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Primary brain metastases are common in oncology. Preoperative stereotactic radiosurgery followed by surgical resection is a perspective approach. OBJECTIVE: To evaluate own experience of preoperative radiosurgery followed by surgical resection (RS+S) of metastasis regarding local control, leptomeningeal progression, surgical and radiation-induced complications; to compare treatment outcomes with surgical resection and subsequent radiotherapy (S+SRT). MATERIAL AND METHODS. A: Retrospective study included 66 patients with solitary brain metastasis. Two groups of patients were distinguished: group 1 (n=34) - postoperative irradiation, group 2 (n=32) - preoperative irradiation. The median age was 49.5 years (range 36-75). RESULTS: Local 3-, 6- and 12-month control among patients with postoperative irradiation was 88.2%, 79.4% and 42.9%, in the group of preoperative irradiation - 100%, 93.3% and 66.7%, respectively (p=0.021). Leptomeningeal progression developed in 11 patients (8 and 3 ones, respectively). The one-year survival rate was 73.5% and 84.4%, respectively (p=0.33). Long-term surgical and radiation-induced complications occurred in 12 (18.2%) patients. CONCLUSION: Preoperative radiosurgery with subsequent resection provides higher local control and lower incidence of leptomeningeal progression in patients with single brain metastases.
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Neoplasias Encefálicas , Radiocirurgia , Humanos , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/secundário , Neoplasias Encefálicas/terapia , Pessoa de Meia-Idade , Masculino , Feminino , Adulto , Idoso , Radiocirurgia/métodos , Estudos Retrospectivos , Terapia CombinadaRESUMO
BACKGROUND: Stereotactic radiosurgery (SRS) for cerebral cavernous malformations has been used for more than 30 years. However, indications for this method and outcomes are still discussable. OBJECTIVE: To analyze available literature data on SRS for cerebral cavernous malformations with assessment of indications for treatment, radiation parameters, radiological and clinical complications and outcomes. RESULTS: The final analysis included 20 reports describing post-SRS outcomes in 1834 patients with cerebral cavernous malformations. The main radiation parameter was mean radiation dose to the edge of cavernous malformation (prescribed dose, 13.25±2.16 Gy). In natural course of malformation, mean incidence of hemorrhages from cavernous malformation when counted after the second hemorrhage was 25.9±14.6%, after the patient's birth - 2.59±0.44%. Mean follow-up period after SRS was 66.7±24.1 months. Incidence of hemorrhages from cavernous malformation after SRS for the first 2 years of follow-up was assessed in 14 studies (4.67±3.51%). Incidence of hemorrhages ≥2 years after SRS was analyzed in 12 studies (1.55±0.8%). CONCLUSION: Despite significant global experience in SRS for cerebral cavernous malformations and many studies devoted to this problem, clear patient selection criteria have not yet been formulated. Modern selection principles have insufficient evidence base.
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Hemangioma Cavernoso do Sistema Nervoso Central , Radiocirurgia , Humanos , Radiocirurgia/métodos , Radiocirurgia/efeitos adversos , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico por imagem , Feminino , MasculinoRESUMO
OBJECTIVE: To identify the characteristics of pain syndrome in patients with schwannomas depending on genetic predisposition. MATERIAL AND METHODS: The study included 46 patients with peripheral, spinal and intracranial schwannomas, corresponding to the schwannomatosis phenotype according to the 2022 clinical criteria. All patients underwent sequencing of the LZRT1, Nf2 and SMARCB1 and a copy number study in the NF2. RESULTS: The most severe widespread pain was observed in patients with pathogenic LZRT1 variants, while patients with mosaic variants may not even have local tumor-related pain. Patients with SMARCB1variants may have no pain or have localized pain that responds well to surgical treatment. CONCLUSION: Further studies of the molecular features of schwannomatosis and driver mutations in the pathogenesis of pain are necessary to improve the effectiveness of pain therapy in this group of patients. Schwannomatosis is a disease from the group of neurofibromatosis, manifested by the development of multiple schwannomas. Neuropathic pain is one of the main symptoms characteristic of peripheral schwannomas, however, the severity and prevalence of the pain syndrome does not always correlate with the location of the tumors. According to modern concepts, the key factors influencing the characteristics of the pain syndrome are the target gene and the type of pathogenic variant. The most severe widespread pain is observed in patients with pathogenic variants in the LZRT1 gene, while patients with mosaic variants may not even have local pain associated with tumors. Patients with variants in SMARCB1 may have no pain or localized pain that responds well to surgical treatment.
