ИСТИНА

Hemangioblastomas are rare, benign, highly vascular tumors most frequently arising in the cerebellum, brainstem, or upper cervical cord, and account for 2-6% of all brain and spinal cord tumors. These lesions are sporadic or seen in association with von Hippel-Lindau (VHL) disease, an inherited autosomal dominant disorder caused by mutation in the VHL tumor suppressor gene. In sporadic hemangioblastomas, the prognosis is relatively favorable. In the presence of a germline VHL mutation, on the contrary, they are characterized by the occurrence of relapses, synchronous and asynchronous multiple hemangioblastomas. Thus, the diagnosis of Hippel-Lindau syndrome is an important part of the examination of patients with hemangioblastomas, especially those under the age of 50. However, within the group of sporadic and hereditary hemangioblastomas, there are differences in the growth rate, recurrence, sensitivity to radiotherapy of inoperable tumors, as well as in the profiles of molecular genetic structural and expression changes in the genome of tumor cells. From 2005 to 2021, at the N.N. Burdenko National Scientific and Practical Center for Neurosurgery 108 patients with hemangioblastomas received radiotherapy (95 patients with VHL syndrome). Sporadic forms hemangioblastomas is characterized the solitary nature of the lesion, localization in the posterior cranial fossa or cervical spinal cord. All cases of multiple hemangioblastomas were associated with VHL syndrome. The average age of the patient is 30.2 years (median 14-76 years). 805 of brain and spinal cord tumors were treated. 627 tumors (78%) were treated in the radiosurgery (16-20 Gy). 138 (17%) in the hypofractionation (22,5-24 Gy in 3 fractions; 27,5-30 Gy in 5 fractions), and 40 (5%) tumors 45-54 Gy in 25-30 fractions. Median follow-up was 40.3 months (range 1-182 months). 5-year PFS was 86%. Thus, confirming stereotactic radiosurgery and radiotherapy are effective and relatively safe methods of radiation treatment of patients with hemangioblastomas. However, the choice of fractionation regimen and doses, depending on the association with the VHL syndrome, requires further research, the long-term efficacy of radiotherapy for hemangioblastomas still needs to be investigated, and the studies exploring the role of radiotherapy for early treatment of asymptomatic lesions is wanting. Perhaps, the study of combinations of germline and somatic VHL mutations and mutations in minor candidate genes of hemangioblastomas will reveal new diagnostic and prognostic criteria for different forms of hemangioblastomas of the central nervous system.