Epidemiology of the vestibular schwannomas in Ukraine and our experience of surgical and radiosurgical treatment

Aim: Figuring out the occurrence of vestibular schwannomas (VS) and their management tendencies in Ukraine.Materials and methods: Data about VS detection and treatment were collected among all Ukrainian neurosurgical and radiological facilities which are enrolled in providing care for these patients. Together with those who were treated overseas the total number in 2016-2018 was 903 people. There were 665 cases (73.6  %) treated surgically, 124 cases (13.8  %) irradiated and 114 (12.6  %) observed via serial imaging.Results: The prevalence of VS in Ukraine is about 7.27 per 1 million people which corresponds to data around the world (CBTRUS trial suggested VS incidence 10-20 people per 1 million during 2004-2009).Most of verified VSs were treated surgically (73.6  %), lesser part was irradiated (13.8  %) and 12.7 % were followed-up by wait-and-scan strategy. Our data regarding surgical management was higher than worldwide. At the same time, the volume of detected tumors was much larger in comparison to published data. Seventy-three per cent of all cases were Koos T4 tumors as a possible result of poor diagnosis and lack of alertness making surgical interventions more common and difficult.Total and subtotal resection rate was 79  % as the result of combined microsurgical and endoscopic techniques under intraoperative electrophysiological neuromonitoring guidance. The facial nerve was preserved in 94.2  % of cases, cochlear — in 8.5  % of cases. The average mortality rate during 2016-2018 in Ukraine was 3.1  % with 1.3  % in Subtentorial Neurooncology Department of the Romodanov Neurosurgery Institute.Conclusions: For further improvements and development of optimal management strategies for patients with VS, it is necessary to improve earlier diagnosis and reasonable to provide neurosurgical care in high-volume centers based on the profound expertise with further advances in technologies for functionally favorable outcomes

Клінічний випадок п’ятирічного локального контролю після радіохірургії інтракраніальних пухлин у хворої на нейрофіброматоз типу II

Type II neurofibromatosis (NF2) is a rare autosomal dominant hereditary disease characterized by the presence of many benign tumors of the nervous system. Stereotactic radiosurgery (SRS) is a generally accepted therapeutic option for sporadic vestibular schwannoma and small and medium-sized meningiomas, which allows treating several tumors simultaneously during a single irradiation session. At the same time, opinions on the use of radiosurgery as the primary treatment for patients with NF2 remain controversial. This article deals with a clinical case of the results of radiosurgical treatment of a 50-year-old patient with NF2, with a follow-up period of 5 years. The final results of SRS in ensuring local control of NF2 associated with vestibular schwannoma and meningiomas should be evaluated not earlier than 24–36 months after irradiation. The advantages of radiosurgical treatment of patients with NF2 include non-invasiveness, the possibility of irradiating several tumors in a single irradiation session, treatment under outpatient conditions, ensuring local control of tumor growth, minimizing of the neurological deficit associated with the treatment toxicity, ensuring the quality of life of patients.Нейрофиброматоз типа II (НФ-2) – редкое аутосомно-доминантное наследственное заболевание, которое характеризуется наличием множества доброкачественных опухолей нервной системы. Стереотаксическая радиохирургия является общепринятой лечебной опцией при спорадических вестибулярных шванномах и менингиомах малого и среднего размера, позволяющей лечить несколько опухолей симультанно во время одного сеанса облучения. Мнения об использовании радиохирургии как первоочередного метода лечения больных с НФ-2 спорные. Представлен клинический случай радиохирургического лечения 50-летней больной с НФ-2 с периодом наблюдения 5 лет. Результаты стереотаксической радиохирургии относительно обеспечения локального контроля НФ-2-ассоциированных вестибулярных шваном та менингиом следует оценивать не раньше чем через 24–36 мес после облучения. Преимуществами радиохирургического лечения больных с НФ-2 являются: неинвазивность, возможность облучения нескольких опухолей за один сеанс, лечение в амбулаторных условиях, обеспечение локального контроля роста опухолей, минимизация неврологического дефицита, связанного с токсичностью лечения, обеспечение качества жизни больных. Следует помнить, что радиохирургическое лечение не влияет на контроль прогрессирования других НФ-2-ассоциированных интракраниальных опухолей.Нейрофіброматоз типу II (НФ-2) – рідкісне аутосомно-домінантне спадкове захворювання, яке характеризується наявністю множинних доброякісних пухлин нервової системи. Стереотаксична радіохірургія є загальноприйнятою лікувальною опцією при спорадичних вестибулярних шваномах та менінгіомах малого і середнього розміру, яка дає змогу лікувати декілька пухлин симультанно під час одного сеансу опромінення. Думки щодо використання радіохірургії як першочергового методу лікування хворих на НФ-2 суперечливі. Представлено клінічний випадок радіохірургічного лікування 50-річної хворої, яка страждає на НФ-2, з періодом спостереження 5 років. Результати стереотаксичної радіохірургії щодо забезпечення локального контролю НФ-2-асоційованих вестибулярних шваном та менінгіом слід оцінювати не раніше ніж через 24–36 міс після опромінення. До переваг радіохірургічного лікування хворих із НФ-2 належать: неінвазивність, можливість опромінення декількох пухлин за один сеанс, лікування в амбулаторних умовах, забезпечення локального контролю росту пухлин, мінімізація неврологічного дефіциту, пов’язаного із токсичністю лікування, збереження якості життя хворих. Слід пам’ятати, що радіохірургічне лікування не впливає на контроль прогресування інших НФ-2-асоційованих інтракраніальних пухлин