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Neurilemoma , Neurofibromatoses , Proteína SMARCB1 , Humanos , Neurilemoma/genética , Neurilemoma/complicações , Neurilemoma/diagnóstico , Neurofibromatoses/complicações , Neurofibromatoses/genética , Masculino , Feminino , Adulto , Proteína SMARCB1/genética , Pessoa de Meia-Idade , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/complicações , Neurofibromina 2/genética , Fatores de Transcrição/genética , Mutação , Neuralgia/genética , Neuralgia/etiologia , Neuralgia/diagnóstico , Predisposição Genética para Doença , Adulto JovemRESUMO
Post-resection or isolated hypofractionated stereotactic radiotherapy (HF-SRT) is a therapeutic option for large brain metastases (>2 cm, LBMs). OBJECTIVE: To compare the results of post-resection or isolated HF-SRT in patients with LBMs. MATERIAL AND METHODS: A prospective study included 115 patients with 129 intact LBMs and 133 patients with 149 resected LBMs who underwent HF-SRT. Median baseline focal size was 22.5 and 28 mm, median target volume - 8.3 and 23.7 cm3, respectively. RESULTS: Median follow-up was 13.9 months, median overall survival - 19.1 months. After 12 months, local recurrences developed in 17 and 31% of patients, respectively (p=0.0078). Local recurrence after 12 months developed in 23% of patients with residual tumor in postoperative cavity compared to 16% of patients after total resection (p=0.0073). After 12 months, incidence of leptomeningeal progression was 27 and 11%, respectively (p=0.033), incidence of symptomatic radiation-induced necrosis - 4 and 23%, respectively (p=0.0006). CONCLUSION: Post-resection HF-SRT demonstrated better local control and less severe symptomatic radiation-induced necrosis compared to patients with intact LBMs. Incidence of leptomeningeal progression is significantly higher after resection of LBMs.
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Neoplasias Encefálicas , Radiocirurgia , Humanos , Estudos Prospectivos , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/secundário , Radiocirurgia/métodos , Hipofracionamento da Dose de Radiação , Estudos Retrospectivos , Necrose/cirurgia , Resultado do TratamentoRESUMO
There are various approaches to the treatment of patients with parasagittal meningiomas. OBJECTIVE: To optimize treatment strategy for meningiomas invading the superior sagittal sinus. MATERIAL AND METHODS: The study included 87 patients with benign parasagittal meningiomas between 2010 and 2012. Of these, 34 patients underwent surgery alone, 27 - radiotherapy, 26 - surgery and subsequent radiotherapy. Both groups were comparable in male-to-female ratio, age and localization of tumors in relation to superior sagittal sinus. The follow-up period was at least 5 years. We analyzed the effect of treatment on neurological status, Karnofsky score and tumor growth control. RESULTS: Mean volume of tumors was 43.3 cm3 in patients undergoing surgery and 6.7 cm3 in the radiotherapy group. In the combined treatment group, mean volume was 65.8 cm3 before surgery and 8.8 cm3 before irradiation. General cerebral symptoms (84%), epileptic seizures (37%) and movement disorders (31%) prevailed. Surgery provided the best results in patients with small meningiomas (<14 cm3) causing focal neurological symptoms. Isolated radiotherapy was the most effective in asymptomatic patients. Large tumors required surgery with adjuvant irradiation. CONCLUSION: Benign parasagittal meningiomas followed by focal neurological symptoms require surgical intervention regarding the best functional outcomes and tumor growth control. Radiotherapy without surgery is advisable for progressive asymptomatic tumors. Resection followed by irradiation is preferable if total resection without the risk of damage to veins and cortex is impossible.
