scholarly journals POS0834 LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 670.2-671
Author(s):  
M. Kreuter ◽  
F. Bonella ◽  
K. Kathrin ◽  
J. Henes ◽  
E. Siegert ◽  
...  

Background:Gastroesophageal reflux disease (GERD) occurs frequently in patients with systemic sclerosis (SSc) and SSc-associated interstitial lung disease (SSc-ILD). PPI use has to been shown to improve survival in patients with idiopathic pulmonary fibrosis, whereas to date there are no data on the use of PPI in SSc-ILD.Objectives:This study was aimed to assess whether use of PPI is associated with progression of SSc-ILD and survival.Methods:We retrospectively analysed 1931 patients with SSc and SSc-ILD from the German Network for Systemic Sclerosis (DNSS) database (2003 onwards). Kaplan–Meier analysis compared overall survival (OS) and progression-free survival (PFS) in patients with vs. without GERD (SSc and SSc-ILD), and PPI vs. no PPI use (SSc-ILD only). Progression was defined as a decrease in either % predicted forced vital capacity ≥10% or single-breath diffusing capacity for carbon monoxide ≥15%, or death.Results:GERD was not associated with decreased OS or PFS in patients with either SSc or SSc-ILD. In patients with SSc-ILD, PPI use was associated with improved OS vs. no PPI use after 1 year (98.4% [95% confidence interval: 97.6–99.3]; n=760 vs. 90.8% [87.9–93.8]; n=290) and after 5 years (91.4% [89.2–93.8]; n=357 vs. 70.9% [65.2–77.1]; n=106; p<0.0001). PPI use was also associated with improved PFS vs. no PPI use after 1 year (95.9% [94.6–97.3]; n=745 vs. 86.4% [82.9–90.1]; n=278) and after 5 years (66.8% [63.0–70.8]; n=286 vs. 45.9% [39.6–53.2]; n=69; p<0.0001).Conclusion:GERD had no effect on survival in SSc or SSc-ILD. PPIs improved survival in patients with SSc-ILD; however, controlled, prospective trials are needed to confirm this finding.Disclosure of Interests:Michael Kreuter Speakers bureau: Boehringer, Consultant of: Boehringer, Grant/research support from: Boehringer, Francesco Bonella Speakers bureau: Boehringer, Roche, GSK, Consultant of: Boehringer, Roche, GSK, Grant/research support from: Boehringer, Kuhr Kathrin: None declared, Jörg Henes Speakers bureau: Abbvie, Boehringer, Chugai, Roche, Janssen, Novartis, SOBI, Pfizer and UCB, Consultant of: Boehringer, Celgene, Chugai, Roche, Janssen, Novartis, SOBI, Grant/research support from: Chugai, Roche, Janssen, Novartis, SOBI, Pfizer, Elise Siegert: None declared, Gabriela Riemekasten Speakers bureau: Novartis, Janssen, Roche, GSK, Boehringer, Consultant of: Janssen, Actelion, Boehringer, Norbert Blank Consultant of: Sobi, Novartis, Roche, UCB, MSD, Pfizer, Actelion, Abbvie, Boehringer, Grant/research support from: Novartis, Sobi, Christiane Pfeiffer: None declared, Ulf Müller-Ladner: None declared, Alexander Kreuter Speakers bureau: MSD, Boehringer, InfectoPharm, Paid instructor for: MSD, PETER KORSTEN Consultant of: Glaxo, Abbvie, Pfizer, BMS, Chugai, Sanofi, Lilly, Boehringer, Novartis, Grant/research support from: Glaxo, Aaron Juche: None declared, Marc Schmalzing Speakers bureau: Chugai Roche, Boehringer, Celgene, Medac, UCB, Paid instructor for: Novartis, Abbvie, Astra Zeneca, Chugai Roche, Janssen, Consultant of: Chugai Roche, Hexal Sandoz, Gilead, Abbvie, Janssen, Boehringer, Margitta Worm Speakers bureau: Boehringer, Ilona Jandova Speakers bureau: Boehringer, Novartis, Abbvie, Laura Susok Speakers bureau: MSD, Novartis, BMS, Sunpharma, Consultant of: MSD, Tim Schmeiser Consultant of: Abbvie, Boehringer, Novartis, UCB, Claudia Guenther Paid instructor for: Advisory Board Boehringer January 2020, Employee of: Novartis 2002-2005, Gernot Keyszer Consultant of: Boehringer, Jan Ehrchen Speakers bureau: Boehringer, Janssen, Chugai, Sobi, Employee of: Pfizer, Actelion (now Janssen), Andreas Ramming Speakers bureau: Boehringer, Gilead, Janssen, Pfizer, Roche, Consultant of: Boehringer, Pfizer, Grant/research support from: Novartis, Pfizer, Ina Kötter Speakers bureau: several companies, Consultant of: several companies, Grant/research support from: several companies, Hanns-Martin Lorenz Speakers bureau: Abbvie, Astra Zeneca, Actelion, Alexion Amgen, Bayer Vital, Baxter, Biogen, Boehringer, BMS, Celgene, Fresenius, Genzyme, GSK, Gilead, Hexal, Janssen, Lilly, Medac, MSD, Mundipharm, Mylan, Novartis, Octapharm, Pfizer, Roche Chugai, Sandoz, Sanofi, Shire SOBI, Thermo Fischer, UCB, Grant/research support from: basic research studies: Pfizer, Novartis, Abbvie, Gilead, Lilly, MSD, Roche Chugai, Pia Moinzadeh Speakers bureau: Boehringer, Actelion, Grant/research support from: Actelion, Nicolas Hunzelmann Speakers bureau: Boehringer Janssen, Roche, Sanofi, Consultant of: Boehringer


