Prognosis of atypical teratoid rhabdoid tumors (AT/RT) treated with multimodal therapy protocols. Report of our series

Neurocirugía ◽  
2016 ◽  
Vol 27 (2) ◽  
pp. 87-94 ◽  
Author(s):  
Alfonso Valencia-Moya ◽  
Laura González-García ◽  
Bienvenido Ros-López ◽  
Tomás Acha-García ◽  
Bernardo Weil-Lara ◽  
...  
Keyword(s):  
Multimodal Therapy ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Fahd Refai ◽  
Haneen Al-Maghrabi ◽  
Hassan Al Trabolsi ◽  
Jaudah Al-Maghrabi
Keyword(s):  
Multimodal Therapy ◽  
Tumor Suppressor Protein ◽  
Central Nervous System Tumors ◽  
Histopathological Diagnosis ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Thoracic Spinal ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for the characteristic loss of the tumor suppressor protein SMARCB1/INI1. Here, we describe a case of thoracic spinal ATRT in a three-year-old boy that showed characteristic aggressive progression until managed with intensive multimodal therapy to achieve durable long-term remission. In doing so, we review the histopathological features, management, and current advances in molecular biology that hold promise for personalized ATRT therapy.

Clinical characteristics, treatment, and survival outcome in pediatric patients with atypical teratoid/rhabdoid tumors: a retrospective study by the Japan Children’s Cancer Group

2020 ◽  
Vol 25 (2) ◽  
pp. 111-120 ◽  
Author(s):  
Kai Yamasaki ◽  
Chikako Kiyotani ◽  
Keita Terashima ◽  
Yuko Watanabe ◽  
Masayuki Kanamori ◽  
...  
Keyword(s):  
Multimodal Therapy ◽  
Pediatric Patients ◽  
Positive Impact ◽  
Conditioning Regimen ◽  
High Dose ◽  
Primary Therapy ◽  
Female Ratio ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

OBJECTIVEThe prognosis of atypical teratoid/rhabdoid tumors (ATRTs) has improved in recent years with the use of multimodal therapy, mainly in cases not involving metastatic disease. The authors wanted to obtain historical control data and evaluate the suitable treatments in Japanese children with ATRTs that were proven negative for INI-1 immunostaining.METHODSThe authors retrospectively collected clinical information on 38 pediatric patients with ATRTs treated from 2005 to 2016 and analyzed the data for this series.RESULTSThe median age of the patient population was 1.3 years, and the male/female ratio was approximately 2:1. Twenty-three patients (60.5%) had metastases. The effects of treatment on prognosis were analyzed for 34 patients after exclusion of 4 patients who could not receive curative treatment. At a median follow-up of 40.9 months, the mean (± SD) progression-free survival (PFS) and overall survival (OS) were 66.6% ± 8.3% and 45.9% ± 8.7% at 2 years and 44.2% ± 9.9% and 34.2% ± 8.9% at 5 years, respectively. The metastasis stage at diagnosis (M0–1 vs M2–4) (HR 2.68, 95% CI 1.08–6.65; p = 0.0338) and gross tumor resection (yes vs no) (HR 3.49, 95% CI 1.01–12.1; p = 0.0481) were prognostic factors for PFS but not for OS. Postoperative chemotherapy was performed in all 34 cases. High-dose chemotherapy was performed in 19 (55.8%) of 34 patients and showed a positive impact on OS (HR 0.31, 95% CI 0.11–0.86; p = 0.0254); the most commonly used regimen was a double-conditioning regimen of thiotepa plus melphalan. Local radiotherapy had a positive impact on both PFS and OS; however, craniospinal irradiation (CSI) performed in 12 patients as the primary therapy was associated with a poor outcome. Disseminated recurrence within 12 months from diagnosis was the most common pattern of treatment failure regardless of CSI.CONCLUSIONSThere has been an improvement in outcomes for pediatric ATRT patients since the introduction of multimodal therapy in Japan, mainly in patients without metastases. Even if selection bias is taken into consideration, CSI did not contribute to an improved prognosis. Novel treatment approaches are required for pediatric ATRT patients with metastases.