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Neoplasias Meníngeas , Meningioma , Radiocirurgia , Humanos , Masculino , Feminino , Meningioma/radioterapia , Meningioma/cirurgia , Seio Sagital Superior/patologia , Seio Sagital Superior/cirurgia , Neoplasias Meníngeas/cirurgia , Resultado do Tratamento , Procedimentos Neurocirúrgicos/métodos , Seguimentos , Estudos RetrospectivosRESUMO
Hemangioblastoma is a benign tumor of the central nervous system arising sporadically or as a component of Von Hippel-Lindau disease. Von Hippel-Lindau disease is a rare autosomal dominant hereditary syndrome with various phenotypes caused by VHL gene variants. To date, only about 40 cases of optic nerve hemangioblastoma have been described in the literature. Stereotactic irradiation may be effective for supratentorial hemangioblastomas including lesions of optic nerves. The authors describe a rare case of stereotactic irradiation of intraorbital hemangioblastoma of the optic nerve in a patient with Von Hippel-Lindau disease.
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Hemangioblastoma , Doença de von Hippel-Lindau , Humanos , Doença de von Hippel-Lindau/complicações , Doença de von Hippel-Lindau/cirurgia , Doença de von Hippel-Lindau/genética , Hemangioblastoma/diagnóstico por imagem , Hemangioblastoma/cirurgia , Hemangioblastoma/complicações , Nervo Óptico/patologiaRESUMO
Schwannomatoses is a new classification unit for all the hereditary diseases caused by chromosome 22 damage followed by multiple benign neoplasms of the peripheral and central nervous system. Schwannomatosis occurs as a result of damage to different genes: NF2, SMARCB1, LZRT1, loss of heterozygosity of the long arm of chromosome 22. Nevertheless, clinical manifestations are similar. Molecular diagnostics not only confirms the diagnosis, but also predicts the course of disease. Thus, the most severe clinical manifestations are observed in patients with violation of semantic sequences and reading frame shift in exons 2-13 of the NF2 gene. A more favorable course with less number of tumors is observed in patients with somatic mosaicism. Stereotactic irradiation and surgery are the main treatment options for schwannomatosis. However, there is evidence of effective targeted therapy with bevacizumab (inhibitor of vascular endothelial growth factor). Bevacizumab is used in patients with bilateral vestibular schwannomas and high risk of hearing loss, as well as for intramedullary tumor growth control.
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Neurilemoma , Neurofibromatoses , Neurofibromatose 2 , Humanos , Bevacizumab , Fator A de Crescimento do Endotélio Vascular , Neurilemoma/genética , Neurilemoma/terapia , Neurofibromatoses/genética , Neurofibromatoses/terapia , Neurofibromatoses/diagnóstico , Neurofibromatose 2/genética , Neurofibromatose 2/terapia , Neurofibromatose 2/diagnósticoRESUMO
Meningioma is the most common primary tumor of the central nervous system. Traditional classification is based on histological properties of tumors and distinguishes different grades of meningioma malignancy. However, knowledge about different molecular mechanisms of tumor provided new data on genetic features of meningiomas. The authors analyze current available data on the main driver mutations, new classifications based on molecular genetic characteristics and potential targets for therapy.
