scholarly journals ATRT-06. RESULTS OF MULTICENTER TRIAL CONCERNING THE TREATMENT OF CHILDREN WITH ATYPICAL TERATOID RHABDOID TUMORS OF THE CENTRAL NERVOUS SYSTEM UNDER 3 YEARS OLD

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i2-i2
Author(s):  
Zheludkova Olga ◽  
Olkhova Liudmila ◽  
Zubarovskaya Ludmilla ◽  
Dinikina Julia ◽  
Smirnova Anna ◽  
...  

Abstract Objective To evaluate the prognostic factors in children with AT/RT aged under 3 years. Patients and methods: The prognostic factors were analyzed in 106patients under 3 years who got treatment and follow-up from 2008 to 2020.There were 41children younger than 12 months and 65patients older than 12 months. The location of the tumor was infratentorial in 58 patients, supratentorial in 46, and spinal cord in 2. There were 54 boys and 52 girls. Among the patients,57 had stage M0,36 had stage М+ or a multifocal tumor, and 13 had stage Mx. All the patients had undergone surgical treatment: total tumor removal in 27, subtotal-33, partial-42, biopsy - 4;67patients had got chemoradiotherapy according to the ATRT-2006(IRS III) protocol; 15, MUV-ATRT protocol; 3, CWS protocol; 9, EU-RHAB protocol; 6, HIT-SKK protocol; and 6 according to individual treatment schemes.12 patients received HDC with AuHCR. Results 47 are alive,1 was lost to follow-up, and 58 died:52 of progressive disease,6 of chemotherapy complications. The five-year PFS was 0.27; the five-year OS was 0.40. The PFS was significantly better in patients older than 12months old compared to patients under 12months: 0.33 and 0.17, respectively; p=0.0047. The PFS after total resection was higher than after subtotal resection, partial resection, and tumor biopsy: 0.51, 0.29, 0.09, and 0%, respectively (p=0.025).The PFS after radiotherapy was markedly higher compared to patients without radiotherapy: 0.63 and 0.0, respectively (р<0.001). The tumor location, stage, and gender did not affect the PFS. The survival rate was statistically significantly higher among the patients who had got treatment according to the ATRT-2006 protocol compared to MUV-ATRT,EU-RHAB, individual therapeutic regimens, CWS, and HIT-SKK: 0.33, 0.26, 0.11, 0.30, and 0.0, respectively; p=0.0020. The PFS was higher among the patients who had got intraventricular/intrathecal chemotherapy; p=0.0002. HDC with AuHCR did not statistically affect the PFS; p=0.0546.In multivariate analysis, the PFS was influenced by the age, tumor location, extent of surgery, radiotherapy, regional chemotherapy, HDC with AuHCR. Conclusions The survival of patients with CNS AT/RT aged under 3years significantly depended on the patients’ age, extent of surgery, chemotherapy protocol, radiotherapy, and regional administration of chemotherapeutic drugs.

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii276-iii276
Author(s):  
Olga Zheludkova ◽  
Lyudmila Olkhova ◽  
Yuri Kushel’ ◽  
Armen Melikyan ◽  
Marina Ryzhova ◽  
...  

Abstract We analyzed 105 patients under 18 years. The median age was 21 months. There were 54 boys and 51 girls. The supratentorial tumors were in 53 patients, infratentorial in 48, and in spinal cord in 4. 60 had stage M0,29-М+and 16-Mx. All the patients got surgical treatment:total tumor removal in 34,subtotal in 37,partial in 30,and biopsy in 4;75 patients got chemoradiotherapy to ATRT-2006;6-CWS;13-EU-RHAB;5-HIT-SKK;individual schemes in 6. RESULTS: 47 are alive,1 was LFU, and 57 died. PFS was 32%±0.05; the five-year OS 40%±0.05. The median survival-30 months, the median progression-free survival-12 months, and the median of follow-up-23 months. PFS was significantly better in patients more than 12 months compared to patients younger than 12 months:40 and 12%;p=0.00161.After total resection PFS was higher compared to subtotal resection, partial resection, and tumor biopsy:48,38,0,and 0%(p=0.025). After chemoradiotherapy, PFS was higher compared to patients without radiotherapy: 49and 0%(р=0.0000000).PFS for stage M0 was higher compared to stage M+and stage Mx:41,15,and 27%,respectively(р=0.00032).PFS was better for the tumors in the spinal cord and infratentorial location compared to the supratentorial location:67,37,and 25%(p=0.0876).The survival rate was higher among the patients who got treatment according to the ATRT-2006 protocol compared to EU-RHAB, individual regimens, CWS, and HIT-SKK:39,19,17,17,and 0% respectively;p=0.00159.The survival was higher among the patients who got intraventricular/intrathecal Methotrexate,Cytarabine, Prednisolone than among the patients who got only Methotrexate or none at all:40,0,and 5%, respectively; p=0.00015. CONCLUSIONS: Survival was significantly better in patients more than 12month, without metastases, with total removal tumor, chemotheradiotherapy by ATRT-2006 protocol with i/t, i/v Methotrexate/Cytarabine/Prednisolone.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e15588-e15588
Author(s):  
Michele Ghidini ◽  
Andrea Botticelli ◽  
Bianca Maria Donida ◽  
Lidia Strigari ◽  
Concetta Elisa Onesti ◽  
...  

