scholarly journals Radiosurgical treatment of patients with intracranial pilocytic astrocytomas

2021 ◽  
Vol 23 (1) ◽  
pp. 38-46
Author(s):  
Yu. Yu. Trunin ◽  
A. V. Golanov ◽  
A. N. Konovalov ◽  
I. N. Pronin ◽  
R. I. Zagirov ◽  
...  
Keyword(s):  
Tumor Growth ◽  
Radiation Treatment ◽  
Primary Treatment ◽  
Clinical Deterioration ◽  
Study Objective ◽  
National Medical ◽  
Pilocytic Astrocytomas ◽  
Incomplete Removal ◽  
Cerebellar Symptoms

The study objective is to assess the outcomes after stereotactic radiosurgical treatment (SRS) in patients with pilocytic astrocytomas after non-radical surgery and after continued tumor growth.Materials and methods. The report includes 56 patients (37 males and 19 females) who have undergone SRS in N. N. Burdenko National Medical Research Center of Neurosurgery from March 2005 to January 2018.Results. The majority of patients (75 %) were children. Almost 43 % of patients underwent SRS as part of the primary treatment after biopsy or incomplete removal, other patients – in the event of continued tumor growth after non-total surgery. Tumors involved the cerebellum (41 %), brainstem (23 %), thalamus (19.6 %) and cerebral hemispheres (16.1 %). The median tumor volume was 1.9 cm3 (0.14–19.00 cm3), 23 % of patients had cysts in the tumor. The prescribed dose was 12 to 22 Gy (median 18 Gy) over 50–80 % isodose line. The follow-up was available for 54 (96.4 %) patients. The median follow-up was 67 months (3–151 months). All patients were alive at the end of the follow-up examination. In 14 (25 %) patients, the development of pseudo-progression (PSP) was noted. The median detection of PSP is 11 months (3–65 months). Of these, in 7 (50 %) patients PSP was accompanied by clinical deterioration: in 5 – an increase in general cerebral symptoms and in 1 patient – an episode and an increase in cerebellar symptoms. Eight (57 %) patients with PSP were reoperated: 4 underwent removal of the tumor, 4 – emptying of the cyst and placement of the Ommaya reservoir, which led to regression of the existing symptoms. No other toxicity was observed in patients. At the time of the completion of the follow-up examination (with a given median follow-up), no relapses were detected. There was no clinical deterioration after SRS.Conclusion. Radiosurgical treatment is an effective and safe method of radiation treatment for patients with primary pilocytic astrocytomas and recurrent pilocytic astrocytomas, providing control over tumor growth in all patients with a low risk of complications.

A New Era of Organ-Preserving Treatment in Pediatric Intraocular Retinoblastoma in Russia: A Multicenter Cohort Study

2018 ◽  
Vol 5 (1) ◽  
pp. 51-69 ◽  
Author(s):  
Тatiana L. Ushakova ◽  
Igor A. Тrofimov ◽  
Оlga V. Gorovtsova ◽  
Аndrey A. Yarovoy ◽  
Svetlana V. Saakyan ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Systemic Chemotherapy ◽  
Focal Therapy ◽  
Primary Treatment ◽  
Local Chemotherapy ◽  
Multicenter Cohort Study ◽  
Cancer Disease ◽  
Tumor Spread ◽  
Group D