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Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/genética , Meningioma/terapia , Neoplasias Meníngeas/genética , Neoplasias Meníngeas/terapia , Biologia MolecularRESUMO
We present the first clinical application of non-invasive stereotaxic radioablation of ventricular tachycardia (VT) refractory to medical and surgical treatment. Based on the results of invasive navigational activation mapping, a pericicatrical zone in the interventricular septum associated with VT was verified. Radiosurgical irradiation of the target in the region of the interventricular septum and the posterior apical segment of the left ventricle was performed on a TrueBeam linear electron accelerator (Varian) in accordance with the segmental scheme of the left ventricle. Irradiation doses for 95% of the internal target volume (ITV, 17 cm3) and planned target volume (PTV, 46 cm3) (31.2 and 25 Gy, respectively) were delivered by two full coplanar arches in 1 session. Irradiation was performed during expiration using a respiratory control system. The loading dose to critical structures was within tolerance. The planned follow-up period is 6 months. According to remote monitoring, the intensity of VT paroxysms over 48 days after treatment was from daily to 2-3 per day. Then, the incidence of VT paroxysms decreased (1-3 per week), and from the 64th to the 185th day (the end of the observation period), no VT paroxysms were recorded, which suggests that the impact was highly precise, conformal, and involved the total wall thickness. No undesirable effects and damage to adjacent organs were observed.
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Radiocirurgia , Taquicardia Ventricular , Septo Interventricular , Humanos , Radiocirurgia/efeitos adversos , Radiocirurgia/métodos , Elétrons , Ventrículos do CoraçãoRESUMO
Treatment of craniovertebral junction meningioma is a difficult task. Surgical treatment is the gold standard for these patients. However, it is associated with high risk of neurological impairment, while combined treatment (surgery + radiotherapy) provides more favorable outcomes. OBJECTIVE: To present the results of surgical and combined treatment of patients with craniovertebral junction meningioma. MATERIAL AND METHODS: There were 196 patients with craniovertebral junction meningioma who underwent surgical or combined (surgery + radiotherapy) treatment at the Burdenko Neurosurgery Center between January 2005 and June 2022. The sample included 151 women and 45 men (3.4:1). Resection of tumor was performed in 97.4% of patients, craniovertebral junction decompression with dural defect closure - 2%, ventriculoperitoneostomy - 0.5%. As the second stage, 40 patients (20.4%) underwent radiotherapy. RESULTS: Total resection was achieved in 106 patients (55.2%), subtotal - 63 (32.8%), partial - 20 (10.4%), tumor biopsy was performed in 3 (1.6%) cases. Intraoperative complications occurred in 8 patients (4%), postoperative complications - in 19 (9.7%) cases. Radiosurgery was carried out in 6 (15%) patients, hypofractionated irradiation - 15 (37.5%), standard fractionation - 19 (47.5%) patients. Tumor growth control after combined treatment made up 84%. CONCLUSION: Clinical outcomes in patients with craniovertebral junction meningioma depend on tumor dimensions, topographic and anatomical localization of tumor, resection quality and relationship with surrounding structures. Combined treatment of anterior and anterolateral meningiomas of the craniovertebral junction is preferable compared to total resection.
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Neoplasias Meníngeas , Meningioma , Neoplasias da Base do Crânio , Masculino , Humanos , Feminino , Meningioma/diagnóstico por imagem , Meningioma/radioterapia , Meningioma/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Neurocirúrgicos/métodos , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Neoplasias da Base do Crânio/cirurgiaRESUMO
Using the example of a recurrent tumor with a 10-year follow-up, the authors show that mutation of the IDH1/2 genes in astrocytomas is not always an early event in the pathogenesis of glioma, that in rare cases a 1p19q codeletion can be found in astrocytomas, and that IDH-mutant tumors can occur in childhood.
