Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

Abstract

<jats:p>Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.</jats:p>

Spinal intradural primary malignant peripheral nerve sheath tumor with leptomeningeal seeding: case report and literature review

“…As found in our patient, the spinal MPNST lesions were described as non-homogenous hyperintense in T2WI and isointense in T1WI that enhanced after gadolinium injection (15,21). The hyperintense area on T2WI was reported as a central cystic lesion or necrosis (4).…”

“…We summarized them in Table I. They are primarily distributed in: cervical in 7 patients (30%) (2,8,17,23), thoracic and thoracolumbar in 6 (26%) (7,15,17,23), and lumbar in 10 (44%) (1,3,7,14,17,21,22,24,25). Our patient had an intradural extramedullary cervical tumor extending from foramen magnum to lower cervical vertebrae with severely invasion of the spinal cord.…”

“…Patients usually present with pain, weakness, sensory loss, and bladder/bowel deficits. Hydrocephalus was seen in 4 patients with spinal MPNSTs (8,14,15), including 3 patients with brain metastasis and one with the cervical spinal cord involvement. Our patient developed acute hydrocephalus during hospitalization.…”

“…The hyperintense area on T2WI was reported as a central cystic lesion or necrosis (4). The leptomeningeal metastasis was hypointense in T2WI and enhanced homogenously (14,15,21,24). The spinal seeding is defined as a "string-of-beads" pattern since they are small enhancing lesions spreading along the spinal cord (15).…”

Ominous Occurrence of Spinal Intradural Primary Malignant Peripheral Nerve Sheath Tumor Four Decades following Radiation Therapy for Testicular Seminoma

“…Unique to this case, however, is the rapid development of disseminated disease two weeks a er surgery. Patients in other reported cases of intradural MPNSTs with leptomeningeal spread were diagnosed with dissemination ranging from present on presentation to 24 months following initial surgery [10][11][12][13][14][15][16][17][18][19]. e authors advocate that regardless of imaging characteristics or the duration since radiation, surgeons should retain a high index of suspicion for a MPSNT.…”