Background.Retinoblastoma (RB) is a life threatening cancer disease. A breakthrough in the treatment of children with RB is associated with the improvement of conservative treatment that was administered in at least one of the two tumor-affected eyes in most bilateral cases, that was chemotherapy both systemic and local (selective intra-arterial and intravitreal) in most cases combined with laser therapy, cryotherapy, or brachytherapy. The development of such techniques as local chemotherapy is focused on preservation of visual functions, reducing the number of enucleations and radiotherapy (RT) course. The success of the healing of RB is closely associated with a multidisciplinary approach to diagnosis and treatment, as well as specialized longterm follow-up clinical examination.Objective.eye and vision preservation against large intraocular tumors with different growth types and localization without the course of remote radiation therapy was the main purpose.Methods.In the period from September 2012 to January 2016, the study enrolled 45 patients with RB when at least one eye had intraocular tumor spread corresponding to the group C or D. According to the ABC international classification, patients have a relatively good prognosis for organ-preserving treatment. 4 of 18 children with bilateral RB had undergone primary enucleation of worse eye the worst eye, group E; 49 (77.8%) of the 63 affected eyes had features for groups C and D. In this study, no patient received local chemotherapy initially, only after prior systemic chemotherapy. Selective intra-arterial chemotherapy (SIAC) was applied to 41 patients (45 eyes; mean course number was 2), and 32 patients (34 eyes) had undergone intravitreal chemo therapy (IViC) (mean course number was 2). Focal therapy and local chemotherapy were the main methods of treatment for progression (new lesions on the retina) in 8 (16.3%) of 49 eyes with tumors of group C (n=1) and D (n=7); the relapse in 14 of 49 (new lesions on the retina) in eyes with tumors of group C (n=5) and D (n=6) and (new lesions on the retina and the vitreous) in eyes with tumors of group D (n=3) (28.5%), and stabilization of disease n=23 (46.9%). We should note that 2 patients underwent repeated course of in case of systemic chemotherapy, 1 patient — a Gamma Knife procedure due to registered disease stabilization, progression or relapse.Results.10 (20.4%) of 49 eyes saved due to the combined chemotherapy. In 45 patients diseasefree survival rate was 56.1±8.9 % (with mean follow-up period 26.9±2.5 months). 1 of 45 patients died from leukemia. 44 of 45 patients are alive without metastasis. The mean follow-up was 20 months (3 to 43 months). Eye salvage rate in group C — 14 (93.3%) of 15, in group D — 31 (91.2%) of 34.Conclusion.These methods: second line of systemic chemotherapy, RT, and a Gamma Knife procedure should be considered as a failure of primary treatment. Our study demonstrated a high efficacy of local chemotherapy with promissing techniques of conservation therapy, which safety increases due to experience.

Gamma knife radiosurgery as a single treatment modality for large cerebral arteriovenous malformations

2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 113-119 ◽  
Author(s):  
D. Hung-Chi Pan ◽  
Wan-Yuo Guo ◽  
Wen-Yuh Chung ◽  
Cheng-Ying Shiau ◽  
Yue-Cune Chang ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Arteriovenous Malformations ◽  
Radiation Treatment ◽  
Gamma Knife Radiosurgery ◽  
Consecutive Series ◽  
Content Type ◽  
Obliteration Rate ◽  
Complete Obliteration ◽  
Fine Print

Object. A consecutive series of 240 patients with arteriovenous malformations (AVMs) treated by gamma knife radiosurgery (GKS) between March 1993 and March 1999 was evaluated to assess the efficacy and safety of radiosurgery for cerebral AVMs larger than 10 cm3 in volume. Methods. Seventy-six patients (32%) had AVM nidus volumes of more than 10 cm3. During radiosurgery, targeting and delineation of AVM nidi were based on integrated stereotactic magnetic resonance (MR) imaging and x-ray angiography. The radiation treatment was performed using multiple small isocenters to improve conformity of the treatment volume. The mean dose inside the nidus was kept between 20 Gy and 24 Gy. The margin dose ranged between 15 to 18 Gy placed at the 55 to 60% isodose centers. Follow up ranged from 12 to 73 months. There was complete obliteration in 24 patients with an AVM volume of more than 10 cm3 and in 91 patients with an AVM volume of less than 10 cm3. The latency for complete obliteration in larger-volume AVMs was significantly longer. In Kaplan—Meier analysis, the complete obliteration rate in 40 months was 77% in AVMs with volumes between 10 to 15 cm3, as compared with 25% for AVMs with a volume of more than 15 cm3. In the latter, the obliteration rate had increased to 58% at 50 months. The follow-up MR images revealed that large-volume AVMs had higher incidences of postradiosurgical edema, petechiae, and hemorrhage. The bleeding rate before cure was 9.2% (seven of 76) for AVMs with a volume exceeding 10 cm3, and 1.8% (three of 164) for AVMs with a volume less than 10 cm3. Although focal edema was more frequently found in large AVMs, most of the cases were reversible. Permanent neurological complications were found in 3.9% (three of 76) of the patients with an AVM volume of more than 10 cm3, 3.8% (three of 80) of those with AVM volume of 3 to 10 cm3, and 2.4% (two of 84) of those with an AVM volume less than 3 cm3. These differences in complications rate were not significant. Conclusions. Recent improvement of radiosurgery in conjunction with stereotactic MR targeting and multiplanar dose planning has permitted the treatment of larger AVMs. It is suggested that gamma knife radiosurgery is effective for treating AVMs as large as 30 cm3 in volume with an acceptable risk.

