Rhabdoid tumor of the central nervous system

Weinblatt, Mark E.; Kochen, Joseph

doi:10.1002/mpo.2950200317

Cited by 38 publications

(18 citation statements)

References 1 publication

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…We 9 and other investigators 7,8 have previously shown that multiagent chemotherapy containing an anthracycline can be efficacious, and our current regimen and another recent report 19 support the rationale for anthracycline-based regimens.…”

Section: Discussionsupporting

confidence: 66%

Intensive Multimodality Treatment for Children With Newly Diagnosed CNS Atypical Teratoid Rhabdoid Tumor

Susan

Zimmerman

Yao

et al. 2009

JCO

402

281

View full text Add to dashboard Cite

A B S T R A C T PurposeAtypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with a historic median survival ranging from 6 to 11 months. Based on a previous pilot series, a prospective multi-institutional trial was conducted for patients with newly diagnosed CNS ATRT. Patients and MethodsTreatment was divided into five phases: preirradiation, chemoradiation, consolidation, maintenance, and continuation therapy. Intrathecal chemotherapy was administered, alternating intralumbar and intraventricular routes. Radiation therapy (RT) was prescribed, either focal (54 Gy) or craniospinal (36 Gy, plus primary boost), depending on age and extent of disease at diagnosis. ResultsBetween 2004 and 2006, 25 patients were enrolled; 20 were eligible for evaluation. Median age at diagnosis was 26 months (range, 2.4 months to 19.5 years). Gross total resection of the primary tumor was achieved in 11 patients. Fourteen patients had M0 disease at diagnosis, one patient had M2 disease, and five patients had M3 disease. Fifteen patients received radiation therapy: 11 focal and four craniospinal. Significant toxicities, in addition to the expected, included radiation recall (n ϭ 2) and transverse myelitis (n ϭ 1). There was one toxic death. Of the 12 patients who were assessable for chemotherapeutic response (pre-RT), the objective response rate was 58%. The objective response rate observed after RT was 38%. The 2-year progression-free and overall survival rates are 53% Ϯ 13% and 70% Ϯ 10%, respectively. Median overall survival has not yet been reached. ConclusionThis intensive multimodality regimen has resulted in a significant improvement in time to progression and overall survival for patients with this previously poor-prognosis tumor.

show abstract

Section: Discussionsupporting

confidence: 66%

Intensive Multimodality Treatment for Children With Newly Diagnosed CNS Atypical Teratoid Rhabdoid Tumor

Susan

Zimmerman

Yao

et al. 2009

JCO

402

281

View full text Add to dashboard Cite

show abstract

“…Nevertheless, multiagent chemotherapy containing anthracyclines and alkylating agents in an approach similar to that used to treat rhabdomyosarcoma has been efficacious in the treatment of ATRT. 12,[20][21][22][23] Intrathecal chemotherapy is associated with prolonged survival, and its role in treatment continues to be investigated. 11,14 There is also evolving evidence that dose-intensive chemotherapy and autologous hematopoietic stem cell rescue, particularly with the use of high-dose methotrexate, may be of benefit in young children with ATRT.…”