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1109.2-1110
Author(s):  
M. G. Lazzaroni ◽  
C. Campochiaro ◽  
E. Marasco ◽  
J. De Vries-Bouwstra ◽  
F. Franceschini ◽  
...  

Background:The main clinical associations of anti-PM/Scl in Systemic Sclerosis (SSc) so far reported include calcinosis, myositis and interstitial lung disease (ILD). Nevertheless, data regarding the long-term outcome of ILD in these patients are lacking. A single centre Spanish cohort reported a better functional outcome in 14 SSc-ILD patients anti-Pm/Scl+ as compared to 49 anti-Topo I after a mean follow-up of 7 years (1).Objectives:To analyze the long-term outcome of ILD in a large multicentre EUSTAR study dedicated to anti-Pm/Scl SSc patients.Methods:A case-control study within the EUSTAR cohort collected 165 anti-PM/Scl+ SSc cases and 257 anti-PM/Scl- SSc controls, matched for sex, cutaneous subset, disease duration, and age at onset. Data for ILD at HRCT were available for 162/165 cases and 249/257 controls. Data for pulmonary function tests (PFT) at the baseline (T0), 1 year after diagnosis (T1) and at the last visit (LV) were analyzed.Results:A significantly higher frequency of ILD was reported in anti-Pm/Scl+ cases vs anti-Pm/Scl- controls (62.3% vs 39.4%, p:<0.0001, OR 95%, CI 2.55, 1.70-3.83). Complete PFTs data were available for 81/101 ILD anti-Pm/Scl+ cases and 78/98 anti-Pm/Scl- ILD controls, with similar age at onset and female/male ratio and disease duration at LV (112±81 months vs. 115±64 months, p:0.77). Diffuse cutaneous involvement was less frequent in cases than in controls (27.2% vs. 44.9%, p:0.03).In ILD cases, %pFVC tended to improve from T0 (85.1±18.3) to T1 (89.5±16.5, p:0.045) and to LV (87.9±16.9, p:0.057), while in ILD controls remained stable from T0 (90.4±18.5) to T1 (91.1±16.5, p:0.38) and significantly declined to LV (85.0±18.0, p:0.0002). %pDLCO remained stable from T0 (60.5±16.8) to T1 (60.1±17.6, p:0.87) and to LV (60.4±16.9, p:0.77) in ILD cases, while significantly declined from T0 (67.0±18.9) to T1 (62.7±18.2, p:0.0016) and to LV (59.6±18.4, p<0.0001) in the control group. Mean %pFVC and %pDLCO at the 3 time points were not significantly different between the two groups.Delta %pFVC (LV-T0) was 2.85±11.3 for the anti-Pm/Scl+ group vs -5.42±13.4 in the control group (p:0.0004) with a significant smaller proportion of patients with FVC loss ≥10% from T0 to LV in the anti-PM/Scl group (12.3% vs. 39.7%, p:0.0001). Delta %pDLCO (LV-T0) was -0.13±10.8 for the anti-PM/Scl+ group vs -7.38±14.6 in the control group (p:0.0015), with a significant smaller proportion of patients with DLCO loss ≥10% from T0 to LV in the anti-PM/Scl+ group (13.6% vs. 42.3%, p<0.0001).Conclusion:In this multicenter real-life study, the long-term pulmonary functional outcome in SSc-ILD patients with anti-Pm/Scl positivity seems to be more favorable than in patients without anti-Pm/Scl antibodies.References:[1]Guillen-Del Castillo A, Semin Arthritis Rheum 2014, 44 (3), 331-7.Disclosure of Interests: :Maria Grazia Lazzaroni: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Emiliano Marasco: None declared, Jeska de Vries-Bouwstra: None declared, Franco Franceschini: None declared, Francesco Del Galdo: None declared, Christopher Denton Grant/research support from: GlaxoSmithKline, CSL Behring, and Inventiva, Consultant of: Medscape, Roche-Genentech, Actelion, GlaxoSmithKline, Sanofi Aventis, Inventiva, CSL Behring, Boehringer Ingelheim, Corbus Pharmaceuticals, Acceleron, Curzion and Bayer, Lorenzo Cavagna: None declared, Oliver Distler Grant/research support from: Grants/Research support from Actelion, Bayer, Boehringer Ingelheim, Competitive Drug Development International Ltd. and Mitsubishi Tanabe; he also holds the issued Patent on mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143)., Consultant of: Consultancy fees from Actelion, Acceleron Pharma, AnaMar, Bayer, Baecon Discovery, Blade Therapeutics, Boehringer, CSL Behring, Catenion, ChemomAb, Curzion Pharmaceuticals, Ergonex, Galapagos NV, GSK, Glenmark Pharmaceuticals, Inventiva, Italfarmaco, iQvia, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Roche, Sanofi and UCB, Speakers bureau: Speaker fees from Actelion, Bayer, Boehringer Ingelheim, Medscape, Pfizer and Roche, Yannick Allanore Grant/research support from: BMS, Inventiva, Roche, Sanofi, Consultant of: Actelion, Bayer AG, BMS, BI, Paolo Airò: None declared