Stereotactic radiation treatment planning and follow-up studies involving fused multimodality imaging

2004 ◽  
Vol 101 (Supplement3) ◽  
pp. 326-333 ◽  
Author(s):  
Klaus D. Hamm ◽  
Gunnar Surber ◽  
Michael Schmücking ◽  
Reinhard E. Wurm ◽  
Rene Aschenbach ◽  
...  
Keyword(s):  
Image Fusion ◽  
Treatment Planning ◽  
Radiation Treatment ◽  
Data Sets ◽  
Slice Thickness ◽  
Radiation Treatment Planning ◽  
Content Type ◽  
Follow Up Studies ◽  
Fine Print

Object. Innovative new software solutions may enable image fusion to produce the desired data superposition for precise target definition and follow-up studies in radiosurgery/stereotactic radiotherapy in patients with intracranial lesions. The aim is to integrate the anatomical and functional information completely into the radiation treatment planning and to achieve an exact comparison for follow-up examinations. Special conditions and advantages of BrainLAB's fully automatic image fusion system are evaluated and described for this purpose. Methods. In 458 patients, the radiation treatment planning and some follow-up studies were performed using an automatic image fusion technique involving the use of different imaging modalities. Each fusion was visually checked and corrected as necessary. The computerized tomography (CT) scans for radiation treatment planning (slice thickness 1.25 mm), as well as stereotactic angiography for arteriovenous malformations, were acquired using head fixation with stereotactic arc or, in the case of stereotactic radiotherapy, with a relocatable stereotactic mask. Different magnetic resonance (MR) imaging sequences (T1, T2, and fluid-attenuated inversion-recovery images) and positron emission tomography (PET) scans were obtained without head fixation. Fusion results and the effects on radiation treatment planning and follow-up studies were analyzed. The precision level of the results of the automatic fusion depended primarily on the image quality, especially the slice thickness and the field homogeneity when using MR images, as well as on patient movement during data acquisition. Fully automated image fusion of different MR, CT, and PET studies was performed for each patient. Only in a few cases was it necessary to correct the fusion manually after visual evaluation. These corrections were minor and did not materially affect treatment planning. High-quality fusion of thin slices of a region of interest with a complete head data set could be performed easily. The target volume for radiation treatment planning could be accurately delineated using multimodal information provided by CT, MR, angiography, and PET studies. The fusion of follow-up image data sets yielded results that could be successfully compared and quantitatively evaluated. Conclusions. Depending on the quality of the originally acquired image, automated image fusion can be a very valuable tool, allowing for fast (∼ 1–2 minute) and precise fusion of all relevant data sets. Fused multimodality imaging improves the target volume definition for radiation treatment planning. High-quality follow-up image data sets should be acquired for image fusion to provide exactly comparable slices and volumetric results that will contribute to quality contol.

scholarly journals Natural history and treatment options of radiation-induced brain cavernomas: a systematic review

2021 ◽  
Author(s):  
Gildas Patet ◽  
Andrea Bartoli ◽  
Torstein R. Meling
Keyword(s):  
Systematic Review ◽  
Natural History ◽  
Radiation Treatment ◽  
Neurological Deficits ◽  
Cochrane Library ◽  
Average Dose ◽  
Cavernous Malformations ◽  
Radiation Induced ◽  
The Mean

AbstractRadiation-induced cavernous malformations (RICMs) are delayed complications of brain irradiation during childhood. Its natural history is largely unknown and its incidence may be underestimated as RCIMS tend to develop several years following radiation. No clear consensus exists regarding the long-term follow-up or treatment. A systematic review of Embase, Cochrane Library, PubMed, Google Scholar, and Web of Science databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was performed. Based on our inclusion/exclusion criteria, 12 articles were included, totaling 113 children with RICMs, 86 were treated conservatively, and 27 with microsurgery. We were unable to precisely define the incidence and natural history from this data. The mean age at radiation treatment was 7.3 years, with a slight male predominance (54%) and an average dose of 50.0 Gy. The mean time to detection of RICM was 9.2 years after radiation. RICM often developed at distance from the primary lesion, more specifically frontal (35%) and temporal lobe (34%). On average, 2.6 RICMs were discovered per child. Sixty-seven percent were asymptomatic. Twenty-one percent presented signs of hemorrhage. Clinical outcome was favorable in all children except in 2. Follow-up data were lacking in most of the studies. RICM is most often asymptomatic but probably an underestimated complication of cerebral irradiation in the pediatric population. Based on the radiological development of RICMs, many authors suggest a follow-up of at least 15 years. Studies suggest observation for asymptomatic lesions, while surgery is reserved for symptomatic growth, hemorrhage, or focal neurological deficits.