Section: Discussionmentioning

confidence: 99%

Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis

Buscariollo

Park

Roberts

et al. 2011

Cancer

157

170

View full text Add to dashboard Cite

BACKGROUND: Atypical teratoid rhabdoid tumor (ATRT) is a rare central nervous system malignancy with a poor prognosis that affects mostly young children. Although radiotherapy (RT) historically has been delayed in patients aged <3 years, emerging evidence suggests a role for RT to achieve long-term survivorship. Clinical features and age-dependent trends of RT use were evaluated for patients with ATRT. METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results database was used to identify 144 patients with ATRT from 1973 to 2008. The primary endpoint was median overall survival (OS). Clinical and treatment variables were assessed for an association with OS using Cox proportional hazards models. Landmark analysis was used to correct for immortal time bias of adjuvant RT. RESULTS: The median age at diagnosis was 1 year (range, 0-67 years). Gross total resection of the primary tumor was achieved in 39% of patients, and 33% of patients received RT. From 1992 to 2008, RT use increased 2.4-fold in patients aged 3 years. The median OS for was 10 months. In multivariate analyses, metastatic disease (hazard ratio, 2.83; 95% confidence interval, 1.53-5.23; P ¼ .001) and RT (hazard ratio, 0.10; 95% confidence interval, 0.01-0.73; P ¼ .02) were identified as independent predictors of survival. Landmark analysis confirmed a robust association between RT use and survival, which was attenuated in patients ages 4 to 17 years compared with younger patients. CONCLUSIONS: The current results indicated that RT may offer a significant survival benefit for patients with ATRT and that patients aged 3 years may derive more benefit from initial RT compared with older children. The authors concluded that prospective clinical trials are needed to examine the role of RT in the initial management of ATRT in patients aged <3 years. Cancer 2012;118:4212-9. V C 2011 American Cancer Society.KEYWORDS: atypical teratoid rhabdoid tumor, central nervous system, epidemiology, radiotherapy, children. INTRODUCTIONAtypical teratoid rhabdoid tumor (ATRT) of the central nervous system (CNS) is a highly malignant embryonal neoplasm (grade 4 according to the World Health Organization classification) that typically occurs in children aged <3 years. 1-5 ATRT is extremely rare in adults, and only 31 patients have been reported in the literature. 6 Originally described in the 1980s, ATRT has been widely recognized as a rare but important clinical entity only over the past decade. 2,4 The diagnosis of ATRT has been greatly facilitated by the rise in accessibility of immunohistochemical testing in the late 1990s, which takes advantage of the frequent loss of switch/sucrose nonfermentable (SWI/SNF)-related, matrix-associated, actin-dependent regulator of chromatin, subfamily b, member 1 (SMARCB1) (also known as SNF5 homolog [hSNF5] and INI1) tumor suppressor gene products in these tumors. 2,5,7 Although ATRT comprises <5% of CNS tumors in children aged <18 years, it reportedly represents up to 20% of CNS tumors in children aged <3 years...

show abstract

“…Intergrup Rabdomyosarcom Çalışması (IRS) III esas alınarak multiajan kemoterapi uygulanan bir çalışmada, GTR sonrası 4140 cGy fokal RT, yoğun kemoterapi ve üçlü intratekal kemoterapi almış ve 16 yıllık hastalık-sız sağkalım rapor edilmiştir. [39] Olson ve ark. üç olguda yine cerrahi sonrası kraniospinal ve fokal radyoterapi ile uzun remisyon süresi bildirmiş-lerdir.…”

Section: Discussionunclassified

Atipical teratoid rhabdoid tumor: case report and review of the literature

Şahintürk¹

2014

TJ Oncol

View full text Add to dashboard Cite

Atipical teratoid rhabdoid tumor (ATRT) is a rare and highly agressive malign tumor in the early childhood. Mean survival has been reported as 6-11 months. Despite the optimal treatment is unclear surgery, chemotherapy and radiotherapy are the well known treatment options. We would like to report a 4 year old boy who had the diagnosis of ATRT at the temporooccipital region to make a contribution to the literature. Atipik teratoid rabdoid tümör (ATRT) erken çocukluk çağının nadir görülen ve oldukça agresif seyirli bir malign tümörüdür. Ortalama sağkalım süresi 6-11 ay olarak bildirilmektedir. Optimal tedavisi tartışmalı olmakla beraber cerrahi, radyoterapi ve kemoterapi seçenekler arasındadır. Yazımızda temporooksipital bölge yerleşimli ATRT tanılı dört yaşındaki erkek olguyu literatüre katkı sağlaması amacı ile sunuyoruz.

show abstract

Rhabdoid tumor of the central nervous system

Cited by 38 publications

References 1 publication

Intensive Multimodality Treatment for Children With Newly Diagnosed CNS Atypical Teratoid Rhabdoid Tumor

Intensive Multimodality Treatment for Children With Newly Diagnosed CNS Atypical Teratoid Rhabdoid Tumor

Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis

Atipical teratoid rhabdoid tumor: case report and review of the literature

Contact Info

Product

Resources

About