtotal resection performed in the remaining 3 (37%). Radiotherapy and chemotherapy after surgery were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence was evidenced in 5 (63%) cases and distant metastatic disease in 2 (25%) cases. The median progression-free survival time was 26.0 months, and 5 (63%) patients died due to recurrent disease. Early diagnosis and initiation of treatment is key as these tumors are highly aggressive with increased local invasion and distant metastatic rates. Maximal safe gross total resection with negative margins with adjuvant radiation therapy is associated with improved survival.