scholarly journals Longitudinal Event-Level Sexual Risk and Substance Use among Gay, Bisexual, and Other Men Who Have Sex with Men

2021 ◽  
Vol 18 (6) ◽  
pp. 3183
Author(s):  
Jordan M. Sang ◽  
Zishan Cui ◽  
Paul Sereda ◽  
Heather L. Armstrong ◽  
Gbolahan Olarewaju ◽  
...  
Keyword(s):  
Substance Use ◽  
Sexual Risk ◽  
Health Study ◽  
Anal Sex ◽  
Study Objective ◽  
Condomless Anal Sex ◽  
Gamma Hydroxybutyrate ◽  
Sex With Men ◽  
Hiv Negative

(1) Background: Condomless anal sex and substance use are associated with STI risk among gay, bisexual, and other men who have sex with men (gbMSM). Our first study objective was to describe event-level sexual risk and substance use trends among gbMSM. Our second study objective was to describe substances associated with event-level sexual risk. (2) Methods: Data come from the Momentum Health Study in Vancouver, British Columbia and participants were recruited from 2012–2015, with follow-up until 2018. Stratified by self-reported HIV status, we used generalized estimating equations to assess trends of sexual event-level substance use and assessed interactions between substance use and time period on event-level higher risk sex defined as condomless anal sex with an HIV serodifferent or unknown status partner. (3) Results: Event-level higher risk anal sex increased across the study period among HIV-negative/unknown (baseline prevalence: 13% vs. study end prevalence: 29%) and HIV-positive gbMSM (baseline prevalence: 16% vs. study end prevalence: 38%). Among HIV-negative/unknown gbMSM, event-level erectile drug use increased, while alcohol use decreased over the study period. Overall, interactions between substance use and time on higher risk anal sex were not statistically significant, regardless of serostatus. However, we found a number of time-specific significant interactions for erectile drugs, poppers, Gamma-hydroxybutyrate (GHB), crystal methamphetamine and ecstasy/MDMA use among HIV-negative/unknown gbMSM. (4) Conclusion: Significant differences in substance use trends and associated risks exist and are varied among gbMSM by serostatus. These findings provide a more comprehensive understanding of the effects of event-level substance use on sexual risk through longitudinal follow-up of nearly six years.

scholarly journals THU0502 EFFICACY AND SAFETY OF SECUKINUMAB TREATMENT IN JUVENILE IDIOPATHIC ARTHRITIS PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 489.3-489
Author(s):  
I. Kriulin ◽  
E. Alexeeva ◽  
T. Dvoryakovskaya ◽  
K. Isaeva ◽  
A. Chomakhidze ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Psoriatic Arthritis ◽  
Response To Therapy ◽  
Inactive Disease ◽  
Hla B27 ◽  
Serious Adverse Events ◽  
National Medical ◽  
Alternative Drug ◽  
Baseline Information