e15588 Background: Chemotherapy (CT) and chemoradiotherapy (CTRT) both in neoadjuvant (neoadj) or adjuvant (adj) setting are associated with better overall survival (OS) over surgery alone in patients (pts) with resectable gastroesophageal (GE) adenocarcinoma (ADK). The best sequence and timing of treatments have still not been defined. A large cohort of GE ADKs derived from 2 high-volume Italian centers was analyzed to describe clinical outcomes and prognostic factors. Methods: 497 patients (pts) diagnosed with GE ADK who underwent surgery with curative intent from 2007 to 2016 were considered. Variables analyzed were: age, sex, tumor location, histology, T, N, M, R, G, HER-2, Helicobacter Pylori (HP) infection, (neo)adj CT, and adj CTRT. Analysis was performed according to ITT principle. Results: Median age at diagnosis was 71 years (range 35-92). At 26.7 months (mo) median follow-up, median OS was 27.6 mo (range 1-127) and median time to recurrence (TTR) 10.8 mo (range 7.8-13.1). Adj CT was administered in 203 cases (41%); 116 pts (23%) had adj CTRT and 47 (9%) neoadj CT. Statistically significant variables for OS and/or TTR at 12 months at univariate analysis were: age, T, N, M, R, G, adj CTRT, neoadj CT and adj CT. Results of multivariate analysis (MVA) are shown in Table 1. Conclusions: Despite a short follow-up, our analysis performed on a very large cohort of consecutive pts confirms the prognostic value of T and N for both OS and TTR. Adj CT and CTRT had a significant impact on 1 year OS, while neoadj CT gave only a 12 months TTR significant benefit. Based on these results, perioperative treatment strategies should always be considered in the management of resectable GE cancer. [Table: see text]


2001 ◽  
Vol 10 (3) ◽  
pp. 1-11 ◽  
Author(s):  
Benedicto O. Colli ◽  
Ossama Al-Mefty

Object Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordoma is a challenge. The authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordoma and 10 patients with chondrosarcoma. Methods Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan–Meier method. Statistical analysis was performed using the Fisher exact and Kruskal–Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordoma) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam radiotherapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively at 4 years posttreatment). Karyotypically abnormal tumors were associated with worst RFS rates as compared with karyotypically normal lesions (44.5% and 90.3%, respectively at 3 years). Cases of cranial nerve palsy, followed by CSF leakages were the most frequent postoperative complication. Permanent postoperative neurological deficit was observed in 28.6% of the patients. Conclusions A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and the patient's age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam radiotherapy provided better prognosis for these patients.


2007 ◽  
Vol 106 (4) ◽  
pp. 575-581 ◽  
Author(s):  
Takuma Nomiya ◽  
Kenji Nemoto ◽  
Toshihiro Kumabe ◽  
Yoshihiro Takai ◽  
Shogo Yamada

