Hamangioblastoma Hemangiopericytoma

Hemangioblastoma

  • Benign vascular tumors
  • 3rd - 4th decade
  • 1% to 2% of primary CNS tumors in adults
  • Mostly from cerebellum
  • Most common primary cerebellar tumors in adults
  • Association with von Hippel-Lindau disease is noted in 10% of patients
    • Imaging of craniospinal —> multiple lesions in VHL

  • Histology and Radiology
    • Closely packed, thin-walled blood vessels in a stroma of large, oval foamy cells
    • Intensely enhancing on CT and MRI
    • Angiography confirms the vascular nature of the lesion

  • Treatment
    • Surgery
    • Complete resection is curative
    • If unresectable —> Radiosurgery
      • Higher rates of recurrence

Hemangiopericytoma

  • Sarcomatous lesion
  • Developing from smooth muscle in blood vessels
  • Usually along the base of the skull
    • More rarely intraparenchymal
  • Systemic metastases: COMMON

  • Treatment:
    • Surgery
      • 90% 9-year actuarial risk for local failure
    • Post-Op RT
      • 50 to 60 Gy
        • Reduces the risk of recurrence rate & improves overall survival
        • Tumor control is dose dependent
          • Doses >50 Gy —> superior outcomes
          • Radiographic response is slow
  • Recurrent : Radiosurgery
    • Local control rates of approximately 80%

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Evidence-Based Treatment Summary

  • Surgical resection is recommended, when feasible, for both of these diseases.
  • Radiotherapy is generally reserved for subtotally resected progressive hemangioblastoma, but there are no prospective data.
  • Postoperative radiotherapy is recommended for subtotally resected hemangiopericytoma, but there are no prospective data.
  • Radiotherapy or radiosurgery may be considered for unresectable tumours.