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Astrocitoma , Neoplasias Encefálicas , Glioma , Humanos , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Glioma/genética , Astrocitoma/genética , Mutação , Isocitrato Desidrogenase/genéticaRESUMO
INTRODUCTION: Stereotactic radiosurgery is one of the main treatments for vestibular schwannomas (VS). Their feature is frequent post-radiation pseudoprogression. This may be due to hormonal status of patients. OBJECTIVE: To analyze expression of progesterone and estrogen receptors in women and men with VS. MATERIAL AND METHODS: Immunohistochemical analysis of expression of progesterone (PR) and estrogen receptors (ER) after biopsy was performed in 240 patients with VS between 2018 and 2021. ER/PR expression was assessed in men (n=120) and women (n=120) in 3 age subgroups: young age (18-44 years), middle age (45-59 years) and old age (60-79 years). Each subgroup included 40 patients. Statistical analysis was performed using the Mann-Whitney test and MedCalc software. RESULTS: ER expression is not typical for VS (men - 1 (0.01%), women - 3 (2.5%)). At the same time, PR expression was found in 29 (24.2%) men and 21 (17.5%) women. We found no significant difference in expression of ER and PR between men and women. However, variability in PR expression was revealed, i.e. predominance of this indicator in young women (p=0.0463) and middle-aged men (p=0.0110). Expression of PR was similar in elderly patients (p=0.2382). CONCLUSION: The established incidence of PR expression may be one of the probable causes affecting development and duration of VS pseudoprogression after radiosurgery without clear relationship between sex and age. Further prospective research is needed to predict the risks of pseudoprogression.
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Neoplasias da Mama , Neuroma Acústico , Pessoa de Meia-Idade , Masculino , Idoso , Humanos , Feminino , Adolescente , Adulto Jovem , Adulto , Receptores de Progesterona/análise , Receptores de Progesterona/metabolismo , Receptores de Estrogênio/análise , Receptores de Estrogênio/metabolismo , Progesterona , Neuroma Acústico/cirurgia , EstrogêniosRESUMO
Optic nerve sheath meningioma (ONSM) is a slow-growing benign tumor that tends to extend intracranially. The tumor is characterized by vision disturbance, optic nerve atrophy and edema. At present, radiotherapy is recommended as the treatment of choice for ONSM. PURPOSE: To assess the neuro-ophthalmic symptomatology of ONSM and how it changes in response to radiotherapy. MATERIAL AND METHODS: In the period from 2004 to 2018, 112 patients with ONSM aged 17 to 76 years underwent stereotactic radiotherapy. In 91 patients, visual functions varied from light perception to 1.0; in 21 patients the affected eye was blind. The prospective follow-up of 103 patients lasted 6 months to 10 years (mean follow-up duration was 57 months). Studied patients underwent either conventional fractionated radiotherapy with Novalis (n=88) or hypofractionation radiotherapy with CyberKnife (n=24). RESULTS: According to ophthalmological examination, in 60.5% of cases an improvement of visual functions was observed, 39.5% of patients had visual functions remain stable at the initial level. No vision impairment during irradiation was detected. Vision deterioration in the long-term follow-up was observed in 2 patients: extended tumor growth in one case and radio-induced retinopathy in the other. CONCLUSION: The study showed that this method of treatment for ONSM is effective and safe, allowing preservation of visual functions and in some cases - its improvement, while tumor growth remains under control.
