scholarly journals Advancing biology-based therapeutic approaches for atypical teratoid rhabdoid tumors

2020 ◽  
Vol 22 (7) ◽  
pp. 944-954 ◽  
Author(s):  
Lindsey M Hoffman ◽  
Elizabeth Anne Richardson ◽  
Ben Ho ◽  
Ashley Margol ◽  
Alyssa Reddy ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Molecular Heterogeneity ◽  
Whole Genome ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Therapeutic Approaches ◽  
Central Nervous System Cancer ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors ◽  
Nervous System Cancer

Abstract Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant central nervous system cancer arising in infants and younger children, historically considered to be homogeneous, monogenic, and incurable. Recent use of intensified therapies has modestly improved survival for ATRT; however, a majority of patients will still succumb to their disease. While ATRTs almost universally exhibit loss of SMARCB1 (BAF47/INI1/SNF5), recent whole genome, transcriptome, and epigenomic analyses of large cohorts reveal previously underappreciated molecular heterogeneity. These discoveries provide novel insights into how SMARCB1 loss drives oncogenesis and confer specific therapeutic vulnerabilities, raising exciting prospects for molecularly stratified treatment for patients with ATRT.

scholarly journals Atypical teratoid/rhabdoid tumor: A rare case report

2017 ◽  
Vol 5 (3) ◽  
pp. 70-72
Author(s):  
R Shrestha ◽  
S Gauchan ◽  
A K Jha
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Case ◽  
Primitive Neuroectodermal Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Embryonal Tumor ◽  
Rare Case Report ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Atypical Teratoid/Rhabdoid Tumors (AT/RTs) is rare, highly malignant embryonal tumor of central nervous system that predominantly occurs in infancy and young children. The majority of tumors arise (approximately two-third) in the posterior fossa. The diagnosis of AT/RTs relies predominantly on morphologic and immunohistochemical criteria. The most common differential diagnosis are Primitive Neuroectodermal Tumor and malignant glioma. Herein we present a case of 5 year old child who initially presented with headache and vomiting.

scholarly journals AT-18INVESTIGATING THE BIOLOGY OF ATYPICAL TERATOID/RHABDOID TUMORS BY WHOLE GENOME CRISPR/CAS9 SCREENING

2016 ◽  
Vol 18 (suppl 3) ◽  
pp. iii5.2-iii5
Author(s):  
Matthew P. Selby ◽  
Martina A. Finetti ◽  
Matthew Bashton ◽  
Ruth E. Cranston ◽  
Alicia Del-Carpio-Pons ◽  
...  
Keyword(s):  
Whole Genome ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals Atypical teratoid rhabdoid tumor in a 65-year-old man presenting with disseminated leptomeningeal disease: A case report and review of the literature

2018 ◽  
Vol 6 ◽  
pp. 2050313X1877529
Author(s):  
Marc-Alain Babi ◽  
Peter Fecci ◽  
Matthew Luedke ◽  
Olinda Pineda ◽  
Yasmin Ali O’Keefe
Keyword(s):  
Brain Biopsy ◽  
Rhabdoid Tumor ◽  
Leptomeningeal Disease ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Review Of The Literature ◽  
Progressive Encephalopathy ◽  
Atypical Teratoid ◽  
Aggressive Tumor ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Central nervous system atypical teratoid rhabdoid tumors are very rare aggressive tumor of childhood, primarily occurring at age of less than 3 years old. The prognosis of these tumors is very poor, with a reported median survival of 6–12 months in most cases. Treatment typically consists of aggressive chemotherapy and radiotherapy. We present the case of a 65-year-old man who presented with progressive encephalopathy and change in personality over 3 months period. The patient had further accelerated decline over 3 weeks. The diagnosis of atypical teratoid rhabdoid tumor initially remained elusive despite very extensive workup, but was eventually confirmed via open brain biopsy. To the best of our knowledge, this is the oldest reported case of atypical teratoid rhabdoid tumor in the literature. We further extend the spectrum of this rare disease.

scholarly journals Atypical teratoid/rhabdoid tumors of the central nervous system in children: the state of the problem today. Literature review

2019 ◽  
Vol 5 (4) ◽  
pp. 60-73 ◽  
Author(s):  
Yu. V. Dinikina ◽  
M. B. Belogurova
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Conflict Of Interest ◽  
Malignant Tumors ◽  
Young Patients ◽  
Genetic Profile ◽  
Atypical Teratoid ◽  
The Central Nervous System ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Atypical teratoid/rhabdoid tumors (AT/RT) are a group of rare highly aggressive malignant tumors in young patients. Among all the malignant tumors of the central nervous system (CNS) in children, they are 1–2 %, which, due to the small number of groups, makes it difficult to develop uniform recommendations for antitumor therapy. The molecular genetic profile of AT/RT, which largely determines the characteristics of the disease, has been studied sufficiently. Despite the large number of ongoing clinical studies, the results of treatment of AT/RT CNS in the world today remain unsatisfactory. The early age of patients limits the use of radiation therapy, which leads to the need to intensify chemotherapy regimens and to choose the optimal strategy in the toxicity – benefit ratio. The article describes modern approaches to the treatment of central nervous system disorders in children, presents the results of studies with the largest number of included patients, using the multimodal treatment strategy, identifies current trends in targeted therapy.Conflict of interest. The authors declare no conflict of interest.Funding. The study was performed without external funding.

CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system

2008 ◽  
Vol 50 (5) ◽  
pp. 447-452 ◽  
Author(s):  
Monika Warmuth-Metz ◽  
Brigitte Bison ◽  
Elke Dannemann-Stern ◽  
Rolf Kortmann ◽  
Stefan Rutkowski ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Mr Imaging ◽  
Atypical Teratoid ◽  
The Central Nervous System ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors
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