Cerebellar and multiple spinal hemangioblastomas and intraventricular meningioma managed with subtotal resection and external beam radiotherapy; report of a case with literature review.
Hemangioblastomas are cystic, highly vascular benign neoplasms that constitute 1.5-2.5% of all intracranial tumors and 7-10% of primary posterior fossa tumors. They occur sporadically (80%) or in association with von Hippel-Lindau (VHL) disease (20%). This disease consists of multiple intracranial, retinal and spinal hemangioblastomas, pheochromocytoma, retinal angiomas, pancreatic cysts, renal cell carcinomas and adrenal tumors. Our patient was a 21-year-old male who presented with cerebellar and multiple spinal hemangioblastomas, and intraventricular meningioma. There was a positive family history (mother and brother) of VHL disease. Intracranial and spinal lesions were treated with external beam radiotherapy following subtotal excision of the cerebellar lesion. Three-year follow-up revealed radiologically stable lesions. Microsurgical resection remains the treatment of choice for the vast majority of symptomatic and sporadic cystic hemangioblastomas. However, since hemangioblastoma is a highly vascular tumor and local invasion of critical structures is frequent and multifocality is often a characteristic of the hemangioblastomas that are associated with VHL disease, subtotal excision is frequent and adjuvant therapies such as external beam radiotherapy or stereotactic radiosurgery represent a reasonable treatment in such cases.
Aksu
G
G
Gulhane Military Faculty of Medicine, Department of Radiation Oncology, Ankara, Turkey.
Ulutin
C
C
Fayda
M
M
Saynak
M
M