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Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases: A case report.

Frank Chen1, Shyh-Shin Chiou, Sheng-Fung Lin

  • 1aDepartment of Radiation Oncology bDepartment of Pediatrics cDepartment of Internal Medicine dDepartment of Surgery eDepartment of Pathology, Kaohsiung Medical University Hospital, Kaohsiung Medical University fFaculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.

Medicine
|November 18, 2017
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Summary

Primary spinal primitive neuroectodermal tumor (PNET) is rare and often has a poor prognosis. This case highlights successful multimodality treatment for recurrent spinal PNET with extensive metastases, achieving long-term survival.

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Area of Science:

  • Neuro-oncology
  • Spinal cord tumors
  • Pediatric oncology

Background:

  • Primary spinal primitive neuroectodermal tumors (PNETs) are rare, aggressive central nervous system neoplasms.
  • Prognosis for spinal PNETs is generally poor, with a median survival of 1-2 years.

Observation:

  • A 14-year-old female presented with progressive back pain and lower extremity weakness.
  • Initial diagnosis revealed an intradural extramedullary T5-T10 PNET (WHO grade IV).
  • Recurrence involved epidural tumor with foraminal invasion, extensive brain metastases (calvarial and leptomeningeal), and bone metastases.

Findings:

  • The patient underwent initial tumor removal and adjuvant radiotherapy.
  • Following recurrence, salvage surgery, chemotherapy, and whole brain irradiation with hippocampal sparing were administered.
  • Follow-up imaging demonstrated disease control without neurological sequelae.

Implications:

  • Multimodality treatment, including surgery, radiotherapy, and chemotherapy, is crucial for managing spinal PNETs.
  • Salvage chemotherapy can be effective in recurrent cases with widespread metastases.
  • This case demonstrates the potential for long-term survival in recurrent spinal PNET with aggressive, comprehensive treatment.