Suprasellar mature teratoma: case report

Intracranial teratomas are tumors that occur mainly in childhood and extremely rare in adulthood. They account up to 50% congenital CNS tumors. In this article a case of mature teratoma in 33 year-old female patient with progressive visual impairment is presented. Differential diagnosis at preoperative stage was difficult. Outcome analysis of neuroimaging study method and clinical picture provided evidence of epidermal cyst. Transnasal endoscopic approach as a treatment method was chosen, but during the surgery the atypical tissue for epidermal cyst was identified with tight adhesion to the right internal carotid artery which limited the extent of surgical tretment. Pathohistological and immune histochemical study detected mature teratoma. Detailed visual impairment dynamic and instrumental methods of diagnosis during postoperative supervision are presented in the article. The choice of management, namely, surgical intervention using extended endoscopic transnasal approach is considered to be controversial and risky among different authors taking into account intraoperative characteristics of this tumor

Current state of antiangiogenic therapy in neuro-oncology and own experience of its use in the radiosurgical treatment of recurrent glioblastoma

Objective: to study the effect of antiangiogenic therapy on the quality of life and the level of headache in patients with recurrent glioblastoma who underwent radiosurgical treatment. Materials and methods. A prospective randomized single-center study carried out at the Romodanov Neurosurgery Institute of National Academy of Medical Sciences of Ukraine in 2019-2020 involving 45 patients with GB with clinical and radiological signs of disease progression and local tumor recurrence. In this regard, patients underwent radiosurgical treatment. In the main group (BEV+) 21 patients after stereotactic radiosurgery (SRS) underwent antiangiogenic therapy with Bevacizumab (BEV). In the control group (BEV–), 24 patients did not receive antiangiogenic therapy after SRS. SRS with the use of a linear accelerator «Trilogy» (6 MeV) using intensity-modulated radiotherapy (IMRT). BEV was administered intravenously, once every 3 weeks at a dose of 10 mg / kg body weight. Antiangiogenic therapy was performed under the condition of preserved liver and kidney function, values of full blood count and blood biochemistry within normal range. Global health status and headache levels were calculated according to EORTC QLQ-C30 v. 3.0 and QLQ-BN20 before and six weeks after radiosurgery in the main and control groups. Results. There was no a statistically significant difference between the studied groups of patients’ in quality of life (p = 0.707372) and in headache level (p = 0.846660) before the SRS. Six weeks after SRS, patients in the main group had a statistically significantly higher quality of life (p = 0.000015) and a lower level of headache than patients in the control group (p = 0.000035). During the observation period in patients of both groups there were no adverse events of III-IV degree of toxicity, in particular specific complications of antiangiogenic therapy (hypertension, bleeding, thromboembolism, leukopenia, proteinuria, gastrointestinal disorders, etc.). Conclusions. Antiangiogenic therapy statistically significantly improves the quality of life and reduces the level of headache in patients who underwent radiosurgical treatment for glioblastoma recurrence

Hypofractionated radiotherapy of patients with glioblastoma: the first experience in Ukraine and prospects view

Objective: To assess the survival of patients (pts) with newly diagnosed glioblastoma (GBM) in groups with hypofractionated regime radiotherapy, standard fractionationated regime radiotherapy and whole brain irradiation.Materials and methods: A retrospective non-randomized single-center study of 205 pts with GBM grade 4 according to the WHO classification treated in Romodanov Neurosurgery Institute named after Acad. A.P. Romodanov National Academy of Medical Science of Ukraine (2014–2020). The mean age of pts 53.8 years (95% CI 52.1 - 55.5); there were 114 men (55.6%) and 91 women (44.4%). According to the regimen radiotherapy (RT), pts were divided into 3 groups: 49 (23.9%) pts (standard RT: 2.0 Gy per fraction in 30 fractions, total dose 60.0 Gy) – group "sRT", 110 (53.7%) pts (hypofractionated RT: 3.5 Gy per fraction in 15 fractions, total dose 52, 5 Gy) - group "hRT", 46 (22.4%) pts – group "WBRT" (whole brain radiation). Differences in progression-free survival (PFS) and overall survival (OS) between the three groups were analyzed using Kaplan–Meier survival curve, log-rank test and Pearson Chi-square test.Results: The median OS is 15,0 (95% CI 14,1 – 17,1), 16,5 (95% CI 14,1 – 18,8) and 8,7 (95% CІ 7,5 – 9,5) months for sRT, hRT and WBRT, respectively. There is a significant difference in OS for sRT and hRT compared to WBRT (p=0.00000), without difference in OS between sRT and hRT (p=0.06757). The median PFS in sRT and hRT does not differ significantly: sRT – 9.0 (95% CI 9.0 - 10.0) months; hRT – 9.0 (95% CI 8.0 - 10.0) months. The median OS for WBRT is 5.1 (95% CI 4.0 - 6.0) months. There is a significant difference in PFS for sRT and hRT compared to the WBRT (p=0.00000), without difference in PFS between sRT and hRT (p=0.43374). The risk of death for WBRT compared to sRT is 2.5 times higher (HR 2.5 [95% CI, 1.45 - 4.46)) and 3.5 times higher compared to the hRT group (HR 3.5 [95% CI, 2.09-5.88)). The risk of progression for WBRT is 2.8 times higher (HR 2.78 [95% CI, 1.63-4.74)) compared to sRT, and 3.1 times higher (HR 3.12 [95% CI, 1.91-5.10)) compared to hRT.The broad implementation of hRT into clinical practice is specific to all modern radiation oncology. This trend is currently underway due to the specific positive clinical effects of hRT, which are discussed in detail in our publication.Conclusions: Our study demonstrates comparable survival outcome between sRT and hRT groups. This is an argument in favor of the feasibility of using hRT as a part of multimodal GBM treatment in terms of oncological outcomes. Further studies are needed to identify specific stratification groups of GMB patients with the greatest survival and quality of life benefits due to hRT