MODERN METHODS OF DIAGNOSIS AND TREATMENT OF ATYPICAL TERATOID RHABDOID TUMORS CNS IN CHILDREN

2021 ◽  
Vol 100 (3) ◽  
pp. 120-129
Author(s):  
L.V. Olkhova ◽  
◽  
O.G. Zheludkova ◽  
Keyword(s):  
Multimodal Therapy ◽  
Chemoradiation Therapy ◽  
Malignant Neoplasms ◽  
Biallelic Mutation ◽  
Atypical Teratoid ◽  
Grade Of Malignancy ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors ◽  
Brain And Spinal Cord ◽  
The Brain

Atypical teratoid rhabdoid tumors (ATRT) of the CNS in children are a group of aggressive malignant neoplasms with extremely poor prognosis, and therefore these tumors are classified as IV (highest) grade of malignancy. At the time of diagnosis, 20% of patients are found to have metastasized to the brain and spinal cord. The pathogenesis of ATRT is based on the biallelic mutation of the SMARCB1 gene, more rarely SMARCA4 genes. Despite the use of intensive multimodal therapy, the survival rate of patients with ATRT remains low. Recently, several groups of scientists, based on epigenetic and transcriptional studies, have identified 3 molecular groups of ATRT with different therapeutic sensitivity to chemoradiation therapy and, accordingly, have a different prognosis of the disease. This article presents the current literature data on epidemiology, pathogenesis, clinic, diagnosis and therapy of patients with ATRT, and describes the directions of treatment.

scholarly journals ATRT-05. RESULTS OF MULTICENTER TRIAL CONCERNING THE TREATMENT OF CHILDREN WITH ATYPICAL TERATOID/RHABDOID TUMORS (ATRT) OF THE CENTRAL NERVOUS SYSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii276-iii276
Author(s):  
Olga Zheludkova ◽  
Lyudmila Olkhova ◽  
Yuri Kushel’ ◽  
Armen Melikyan ◽  
Marina Ryzhova ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Progression Free Survival ◽  
Multicenter Trial ◽  
Intrathecal Methotrexate ◽  
Subtotal Resection ◽  
Tumor Biopsy ◽  
Atypical Teratoid ◽  
The Central Nervous System ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

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.

scholarly journals Histomorphology Evolution of Recurrent Atypical Teratoid/Rhabdoid Tumors

2015 ◽  
Vol 144 (suppl 2) ◽  
pp. A023-A023
Author(s):  
Esther Yoon ◽  
Taliya Farooq ◽  
Lawrence Sann ◽  
Eric Vail ◽  
George Kleinman
Keyword(s):  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals ATRT-26. META-ANALYSIS OF TREATMENT MODALITIES IN METASTATIC ATYPICAL TERATOID/RHABDOID TUMORS IN CHILDREN

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii281-iii281
Author(s):  
Reena M Underiner ◽  
Mostafa Eltobgy ◽  
Joseph R Stanek ◽  
Jonathan L Finlay ◽  
Mohamed S AbdelBaki
Keyword(s):  
Surgical Resection ◽  
Meta Analysis ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
Substantial Effect ◽  
Treatment Modalities ◽  
Risk Of Death ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract BACKGROUND Metastatic atypical teratoid/rhabdoid tumors (AT/RT) are aggressive central nervous system tumors that present during infancy and are associated with dismal outcomes. Patients receive multimodal treatment including surgical resection, systemic chemotherapy and one or more of intrathecal chemotherapy (IT), marrow-ablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and radiation therapy (XRT). While data regarding treatment modalities for AT/RT patients exist, no comprehensive data have been published regarding the metastatic patient population. METHODS We performed a meta-analysis of 1,578 articles published through September 2018, including 44 studies with a total of 123 subjects. Additionally, seven patients were incorporated through chart review of patients treated at Nationwide Children’s Hospital. RESULTS Analysis of 130 patients revealed a 3-year overall survival (OS) of 25%. Age at diagnosis had a significant impact on survival (p=0.0355); 3-year OS for infants < 18 months was 21%; 18–36 months was 26%; and > 36 months was 36%. Location of the primary tumor, metastatic stage and extent of surgical resection did not have significant impact on OS. On univariate analysis, XRT (p<0.0001), IT (p=0.01) and AuHCR (p<0.0001) were found to significantly improve survival. The most substantial effect was noted in patients who received AuHCR (3-year OS of 60% versus 9% in those who did not). On multivariable analysis XRT (p=0.0006), IT (p=0.0124) and AuHCR (p<0.0001) were independently associated with reduced risk of death.

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