Object The purpose of this retrospective study was to estimate the prognostic impact of treatment parameters for 170 patients with anaplastic astrocytoma (AA). Methods Survival outcome and prognostic factors were analyzed for 170 patients with AA. In the multivariate analysis, site of lesion (frontal or parietal lobe, p = 0.002), extent of surgery (total or subtotal resection, p = 0.001), Karnofsky Performance Scale status (0–2, p = 0.021), age (≤ 50 years, p = 0.024), and total dose of radiation therapy (> 60 Gy, p = 0.029) were significant favorable prognostic factors. In the analysis of groups according to extent of surgery, patients who underwent total or subtotal resection had a significantly more favorable prognosis than did patients who underwent partial resection or biopsy (5-year survival rate 54.0% for total or subtotal resection compared with 17.5% for partial resection or biopsy; median survival time [MST] 62.6 months compared with 22.9 months [p < 0.0001, log-rank test]; hazard ratio [HR] 0.67; and 95% confidence interval [CI] 0.52–0.85 [p = 0.001]). In the analysis of groups according to total radiation dose, the group of patients who received doses greater than 60 Gy had a significantly more favorable prognosis than did the group who received 60 Gy or less (5-year survival rate 45.0% for patients who received doses greater than 60 Gy compared with 21.1% for those receiving 60 Gy or less; MST 48.9 months compared with 21.6 months [p = 0.0006, log-rank test]; HR 0.96; 95% CI 0.93–0.99 [p = 0.029]). Conclusions The most important parameter in the treatment of AA was extent of surgery, and total radiation dose was the second most important factor. Resection of as much of the tumor as possible and delivery of a total radiation dose of greater than 60 Gy seem to be required for local control of AA.


2020 ◽  
Author(s):  
Brooke E Patteson ◽  
Sujith Baliga ◽  
Benjamin V M Bajaj ◽  
Shannon M MacDonald ◽  
Beow Y Yeap ◽  
...  

Abstract Background Treatment for pediatric ependymoma includes surgical resection followed by local radiotherapy (RT). Proton RT (PRT) enables superior sparing of critical structures compared with photons, with potential to reduce late effects. We report mature outcomes, patterns of failure, and predictors of outcomes in patients treated with PRT. Methods One hundred fifty patients (&lt;22 y) with World Health Organization grades II/III ependymoma were treated with PRT between January 2001 and January 2019 at Massachusetts General Hospital. Demographic, tumor, and treatment-related characteristics were analyzed. Event-free survival (EFS), overall survival (OS), and local control (LC) were assessed. Results Median follow-up was 6.5 years. EFS, OS, and LC for the intracranial cohort (n = 145) at 7 years were 63.4%, 82.6%, and 76.1%. Fifty-one patients recurred: 26 (51.0%) local failures, 19 (37.3%) distant failures, and 6 (11.8%) synchronous failures. One hundred sixteen patients (77.3%) underwent gross total resection (GTR), 5 (3.3%) underwent near total resection (NTR), and 29 (19.3%) underwent subtotal resection (STR). EFS for the intracranial cohort at 7 years for GTR/NTR and STR was 70.3% and 35.2%. With multivariate analysis, the effect of tumor excision persisted after controlling for tumor location. There was no adverse effect on disease control if surgery to RT interval was within 9 weeks of GTR/NTR. Conclusion PRT is effective and safe in pediatric ependymoma. Similar to previous studies, GTR/NTR was the most important prognostic factor. Intervals up to 9 weeks from surgery to PRT did not compromise disease outcomes. There was no LC benefit between patients treated with &gt;54 Gray relative biological effectiveness (GyRBE) versus ≤54 GyRBE.


2020 ◽  
Author(s):  
Felix Behling ◽  
Christina Fodi ◽  
Irina Gepfner-Tuma ◽  
Kristina Kaltenbach ◽  
Mirjam Renovanz ◽  
...  

Abstract Background A loss of the trimethylation of lysine 27 of histone H3 (H3K27me3) in meningioma has been recently suggested as an adjunct to identify subsets of higher risk of recurrence. The aim of the present study was to assess the prognostic value of H3K27 histone trimethylation and its potential clinical utility in the “Tübingen meningioma cohort”. Methods Patients who underwent meningioma resection between 10/2003 and 1/22015 at the University Hospital Tübingen were included. Immunohistochemical stainings for H3K27me3 and the proliferation marker MIB1 were assessed and correlated with clinical parameters using univariate and multivariate cox regressions as well as Pearson’s chi-squared and log-rank test. Results Overall, 1268 meningiomas were analyzed with a female to male ratio of 2.6 and a mean age of 58.7 years (range 8.3 – 91.0). With 163 cases lost to follow up, 1103 cases were available for further analysis with a mean follow-up of 40.3 months (range 1.1 – 186.3). Male gender, younger age, intracranial tumor localization, progressive tumor, subtotal resection, higher WHO grade, increased MIB1 rate and loss of H3K27me3 were significant negative prognostic factors in the univariate analysis. H3K27me3 status and all other prognostic factors, except age and tumor location, remained significant in the multivariate model. Furthermore, adjuvant radiotherapy was an independent positive prognostic factor. Conclusions Loss of H3K27me3 combined with MIB1 labeling index are independent prognostic factors in meningioma. These data from the Tübingen meningioma cohort support the clinical utility of H3K27me3 immunohistochemical staining in meningioma and its integration into the routine histopathological workup.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 2083-2083
Author(s):  
Hannah Yoon ◽  
Irene B. Helenowski ◽  
Karthikeyan Perumal ◽  
MaryAnne H. Marymont ◽  
James Chandler ◽  
...  