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Neoplasias Meníngeas , Meningioma , Neoplasias do Nervo Óptico , Humanos , Meningioma/diagnóstico , Meningioma/radioterapia , Estudos Prospectivos , Fracionamento da Dose de Radiação , Acuidade Visual , Neoplasias do Nervo Óptico/diagnóstico , Neoplasias do Nervo Óptico/radioterapia , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/radioterapia , Nervo Óptico , Resultado do TratamentoRESUMO
Optic nerve glioma is a rather rare tumor. It predominantly arises in pediatric patients, including those with type I neurofibromatosis. This neoplasm is accompanied by decreased visual function and exophthalmos. Treatment strategy is individualized depending on age, volume and spread of tumor, as well as severity of clinical manifestations. Possible treatment options are surgical resection, chemotherapy, radiotherapy and their combination. Radiotherapy can be recommended for patients with intact visual functions, no severe proptosis and trophic lesions. Classic fractionation mode is used as a standard. Currently, the possibility of hypofractionated irradiation is being considered. OBJECTIVE: To evaluate safety and efficacy of hypofractionated radiotherapy in patients with optic nerve glioma. MATERIAL AND METHODS: Sixteen patients with optic nerve gliomas underwent hypofractionated stereotactic irradiation (CyberKnife) between May 2014 and October 2019. Single focal dose was 5.5 Gy. There were 5 fractions up to total focal dose of 27.5 Gy. The sample enrolled 14 children with a median age of 4 years (range 23 months - 13 years) and 2 adults aged 47 and 66 years, respectively. Median of tumor volume was 2.77 cm3 (range 1.69-10.01 cm3). RESULTS: Tumor growth control was achieved in all patients, partial remission was observed in 5 (32%) patients. None patient had deterioration of visual function. Improvement of visual acuity was noted in 3 (19%) cases. Visual field enlargement occurred in 4 (67%) out of 6 patients who were preoperatively examined. After irradiation, proptosis decreased by ≥ 1 mm in 9 (60%) out of 15 patients.
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Exoftalmia , Neoplasias , Glioma do Nervo Óptico , Radiocirurgia , Adulto , Criança , Pré-Escolar , Exoftalmia/etiologia , Humanos , Lactente , Glioma do Nervo Óptico/radioterapia , Glioma do Nervo Óptico/cirurgia , Hipofracionamento da Dose de Radiação , Radiocirurgia/efeitos adversos , Resultado do TratamentoRESUMO
To date, no modern methods of treatment allow overcoming malignant potential of glial neoplasms and significant increase of survival. Analysis of glioblastoma radioresistance using cancer cell cultures is one of the perspective directions, as radiotherapy is standard and available treatment method for these neoplasms. This review summarizes current studies identifying many factors of radioresistance of glial tumors, such as hypoxia, microenvironment and metabolic features of tumor, stem cells, internal heterogeneity of tumor, microRNA, features of cell cycle, DNA damage and reparation. We obtained data on involvement of various molecular pathways in development of radioresistance such as MEK/ERK, c-MYC, PI3K/Akt, PTEN, Wnt, JAK/STAT, Notch, etc. Changes in activity of RAD51 APC, FZD1, LEF1, TCF4, WISP1, p53 and many others are determined in radioresistant cells. Further study of radioresistance pathways will allow development of specific target aptamers and inhibitors.
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Glioblastoma , Glioma , MicroRNAs , Humanos , Técnicas de Cultura de Células , Linhagem Celular Tumoral , Glioblastoma/radioterapia , Glioma/radioterapia , Quinases de Proteína Quinase Ativadas por Mitógeno/metabolismo , Fosfatidilinositol 3-Quinases/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismo , Microambiente Tumoral , Proteína Supressora de Tumor p53 , Tolerância a RadiaçãoRESUMO
Identification of specific alterations in tumors (as a rule, these are mutations or gene fusions) makes it possible to prescribe targeted drugs of the second line of therapy or, in some cases of inoperable tumors, to observe not only a gradual partial response of the tumor to treatment, but also the removal of these patients from the category of incurable ones. The article describes a new rare type of BRAF::EPB41L2 gene fusion detected in a piloid astrocytoma that developed in the posterior cranial fossa in an 11-year-old boy.
