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Intraoperative Ultrasound in Spinal Surgery
Published on: August 17, 2022
Intramedullary primitive neuroectodermal tumor presenting with rapidly-progressive cauda equina syndrome
Satoshi Tsutsumi1, Yasuomi Nonaka, Yusuke Abe
1Department of Neurological Surgery, Juntendo University Urayasu Hospital, Urayasu, Chiba, Japan. shotaro@juntendo-urayasu.jp
Neurologia Medico-Chirurgica
|December 3, 2010
Summary
Primitive neuroectodermal tumors (PNETs) are rare spinal cord tumors. This case highlights PNET as a critical diagnosis for intramedullary spinal cord tumors, despite aggressive progression and dissemination.
Area of Science:
- Neuro-oncology
- Spinal Cord Pathology
Background:
- Intramedullary spinal cord tumors (IMSCTs) present diagnostic challenges.
- Primitive neuroectodermal tumors (PNETs) are rare but aggressive CNS neoplasms.
Observation:
- A 39-year-old male presented with rapid gait deterioration, paraparesis, sensory loss, and vesicorectal dysfunction.
- MRI revealed a T12-L1 intramedullary tumor with heterogeneous enhancement.
- Surgical resection showed a hypercellular, atypical tumor with positive immunohistochemistry for GFAP, S-100, synaptophysin, and INI-1, confirming PNET.
Findings:
- Subtotal resection was followed by craniospinal axis irradiation.
- The patient experienced local recurrence at 7 months, with widespread progression and intracranial dissemination by 10 months.
- The patient succumbed to the disease 13 months after symptom onset.
Implications:
- PNET must be considered in the differential diagnosis of intramedullary spinal cord tumors.
- Aggressive behavior and dissemination underscore the need for early and accurate diagnosis.
- This case emphasizes the poor prognosis associated with spinal PNETs, even with multimodal treatment.
