Clinical presentation and long-term outcome of primary spinal intradural malignant peripheral nerve sheath tumors

Abstract Objective Primary spinal intradural malignant peripheral nerve sheath tumors (MPNSTs) are exceedingly rare, and there is limited published information regarding these lesions. The purpose of this study was to analyze the clinical course, treatment, and outcome of primary spinal intradural MPNSTs. Patients and Methods A retrospective study was performed on 8 patients with primary spinal intradural MPNSTs who were surgically treated and pathologically confirmed in our institution between 2007 and 2018. Results There were 3 females and 5 males, with a median age of 46.5 years (range, 21–68 years). Limb weakness was the most common clinical symptom (5/8, 62.5%). The radiological diagnosis was meningioma or schwannoma in most patients (7/8, 87.5%). Malignancies were graded high in 5 cases (62.5%) and low in 3 cases (37.5%). Gross total resection (GTR) was performed in 5 cases, while subtotal resection (STR) was achieved in 3 cases. The mean follow-up period was 48.9 months (range, 10–160 months). During follow-up, local recurrence occurred in 6/8 cases (75.0%) and distant metastasis occurred in 2/8 cases (25.0%). The median survival time (MST) of these 8 patients was 21.0 months. The overall 1-year, 2-year, and 5-year survival rates were 87.5% (7/8), 50.0% (4/8), and 25.0% (2/8), respectively. Conclusions Primary spinal intradural MPNST is a challenging clinical entity given its high local relapse rate. Primary spinal intradural MPNSTs radiologically present themselves heterogeneous and thus difficult to distinguish from schwannomas or meningiomas. Surgical resection, especially GTR, is the preferential treatment for primary spinal intradural MPNSTs.