2083 Background: We evaluated outcome and prognostic factors for high grade meningioma (G2-3) and the role of early post-operative radiotherapy (RT). Methods: From 2000 to 2010, 136 patients were diagnosed with G2-3 meningioma at Northwestern: 124 with atypical (G2) and 12 with anaplastic (G3) meningioma. All were treated with or without RT after initial or subsequent resection. The primary endpoint was progression-free survival (PFS). Results: 21 patients received adjuvant RT, and 115 did not. Median PFS for G2 with and without RT was 68 vs.89 mos. Median PFS for G3 with and without RT was not reached=nr (mean 5) vs.60 mos (mean 35). Median PFS for Simpson G1-3 with and without RT was 18 vs.96 mos. Median PFS for Simpson G4 with and without RT was nr (mean 55) vs.59 mos (mean 44). For median follow-up of 33 mos for Simpson G1-3 and 29 mos for G4, recurrence rate for Simpson G1-3 with and without RT was 40 vs.6%, and for Simpson G4 with and without RT, 9 vs.31%. 3-yr OS for G2 with and without RT was 93 vs.94%. 3-yr OS for G3 with and without RT was 80 vs.80%. In multivariate analysis, G3 histology and Simpson grade were predictive for relapse, while brain invasion, mitoses, adjuvant RT, and location of tumor were not. For those who received adjuvant RT, mean dose of external beam radiation (EBRT) in 2 patients was 58 Gy, while mean dose of stereotactic radiosurgery (SRS) in 15 patients was 15 Gy. 51% EBRT patients recurred, while 22% SRS patients recurred (p = 0.45). Median PFS with EBRT was 43 vs.51 mos for SRS (p = 0.34). Patients were maldistributed between the with and without RT arms in terms of brain invasion, extent of resection, and G3 histology. Conclusions: Patients who received RT had lower PFS compared to those who did not; survival was comparable. This may be due to inherent selection bias for patients with more aggressive disease getting adjuvant RT. Our study may be underpowered to determine the true role of RT in G2-3 meningioma. Patients with subtotal resection, Simpson G4, who received RT had fewer recurrences and higher PFS compared to those who did not. 3-yr OS was equivalent in both G2 and G3 tumors with and without RT. G3 histology and Simpson grade were predictive for relapse while brain invasion, mitoses, adjuvant RT, and location of tumor were not.


2016 ◽  
Vol 34 (2_suppl) ◽  
pp. 372-372
Author(s):  
Hamid Emamekhoo ◽  
Dharmesh Gopalakrishnan ◽  
Puneet Dhillon ◽  
Haider Al taii ◽  
Paul Elson ◽  
...  

372 Background: UTUC is relatively rare (5-10% of UC). Limited data on prognostic factors is available. Methods: A retrospective study of UTUC patients (pts) who had surgery (1995-2014) at Cleveland Clinic (n = 454) was conducted. Univariable (UVA) and multivariable (MVA) analysis (proportional hazards) with a stepwise selection algorithm (p = .10 and .05, as criteria for entry and retention in the model) was used to identify independent predictors of recurrence-free survival (RFS) and overall survival (OS). Results: 192 pts with invasive high grade UTUC were identified; median age at resection was 72; 69% men. 72% of pts had laparoscopic and 17% open nephrouretectomy, 23% had +ve margins (including bladder/ureter cuff), 22% had multifocal tumor. Median tumor size 3.5 cm (0.2-12); 70% had tumors < 5 cm; 65% pT3, 8% pT4 stage; among pts with lymph node (LN) dissection, 25% had +ve LN. All but 3 pts (2 sarcomatoid, 1 small cell) had primarily UC; 28% mixed UC histology; 40% CIS, 54% confirmed lymphovascular invasion (LVI); 10 neoadjuvant, 14 confirmed adjuvant therapy). Among 116 pts with RFS data available 59% had disease recurrence; 8% died with no known recurrence. Estimated median RFS was 13.1 months (m) (95%CI 8.6-18.6). In UVA, age, positive margins, tumor size, smoking history, pT stage, LVI, primary site impacted RFS. In MVA, margin status (p = .04), age (p = .04), pT stage (p = .05) were independently associated with RFS. Among 175 pts with OS data available, 38% died with estimated median OS 44.6 m (95%CI 24.1-107.4); median follow-up was 13.8 m (0.1-185.7) in pts alive at last follow up. In UVA, LVI, tumor size, age, pT stage, tumor location, ECOG PS impacted OS. In MVA, tumor size (p = .002), CIS (p = .004), tumor location (p = .04), LVI (p = .04) independently predicted OS. Using these factors, 3 prognostic groups were identified for each outcome (see table). Conclusions: Clinic-pathological parameters can be prognostic in UTUC; further validation is needed. [Table: see text]


