Hemangioblastoma
Overview
Hemangioblastoma of the central nervous system is a benign tumor (WHO grade I) that is typically cystic and can occur throughout the central nervous system. It is a tumor composed of hemangioblasts, a type of stem cell that normally give rise to blood vessels or blood cells. Its name breaks down to Greek roots as follows: Haema (blood), angeion (vessel), blastos (embryonic), oma (tumor).
Causes
Hemangioblastomas can occur "sporadically", or as a component of the von Hippel-Lindau disease (VHL). VHL is inherited in an autosomal dominant pattern. The VHL gene behaves as a tumor suppressor, i.e. mutation of the VHL gene predisposes individuals to development of a wide variety of benign and malignant tumors. These tumors occur in target tissues such as the kidneys, adrenal gland, central nervous system and retina.
Prognosis
1 low recurrence risk if total resection 2 risk of multiple tumors in VHL and so worse prognosis 3 need to screen for mutation if young person with hemangioblastoma, if family history positive, any other tumors (esp retinal) 4. ook for pheochromocytoma if VHL positive
Treatment
surgery may be curative in sporadic cases sufficient to remove tumor nodule; cyst wall is non-neoplastic radiotherapy is occasionally used to reduce tumor size preoperatively or retard growth in non-surgical candidates (multiple lesions or inoperable location) radiation does not prevent regrowth following subtotal resection