2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii367-iii367
Author(s):  
Nongnuch Sirachainan ◽  
Attaporn Boongerd ◽  
Samart Pakakasama ◽  
Usanarat Anurathapan ◽  
Ake Hansasuta ◽  
...  

Abstract INTRODUCTION Low grade glioma (LGG) is the most common central nervous system (CNS) tumor in children accounted for 30–50%. Regarding benign characteristic of disease, surgical management remains the mainstay of treatment. However, surgical approach is limited in some conditions such as location at brainstem or infiltrative tumor. Chemotherapy and radiation treatments have been included in order to control tumor progression. The 5-years survival rate is approach 90% especially in patients who receive complete resection. However, the outcome of children with LGG in low to middle income is limited. Therefore, the aim of the study was to determine long-term outcome of children with newly diagnosed LGG. METHODS A retrospective study enrolled children aged &lt;18 years who were newly diagnosed LGG during January 2006- December 2019. Diagnosis of LGG was confirmed by histological findings of grade I and II according to WHO criteria. RESULTS A total of 40 patients, female to male ratio was 1:1.35 and mean (SD) for age was 6.7 (4.0) years. The most common location was optic chiasmatic pathway (42.5%), followed by suprasellar region (25.0%). Sixty percent of patients received at least partial tumor removal. Chemotherapy and radiation had been used in 70% and 10.0% respectively. The 10-year progression free survival was 74.1±11.4% and overall survival was 96.2±3.8%. SUMMARY: Treatment of Pediatric LGG mainly required surgical management, however, chemotherapy and radiation had been used in progressive disease. The outcome was excellent.



2010 ◽  
Vol 6 (2) ◽  
pp. 145-149 ◽  
Author(s):  
Kyung Sun Song ◽  
Ji Hoon Phi ◽  
Byung-Kyu Cho ◽  
Kyu-Chang Wang ◽  
Ji Yeoun Lee ◽  
...  