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Astrocitoma , Neoplasias Encefálicas , Astrocitoma/genética , Astrocitoma/patologia , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Criança , Comunicação , Proteínas do Citoesqueleto , Fusão Gênica , Humanos , Masculino , Proteínas de Membrana , Proteínas Proto-Oncogênicas B-raf/genéticaRESUMO
Hypothalamic hamartoma (HH) is a dysplastic lesion fused with hypothalamus and followed by epilepsy, precocious puberty and behavioral disorders. Up to 50% of patients become free of seizures after surgery, but various complications occur in 1/4 of cases. Radiofrequency thermocoagulation, laser interstitial thermal therapy and stereotactic radiosurgery (SRS) are alternative treatment options. OBJECTIVE: To define the indications for SRS in patients with HH and to clarify the irradiation parameters. MATERIAL AND METHODS: Twenty-two patients with HH and epilepsy underwent SRS at the Moscow Gamma-knife Center. A retrospective analysis included 19 patients with sufficient follow-up data. Median age of patients was 11.5 years (range 1.3-25.8). The diameter of irradiated HHs ranged between 5.5 and 40.9 mm. In 8 (36%) cases, the volume of hamartoma exceeded 3 cm3. Mean prescribed dose was 18±2.0 Gy, mean prescribed isodose - 48±4.2%. Median follow-up period was 14.8 months (range 3.4 - 96.1). RESULTS: Three (15.8%) patients were free of seizures. One patient (5.3%) improved dramatically after treatment with compete resolution of generalized seizures and experienced only rare emotional seizures (Engel IB). Eleven (57.8%) patients reported lower incidence of seizures. Severity and incidence of seizures were the same in 4 patients (21.1%). The best results were achieved in mean target dose over 20-22 Gy, minimal target dose over 7-10 Gy, covering by the prescribed dose of at least 70-80% of hamartoma volume, as well as in patients with the prescribed dose of 12 Gy delivered to almost entire volume of tumor. None patient had any complications after SRS. CONCLUSION: SRS is safe regarding neurological, endocrine or visual disturbances. Careful patient selection for SRS makes it an effective option for HH-related epilepsy. The best candidates for SRS are children with seizures aged over 1 year, hamartoma <3 cm3 and area of fusion with hypothalamus <150 mm2.
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Epilepsia , Hamartoma , Radiocirurgia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Epilepsia/etiologia , Epilepsia/cirurgia , Hamartoma/complicações , Hamartoma/patologia , Hamartoma/cirurgia , Humanos , Doenças Hipotalâmicas , Lactente , Radiocirurgia/efeitos adversos , Radiocirurgia/métodos , Estudos Retrospectivos , Convulsões , Resultado do Tratamento , Adulto JovemRESUMO
Glioblastoma (GB) is one of the most aggressive primary brain tumors. Analysis of molecular genetic factors affecting prognosis in patients with GB is an important direction of fundamental and clinical researches. There are literature data on the effect of TERT gene mutations, MGMT methylation and IDH1/2 status on overall survival in patients with GB. OBJECTIVE: To evaluate the incidence of TERT gene promoter mutations in adults with primary GB and to analyze the effect of TERT mutations on relapse-free and overall survival, as well as interaction of these mutations with MGMT gene methylation and IDH1/2 mutations. MATERIAL AND METHODS: The study included 56 patients (26 women and 30 men) with histologically verified GB in which genetic and molecular investigations were performed. There were patients with life duration >3 years (n=15) and people with an extremely unfavorable course of disease (14 ones with primary multiple GB, 8 patients with GB metastases including extraaxial and 8 patients with life duration <8 months). TERT gene sequencingwas performed in all the cases, IDH1/2 status was known for 41 patients, MGMT status - for 23 patients. RESULTS: Overall survival significantly differed between patients with and without TERT mutation (56 vs 17 months, p>0.05). TERT gene promoter mutation increased the effect of IDH1/2 mutations on overall and relapse-free survival (p=0.011). No TERT and IDH1/2 gene mutations worsened prognosis. There were no significant differences between TERT status and development of primary multiple GBs, as well as extra- and intracranial metastases. CONCLUSION: Thus, the combined status of IDH1/2 and TERT mutations was a factor of better prognosis and can be proposed in clinical practice.