Background:Anti-IL-17A biologic drug secukinumab (SEC) proved to be effective for treatment of psoriatic arthritis. However data about its efficacy in juvenile idiopathic arthritis (JIA) are restricted to off-label experience.Objectives:To evaluate the effectiveness and safety of SEC in JIA patients in the National Medical Research Center of Children`s health, Moscow, Russia.Methods:25 patients started SEC therapy from 12/2017 to 11/2019 in single-center prospective study. 3 patients withdrew treatment: two patients (8%) due to AE (1 - allergy followed by MAS after first injection and 1 – leukopenia) and one patient (4%) – after 10 months of treatment due to secondary inefficacy. Among others, 14 patients which were successfully treated for 6 months or longer were included into analysis. At the baseline, information was collected on the characteristics of the onset of the disease, previous therapy and its success. Patients were monitored at least 1 time per year. At each visit, clinical and laboratory characteristics of JIA severity were assessed. Response to therapy was assessed using the ACRPedi 30/50/70/90 criteria, the C.Wallace criteria for inactive disease (WID) and clinical remission. AEs were assessed at each visit.Results:Among 14 patients received SEC for at least 6 months, 7 (50%) have enthesitis-related arthritis, one (7.1%) – persistent oligoarthritis, 4 (28.6%) – RF-negative polyarthritis, 2 (14.3%) – psoriatic arthritis. 6 patients (42.9%) were HLA-B27 positive. Median age of JIA onset was 8.8 (IQR 5:11), age at SEC initiation – 14 (9.9:16.1), disease duration before SEC start – 3.3 (2.7:5.8). 7 (50%) were biologics-naïve, 2 (14.3%) were previously treated with anti-TNF drug, 5 (35.7%) have 2 or more different biologics in anamnesis.SEC demonstrated high efficacy after the first injection resulting in JADAS-71 decreasing in all patients by median 4.3 (1.6:7.1) points and 7/7/5/2 patients (50%/50%/35.7%/14.3%) achieved ACR Pedi 30/50/70/90 response.After 6 months of treatment, WID was achieved by 7 (50%) patients, JADAS-71 decreased from baseline level 15.2 (12.7:20.5) to 0.8 (0:4.2) points, and 14/13/11/9 patients (100%/92.9%/78.6%/64.3%) achieved ACR Pedi 30/50/70/90 response. One patients who had active uveitis at SEC initiation remained with subactive uveitis; one patient with uveitis remission had not flare episodes during follow-up period. One patient (7.1%) had successfully treated evaluation of transaminases after 4-th injection.Conclusion:Secukinumab showed high effectiveness and safety in children with JIA and can be further used both as a first-line drug in JIA associated with HLA-B27, and as an alternative drug for the ineffectiveness of the standard treatment regimen with biologics. No serious adverse events were registered during follow-up period.Disclosure of Interests:Ivan Kriulin: None declared, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Tatyana Dvoryakovskaya: None declared, Ksenia Isaeva: None declared, Aleksandra Chomakhidze: None declared, Evgeniya Chistyakova: None declared, Olga Lomakina: None declared, Rina Denisova: None declared, Anna Mamutova: None declared, Anna Fetisova: None declared, Marina Gautier: None declared, Dariya Vankova: None declared, Elizaveta Krekhova: None declared, Meyri Shingarova: None declared, Alina Alshevskaya: None declared, Andrey Moskalev: None declared

Management of Recurrent or Progressing Skull Base Chondrosarcomas: Predictors of Long-Term Outcomes

2020 ◽  
Author(s):  
Jonathan D. Breshears ◽  
Franco DeMonte ◽  
Ahmed Habib ◽  
Paul W. Gidley ◽  
Shaan M. Raza
Keyword(s):  
Natural History ◽  
Skull Base ◽  
Radiation Treatment ◽  
Systemic Disease ◽  
Primary Treatment ◽  
Salvage Treatment ◽  
Low Grade ◽  
Related Factors ◽  
Factors Associated ◽  
Initial Recurrence

Abstract Background Skull base chondrosarcomas (CSA) are difficult tumors to cure and there is little data regarding salvage therapy. Objective This study aims to identify presentation and treatment-related factors which impact the progression free survival (PFS) and disease specific survival (DSS) for recurrent CSA, and to identify salvage treatment factors associated with successful restoration to the natural history following primary treatment. Methods This single-institution retrospective review included patients with recurrent/progressive CSA over a 25-year period. Survival analysis for factors impacting PFS and DSS was performed. Salvage treatment factors associated with achieving PFS ≥newly diagnosed median PFS were identified using univariate statistics. Analysis was performed on first recurrences and all recurrences combined. Results A total of 47 recurrence/progression events were analyzed from 17 patients (median two events/patient, range = 1–8). The overall PFS and DSS for the initial recurrence was 32 (range = 3–267) and 79 (range = 3–285) months, respectively. Conventional grade III or mesenchymal histology significantly predicted shorter PFS and DSS (p < 0.0001). After stratification by histology, previous radiation predicted shorter PFS for low-grade tumors (p = 0.009). Gross total resection (GTR) after a first time recurrence was significantly associated with successful salvage treatment (p < 0.05); however, this was rare. Conclusion In this series, high grade histology and prior radiation treatment negatively impacted salvage treatment outcomes, while GTR was associated with restoration to natural history following primary treatment. Careful consideration of histology, systemic disease status, previous treatments, and the anatomic extent of the skull base disease can optimize the outcomes of salvage intervention.