Object Glioblastoma is the most common primary malignant brain tumor; however, glioblastoma in children is less common than in adults, and little is known about its clinical outcome in children. The authors evaluated the long-term outcome of glioblastoma in children. Methods Twenty-seven children were confirmed to have harbored a glioblastoma between 1985 and 2007. The clinical features and treatment outcomes were reviewed retrospectively. All patients underwent resection; complete resection was performed in 12 patients (44%), subtotal resection in 12 patients (44%), and biopsy in 3 patients (11%). Twenty-four patients (89%) had radiation therapy, and 14 (52%) patients received chemotherapy plus radiation therapy. Among the latter, 5 patients had radiation therapy concurrent with temozolomide chemotherapy. Four patients with small-size recurrent glioblastoma received stereotactic radiosurgery. Results The median overall survival (OS) was 43 months, and the median progression-free survival was 12 months. The OS rate was 67% at 1 year, 52% at 2 years, and 40% at 5 years. The median OS was significantly associated with tumor location (52 months for superficially located tumors vs 7 months for deeply located tumors; p = 0.017) and extent of removal (106 months for completely resected tumors vs 11 months for incompletely resected tumors; p < 0.0001). Conclusions The prognosis of glioblastoma is better in children than in adults. Radical resection followed by concurrent chemoradiation therapy may be the initial treatment of choice.



Blood ◽  
1996 ◽  
Vol 88 (6) ◽  
pp. 1954-1959 ◽  
Author(s):  
MS Tallman ◽  
D Hakimian ◽  
AW Rademaker ◽  
C Zanzig ◽  
E Wollins ◽  
...  

Although 2-chlorodeoxyadenosine (2-CdA) is effective in inducing complete remissions (CRs) in the majority of patients with hairy cell leukemia (HCL), neither the actual relapse rate, the clinical factors that may predict relapse, the long-term outcome, nor the response rate to re-treatment at relapse has been clearly determined. Fifty-two consecutive patients with previously untreated or treated HCL were treated with 2-CdA at a dose of 0.1 mg/kg/d by continuous intravenous infusion for 7 days. Of 50 assessable patients, 40 (80%) achieved CR, and 9 (18%) achieved partial remission (PR). A total of 7 patients (14%) have relapsed, at a median duration of 24 months (range, 12 to 44). Of the 7 relapsed patients, 5 were re-treated with a second cycle of 2-CdA; 2 achieved a second CR and 3 attained a PR. The progression- free survival (PFS) rate is 72% at 4 years for all 52 patients and 83% for patients achieving CR. The overall survival (OS) rate is 86% at 4 years. Only prior therapy was predictive of relapse. The majority of patients achieve durable CRs with a single cycle of 2-CdA. The relapse rate is low and the long-term prognosis is excellent. The few patients who relapse can attain second remissions after re-treatment with 2-CdA.



2021 ◽  
Vol 35 (2) ◽  
pp. 140-146
Author(s):  
Lima Asrin Sayami ◽  
Al Fazir Omar ◽  
Sheikh Ziarat Islam ◽  
Subasni Govindan ◽  
Zulaikha Zainal ◽  
...  

Objective: Despite the evolution of interventional techniques and operator experience, percutaneous revascularization of complex coronary lesions especially calcified lesions remains challenging because of lower procedural success and higher restenosis rates. Limited data are available on the effect of rotational atherectomy (RA) plus stenting in the treatment of complex calcified lesions of coronary artery disease. This study was aimed to investigate the characteristics, short and long term outcomes in patients undergoing RA. Material and Methods: A database search was performed from the year 2008 to 2013 in National Heart institute, Malaysia. A total of 16009 patients who underwent PCIs were enrolled in 2 groups, RA group (258 patients) and non RA group (15751 patients). The Chi square test and Kaplan - Meier analysis were used. Results: Male patients (73.6%) and elderly population (63.2%) were predominant in this study.The RA group had more co-morbidities such as diabetic on insulin (34%) and chronic kidney disease (57%). The lesions in RA group were more complex with higher Type C lesion (68.8%) and longer lesion (20.6%) compared to non RA group. Despite higher patient risk profile, the success rate of revascularization remains high in RA group (99.3%) as in non RA group (97%) (p value 0.89%). More importantly there were no significant difference in in-hospital mortality, myocardial infarction and stent thrombosis in both group (p value 0.1). In 1 year Kaplan - Meier survival graph, there were better survival noted in non RA group (97.7%) compare to RA (89.6%) (p value <0.005), Conclusion: The use of RA allows debulking of a calcified lesion and possibly explains the higher acute procedural success rates. However, the lower 1-yearsurvival in the RA group highlights the higher associated baseline comorbitidity in this group. Therefore, besides coronary intervention, this RA group requires aggressive medical therapy through a multi-disciplinary approach. Bangladesh Heart Journal 2020; 35(2) : 140-146



2019 ◽  
Vol 105 (3) ◽  
pp. e660-e675 ◽  
Author(s):  
Ulf Elbelt ◽  
Sven M Schlaffer ◽  
Michael Buchfelder ◽  
Ulrich J Knappe ◽  
Greisa Vila ◽  
...  