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Neoplasias Encefálicas , Glioblastoma , Telomerase , Adulto , Biomarcadores Tumorais/genética , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Metilação de DNA , Metilases de Modificação do DNA/genética , Enzimas Reparadoras do DNA/genética , Feminino , Glioblastoma/genética , Glioblastoma/patologia , Humanos , Isocitrato Desidrogenase/genética , Masculino , Mutação/genética , Prognóstico , Regiões Promotoras Genéticas/genética , Telomerase/genética , Proteínas Supressoras de Tumor/genéticaRESUMO
Stereotactic radiosurgery of vestibular schwannoma is an effective and safe method of treatment. The phenomenon of schwannoma pseudo-progression (transient post-radiation enlargement) complicates assessment of the outcomes after radiosurgery. OBJECTIVE: To investigate the changes of vestibular schwannoma in different periods after radiosurgery. MATERIAL AND METHODS: We analyzed early and long-term radiation-induced changes in 333 patients who received Gamma Knife treatment at the Burdenko Neurosurgery Center between April 2005 and December 2015. Mean follow-up period was 60 months (range 15-167). There were 89 men (26.7%) and 244 (73.4%) women. Mean age of patients was 48.2 years. Mean baseline tumor volume was 4.1 cm3 (range 0.1-14.5). Dynamics of changes was assessed using volumetric comparison. RESULTS: Tumor shrinkage without pseudo-progression was observed in 149 (44.7%) patients. Typical pseudo-progression in different variants was found in 131 (39.3%) patients, i.e. short-term (1 year) and long-term (≥2 years) course, complete and incomplete process. Eleven patients had atypical pseudo-progression after initial tumor shrinkage. Progression-free 5- and 10-year survival in the entire group was 87 and 81%, respectively. Progression-free 5-year survival rate was 95 and 92% in patients with and without pseudo-progression, respectively. Ten-year survival rate was 89 and 89%, respectively. CONCLUSION: Knowledge of pseudo-progression features is essential for the most reasonable and reliable assessment of treatment results and justification of timing and frequency of subsequent MR control. Atypical course of pseudo-progression can simulate tumor recurrence. In case of tumor enlargement at any follow-up stage after radiosurgery, advisability of surgery should be determined considering clinical data and likelihood of tumor shrinkage following natural regression of post-radiation tumor enlargement.
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Neuroma Acústico , Radiocirurgia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/radioterapia , Neuroma Acústico/cirurgia , Procedimentos Neurocirúrgicos , Radiocirurgia/efeitos adversos , Radiocirurgia/métodos , Resultado do TratamentoRESUMO
Subarachnoid hemorrhage (SAH) leads to significant long-term cognitive deficits, so-called the post-SAH syndrome. Existing neurological scales used to assess outcomes of SAH are focused on sensory-motor functions. To better evaluate short-term and chronic consequences of SAH, we explored and validated a battery of neurobehavioral tests to gauge the functional outcomes in mice after the circle of Willis perforation-induced SAH. The 18-point Garcia scale, applied up to 4 days, detected impairment only at 24-h time point and showed no significant difference between the Sham and SAH group. A decrease in locomotion was detected at 4-days post-surgery in the open field test but recovered at 30 days in Sham and SAH groups. However, an anxiety-like behavior undetected at 4 days developed at 30 days in SAH mice. At 4-days post-surgery, Y-maze revealed an impairment in working spatial memory in SAH mice, and dyadic social interactions showed a decrease in the sociability in SAH mice, which spent less time interacting with the stimulus mouse. At 30 days after ictus, SAH mice displayed mild spatial learning and memory deficits in the Barnes maze as they committed significantly more errors and used more time to find the escape box but still were able to learn the task. We also observed cognitive dysfunction in the SAH mice in the novel object recognition test. Taken together, these data suggest dysfunction of the limbic system and hippocampus in particular. We suggest a battery of 5 basic behavioral tests allowing to detect neurocognitive deficits in a sub-acute and chronic phase following the SAH.