scholarly journals Changes in hypertension control in a community-based population of older adults, 2011-2013 to 2016-2017

2020 ◽  
Author(s):  
Kathryn Foti ◽  
Kunihiro Matsushita ◽  
Silvia Koton ◽  
Keenan A Walker ◽  
Josef Coresh ◽  
...  
Keyword(s):  
Older Adults ◽  
Baseline Risk ◽  
Treatment Goals ◽  
Hypertension Control ◽  
Community Based ◽  
Study Objective ◽  
Atherosclerosis Risk In Communities ◽  
Black And White ◽  
Aric Study

Abstract Background The 2014 hypertension guideline raised treatment goals in older adults. The study objective was to examine changes in blood pressure (BP) control (&lt;140/90 mmHg) from 2011-2013 to 2016-2017 among Black and white older adults with treated hypertension. Methods Participants were 1600 white and 650 Black adults aged 71-90 years in the Atherosclerosis Risk in Communities (ARIC) Study with treated hypertension in 2011-2013 (baseline) who had BP measured in 2016-2017 (follow up). Factors associated with changes in BP control were examined by race. Results BP was controlled among 75.3% of white and 65.7% of Black participants at baseline and 59.0% of white and 56.5% of Black participants at follow up. Among those with controlled BP at baseline, risk factors for incident uncontrolled BP included age (RR 1.15 per 5 years, 95% CI 1.07-1.25), female sex (RR 1.36, 95% CI 1.16-1.60), and chronic kidney disease (CKD) (RR 1.19, 95% CI 1.01-1.40) among white participants, and hypertension duration (RR 1.14 per 5 years, 95% CI 1.03-1.27) and diabetes (RR 1.48, 95% CI 1.15-1.91) among Black participants. Among those with uncontrolled BP at baseline, white females vs males (RR 0.60, 95% CI 0.46-0.78) and Black participants with CKD vs without (RR 0.58, 95% CI 0.36-0.93) were less likely to have incident controlled BP. Conclusions BP control decreased among white and Black older adults. Black individuals with diabetes or CKD were less likely to have controlled BP at follow up. Higher treatment goals may have contributed to these findings and unintended differences by race.

scholarly journals Disease course of Neurofibromatosis Type 2; a 30-year follow-up study of 353 patients seen at a single institution

2020 ◽  
Author(s):  
Claire Forde ◽  
Andrew T King ◽  
Scott A Rutherford ◽  
Charlotte Hammerbeck-Ward ◽  
Simon K Lloyd ◽  
...  
Keyword(s):  
De Novo ◽  
Radiation Treatment ◽  
Univariate Analysis ◽  
Age At Onset ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Disease Course ◽  
Number Of Patients

Abstract Background Limited data exists on the disease course of Neurofibromatosis Type 2 (NF2) to guide clinical trial design. Methods A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990–2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred and inheritance type. Interventions for NF2-related tumours were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death. Results Three-hundred-and-fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring 60 patients (17.0%) died. The annual mean number of patients undergoing VS surgery or radiotherapy declined, from 4.66 and 1.65 respectively per 100 NF2 patients in 1990-1999 to 2.11 and 1.01 in 2010-2020, as the number receiving bevacizumab increased (2.51 per 100 NF2 patients in 2010-2020). Five patients stopped bevacizumab to remove growing meningioma or spinal schwannoma. 153/353 (43.3%) had at least one neurosurgical intervention/radiation treatment within 5 years of diagnosis. Patients asymptomatic at diagnosis had longer time to intervention and better survival compared to those presenting with symptoms. Those symptomatically presenting &lt;16 and &gt;40 years had poorer overall survival than those presenting at 26-39 years (P=0.03 and P=0.02 respectively) but those presenting between 16-39 had shorter time to VS intervention. Individuals with de novo constitutional variants had worse survival than those with de novo mosaic or inherited disease (P=0.004). Conclusion Understanding disease course improves prognostication, allowing for better informed decisions about care.

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