Abstract Context Despite growing evidence that temozolomide (TMZ) therapy is effective for the treatment of aggressive pituitary tumors (APTs) or carcinomas (PCs), individual therapy decisions remain challenging. Objective We therefore aimed to report on clinical characteristics leading to initiation of TMZ therapy and to add evidence on TMZ long-term effectiveness. Design and subjects Retrospective survey on TMZ treatment in patients with APTs or PCs. TMZ therapy was initiated in 47 patients (22 females) with APTs (n = 34) or PCs (n = 13). Mean age at diagnosis was 45 ± 15 years. The immunohistochemical subtypes were corticotroph (n = 20), lactotroph (n = 18), and nonfunctioning (n = 9) tumors. TMZ therapy started 8 years after initial diagnosis using a standard regimen (median 6 cycles) for the majority of patients. Results Long-term radiological response to TMZ after a median follow-up of 32 months with 4 patients still on TMZ therapy was tumor regression for 9 (20%), stable disease for 8 (17%), and tumor progression for 29 patients (63%) (outcome data available for 46 patients). Progression occurred 16 months after initiation of TMZ. Median estimated progression-free survival was 23 months. Disease stabilization and median progression-free survival did not differ between patients with APTs or PCs. Predictors of tumor response were not identified. Overall, TMZ was well tolerated. Conclusion We performed a nationwide survey on TMZ therapy in patients with APTs and PCs. While early response rates to TMZ are promising, long-term outcome is less favorable. Prolonged TMZ administration should be considered. We were not able to confirm previously reported predictors of tumor response to TMZ.



2009 ◽  
Vol 110 (2) ◽  
pp. 319-326 ◽  
Author(s):  
Behzad Eftekhar ◽  
Mohammad Ali Sahraian ◽  
Banafsheh Nouralishahi ◽  
Ali Khaji ◽  
Zahra Vahabi ◽  
...  

Object The goal of this paper was to investigate the long-term outcome and the possible prognostic factors that might have influenced the persistence of posttraumatic epilepsy after penetrating head injuries sustained during the Iraq–Iran war (1980–1988). Methods In this retrospective study, the authors evaluated 189 patients who sustained penetrating head injury and suffered posttraumatic epilepsy during the Iraq–Iran war (mean 18.6 ± 4.7 years after injury). The probabilities of persistent seizures (seizure occurrence in the past 2 years) in different periods after injury were estimated using the Kaplan-Meier method. The possible prognostic factors (patients and injury characteristics, clinical findings, and seizure characteristics) were studied using log-rank and Cox regression analysis. Results The probability of persistent seizures was 86.4% after 16 years and 74.7% after 21 years. In patients with < 3 pieces of shrapnel or no sphincter disturbances during seizure attacks, the probability of being seizure free after these 16 and 21 years was significantly higher. Conclusions Early seizures, prophylactic antiepileptics drugs, and surgical intervention did not significantly affect long-term outcome in regard to persistence of seizures.



2018 ◽  
Vol 100-B (3) ◽  
pp. 338-345 ◽  
Author(s):  
C. E. L. Watkins ◽  
D. W. Elson ◽  
J. W. K. Harrison ◽  
J. Pooley

Aim The aim of this study was to report the long-term outcome and implant survival of the lateral resurfacing elbow (LRE) arthroplasty in the treatment of elbow arthritis. Patients and Methods We reviewed a consecutive series of 27 patients (30 elbows) who underwent LRE arthroplasty between December 2005 and January 2008. There were 15 women and 12 men, with a mean age of 61 years (25 to 82). The diagnosis was primary hypotrophic osteoarthritis (OA) in 12 patients (14 elbows), post-traumatic osteoarthritis (PTOA) in five (five elbows) and rheumatoid arthritis (RA) in ten patients (11 elbows). The mean clinical outcome scores including the Mayo Elbow Performance Score (MEPS), the American Shoulder and Elbow Surgeons elbow score (ASES-e), the mean range of movement and the radiological outcome were recorded at three, six and 12 months and at a mean final follow-up of 8.3 years (7.3 to 9.4). A one sample t-test comparing pre and postoperative values, and survival analysis using the Kaplan–Meier method were undertaken. Results A statistically significantly increased outcome score was noted for the whole group at each time interval. This was also significantly increased at each time in each of the subgroups (OA, RA, and PTOA). Implant survivorship was 100%. Conclusion We found that the LRE arthroplasty, which was initially developed for younger patients with osteoarthritis, is an effective form of surgical treatment for a wider range of patients with more severe degenerative changes, irrespective of their cause. It is therefore a satisfactory alternative to total elbow arthroplasty (TEA) and has lower rates of complications in the subgroups of patients we have studied. It does not require activities to be restricted to the same extent as following TEA. Based on this experience, we now recommend LRE arthroplasty rather than TEA as the primary form of implant for the treatment of patients with OA of the elbow. Cite this article: Bone Joint J 2018;100-B:338–45.