2019 ◽  
Vol 30 (6) ◽  
pp. 794-800 ◽  
Author(s):  
Sung Mo Ryu ◽  
Sun-Ho Lee ◽  
Kyung Min Lee ◽  
Whan Eoh ◽  
Eun-Sang Kim

OBJECTIVEThe objective of this study was to elucidate the features and surgical outcomes of cervical schwannomas.METHODSThe authors retrospectively reviewed the records of 90 patients who underwent surgically treated cervical schwannomas from January 1995 to December 2017, with an emphasis on MRI findings such as tumor location, tumor size, extent of tumor resection, and growth of a residual tumor.RESULTSThis study included 51 men (56.7%) and 39 women (43.3%) with a mean age of 44.5 years (range 7–77 years). Dumbbell-shaped tumors comprised 62 (68.9%) of 90 cases and gross-total resection (GTR) was achieved in 59 (65.6%) of 90 cases. All nondumbbell tumors (n = 28) underwent GTR. Only 1 case of recurrence in the GTR group showed a gradual increase in size (by 8.9 mm) during the 150-month follow-up period. For the regrowth patients in the subtotal resection group, the mean percentage increase in tumor size was 47.5% ± 33.1% and the mean growth rate was 5.8 ± 4.6 mm/year during the 20.3-month follow-up period. However, the size of residual tumor spontaneously decreased by a mean of 8.3% ± 11.1% during the 48.4-month follow-up period in the nonregrowth group.CONCLUSIONSThese findings suggested that frequent MRI follow-up examinations are required for residual schwannomas in the cervical spine for at least 2 years, and continuous MRI follow-ups are also required thereafter.


Neurosurgery ◽  
2019 ◽  
Vol 86 (2) ◽  
pp. 288-297 ◽  
Author(s):  
Mohamed A Zaazoue ◽  
Peter E Manley ◽  
Mohammed Al Mehdar ◽  
Nicole J Ullrich ◽  
Hormuzdiyar H Dasenbrock ◽  
...  

Abstract BACKGROUND Pediatric low-grade gliomas are among the most common childhood neoplasms, yet their post-treatment surveillance remains nonstandardized, relying on arbitrarily chosen imaging intervals. OBJECTIVE To optimize postoperative magnetic resonance imaging (MRI) surveillance protocols for pediatric low-grade gliomas. METHODS Patients aged 0 to 21 yr with pediatric low-grade gliomas, treated between 1990 and 2016 were retrospectively analyzed. The timing of surveillance imaging and radiologic tumor outcomes were extracted, and the effect of patient age, tumor location, histology, and extent of resection as prognostic factors was studied. An algorithm was developed to analyze the detection efficacy and cost of all possible surveillance protocols. RESULTS A total of 517 patients were included with a median follow-up of 7.7 yr (range: 2-25.1 yr) who underwent 8061 MRI scans (mean 15.6 scans per patient). Tumor recurrence was detected radiologically in 292 patients (56.5%), of whom, 143 underwent reoperation. The hazards ratio (HR) of recurrence was higher in patients who underwent biopsy (HR = 3.60; 95% confidence interval (CI): 2.45-5.30; P &lt; .001), subtotal resection (HR = 2.97; 95% CI: 2.18-4.03; P &lt; .001), and near-total resection (HR = 2.03; 95% CI: 1.16-3.54; P = .01), compared to patients with gross total resection (GTR). For all patients, an 8-image surveillance protocol at 0, 3, 6, 12, 24, 36, 60, and 72 mo (total cost: $13 672 per patient) yielded comparative detection rates to the current 15-image protocol ($25 635). For patients who underwent GTR, a 6-image protocol at 0, 3, 9, 24, 36, and 60 mo ($10 254) is sufficient. CONCLUSION Our data suggest that postoperative surveillance of pediatric low-grade gliomas can be effectively performed using less frequent imaging compared to current practice, thereby improving adherence to follow-up, and quality-of-life, while reducing costs.


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