Primary intradural hemangiopericytoma with intramedullary invasion

J Chin Med Assoc. 2009 Oct;72(10):536-41. doi: 10.1016/S1726-4901(09)70424-1.

Abstract

Hemangiopericytoma (HPC) is a rare tumor of the central nervous system and is usually found intracranially. Intraspinal HPCs are very rare and mostly involve the extradural bony structures. Primary intradural HPC has only been reported in 10 cases, all of which occurred in the extramedullary region. Intramedullary invasion has never been reported. Here, we describe a case of primary intradural HPC of the thoracic spine that presented initially with paresthesia and paraplegia of both legs. Magnetic resonance imaging of the thoracic spine showed an intradural dumbbell-shaped tumor at the T10 level. The initial impression was neurogenic tumor, meningioma, or metastasis. During operation, the tumor was found to have obvious intramedullary invasion. Gross-total removal was done, and the patient's neurological function improved; there was no recurrence at the 3-year follow-up. There is no consensus as to what constitutes the optimal treatment of HPC, but most neurosurgeons will advocate gross-total resection. A comparative analysis between intradural and extradural HPCs showed a higher chance of gross-total resection for intradural HPCs, while the recurrence rates showed no difference. The role of adjuvant radiotherapy remains uncertain. Due to the high risk of recurrence and metastasis of HPCs, close follow-up for a long period is mandatory.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Aged, 80 and over
  • Dura Mater / pathology*
  • Hemangiopericytoma / pathology*
  • Hemangiopericytoma / surgery
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Invasiveness
  • Spinal Cord Neoplasms / pathology*
  • Spinal Cord Neoplasms / surgery
  • Thoracic Vertebrae / pathology*