|
ИСТИНА |
Войти в систему Регистрация |
ИСТИНА ИНХС РАН |
||
Stereotactic irradiation of sporadic and VHL-associated hemangioblastomas of the CNSстатья Тезисы
- Авторы: Лестровая А.И., Голанов А.В., Ветлова Е.Р., Золотова С.В., Антипина Н.А.
- Журнал: JOURNAL OF RADIOSURGERY AND SBRT
- Номер: 8
- Год издания: 2022
- Первая страница: 105
- Последняя страница: 106
- Номер статьи: PO75
- Аннотация: Hemangioblastomas are rare, benign, highly vasculartumors 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 aresporadic or seen in association with von Hippel-Lindau(VHL) disease, an inherited autosomal dominant disordercaused by mutation in the VHL tumor suppressor gene.In sporadic hemangioblastomas, the prognosis isrelatively favorable. In the presence of a germline VHLmutation, on the contrary, they are characterized by theoccurrence of relapses, synchronous and asynchronousmultiple hemangioblastomas. Thus, the diagnosis ofHippel-Lindau syndrome is an important part of theexamination of patients with hemangioblastomas,especially those under the age of 50. However, within thegroup of sporadic and hereditary hemangioblastomas,there are differences in the growth rate, recurrence,sensitivity to radiotherapy of inoperable tumors, as wellas in the profles of molecular genetic structural andexpression changes in the genome of tumor cells.From 2005 to 2021, at the National Scientifc andMedical Center of Neurosurgery named after N.N.Burdenko 108 patients with hemangioblastomasreceived stereotactic irradiation (95 patients with VHLsyndrome). Sporadic forms hemangioblastomas ischaracterized the solitary nature of the lesion, mostlylocalized in the posterior cranial fossa or cervical spinalcord. All cases of multiple hemangioblastomas wereassociated with VHL syndrome. The average age of thepatient is 30.2 years (median 14-76 years). 805 of brainand 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 in25-30 fractions. Median follow-up was 40.3 months(range 1-182 months). 5-year PFS was 86%.Our results are confrming that stereotacticradiosurgery and radiotherapy are effective andrelatively safe methods of radiation treatment ofpatients with hemangioblastomas. However, the choiceof fractionation regimen and doses, depending on theassociation with the VHL syndrome, requires furtherresearch, the long-term effcacy of radiotherapy forhemangioblastomas still needs to be investigated. Alsovery important to conduct studies in which exploring therole of radiotherapy for early treatment of asymptomaticlesions. Perhaps, the study of combinations of germlineand somatic VHL mutations and mutations in minorcandidate genes of hemangioblastomas will revealnew diagnostic and prognostic criteria of course andtreatment including stereotactic irradiation for differentforms of hemangioblastomas of the central nervoussystem
- Добавил в систему: Лестровая Арина Игоревна