2021 ◽  
Author(s):  
Sukwoo Hong ◽  
Kenji Kagawa ◽  
Kengo Sato ◽  
Ryutaro Nomura ◽  
Shunsuke Ichi

Abstract Background The long-term outcomes of CyberKnife-based hypofractionated stereotactic radiotherapy (SRT) for intra/ extracranial non-vestibular schwannomas (nVS) need to be accumulated. Method Patients who received SRT by CyberKnife for nVS from 2010 to 2019 were retrospectively reviewed. Results A total of 45 patients with nVS were identified. The mean age was 53 (± 18) years old, and 23 patients (51%) were female. Twenty-nine patients (64%) had previous procedures. As for the tumor extension, 22 (49%) nVS were classified as primary intracranial, five (11%) were classified as intra/ extracranial (dumbbell shape), and 18 (40%) were classified as primary extracranial. The median prescribed dose, covering 95% of the planning target volume, was 21 (IQR 21 – 25) Gy, and the median target volume was 7 (IQR 3.6-13.1) cm3. The local control rate of nVS for patients without neurofibromatosis type 2 (NF2) was 100%. Old age (OR 0.92, p-value 0.03) and previous surgery (OR 0.02, p-value 0.02) were significant risk factors for no symptomatic improvement. The progression-free survival was 74 (±33) months clinically and 69 (IQR 36 – 94) months radiologically. During follow-up, two cases (4%) with NF2 resulted in treatment failure, 13 cases (41%) resulted in transient tumor expansion (TTE), 10 (22%) suffered from transient adverse radiation effect (ARE), and two (4%) resulted in permanent ARE. Conclusions Hypofractionated SRT for head, neck, and spine nVS was an effective treatment regardless of tumor extension relative to the cranium. Although the risk of permanent ARE was low, some patients experienced transient clinical worsening due to TTE.



2022 ◽  
Author(s):  
Sukwoo Hong ◽  
Kenji Kagawa ◽  
Kengo Sato ◽  
Ryutaro Nomura ◽  
Shunsuke Ichi

Abstract The long-term outcomes of CyberKnife-based hypofractionated stereotactic radiotherapy (SRT) for intra/ extracranial non-vestibular schwannomas (nVS) need to be accumulated. Patients who received SRT by CyberKnife for nVS from 2010 to 2019 were retrospectively reviewed. A total of 45 patients with nVS were identified. The mean age was 53 (± 18) years old, and 23 patients (51%) were female. Twenty-nine patients (64%) had previous procedures. As for the tumor extension, 22 (49%) nVS were classified as primary intracranial, five (11%) were classified as intra/ extracranial (dumbbell shape), and 18 (40%) were classified as primary extracranial. The median prescribed dose, covering 95% of the planning target volume, was 21 (IQR 21 – 25) Gy, and the median target volume was 7 (IQR 3.6-13.1) cm3. The local control rate of nVS for patients without neurofibromatosis type 2 (NF2) was 100%. Old age (OR 0.92, p-value 0.03) and previous surgery (OR 0.02, p-value 0.02) were significant risk factors for no symptomatic improvement. The progression-free survival was 74 (±33) months clinically and 69 (IQR 36 – 94) months radiologically. During follow-up, two cases (4%) with NF2 resulted in treatment failure, 13 cases (41%) resulted in transient tumor expansion (TTE), 10 (22%) suffered from transient adverse radiation effect (ARE), and two (4%) resulted in permanent ARE. Hypofractionated SRT by CyberKnife for head, neck, and spine nVS was an effective treatment regardless of tumor extension relative to the cranium. Although the risk of permanent ARE was low, some patients experienced transient clinical worsening due to TTE.



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