Case-based review: Atypical teratoid/rhabdoid tumor

Cody L. Nesvick, Amulya A. Nageswara Rao, Aditya Raghunathan, Jaclyn A. Biegel, David Daniels

Research output: Contribution to journalReview article

Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare CNS cancer that typically occurs in children younger than 3 years of age. Histologically, AT/RTs are embryonal tumors that contain a rhabdoid component as well as areas with primitive neuroectodermal, mesenchymal, and epithelial features. Compared to other CNS tumors of childhood, AT/RTs are characterized by their rapid growth, short symptomatic prodrome, and large size upon presentation, often leading to brain compression and intracranial hypertension requiring urgent intervention. For decades, the mainstay of care has been a combination of maximal safe surgical resection followed by adjuvant chemotherapy and radiotherapy. Despite advances in each of these modalities, the relative paucity of data on these tumors, their inherently aggressive course, and a lack of molecular data have limited advances in treatment over the past 3 decades. Recent large-scale, multicenter interdisciplinary studies, however, have significantly advanced our understanding of the molecular pathogenesis of these tumors. Multiple clinical trials testing molecularly targeted therapies are underway, offering hope for patients with AT/RT and their families.

Original languageEnglish (US)
Pages (from-to)163-178
Number of pages16
JournalNeuro-Oncology Practice
Volume6
Issue number3
DOIs
StatePublished - Jun 1 2019

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Neoplasms
Hope
Interdisciplinary Studies
Adjuvant Radiotherapy
Intracranial Hypertension
Adjuvant Chemotherapy
Multicenter Studies
Atypical Teratoid Tumor
Typical Teratoid Rhabdoid Tumor
Clinical Trials
Brain
Therapeutics
Growth

Keywords

  • ATRT
  • Epigenetics
  • Infant
  • Pediatric
  • Rhabdoid

ASJC Scopus subject areas

  • Medicine (miscellaneous)

Cite this

Case-based review : Atypical teratoid/rhabdoid tumor. / Nesvick, Cody L.; Nageswara Rao, Amulya A.; Raghunathan, Aditya; Biegel, Jaclyn A.; Daniels, David.

In: Neuro-Oncology Practice, Vol. 6, No. 3, 01.06.2019, p. 163-178.

Research output: Contribution to journalReview article

Nesvick, Cody L. ; Nageswara Rao, Amulya A. ; Raghunathan, Aditya ; Biegel, Jaclyn A. ; Daniels, David. / Case-based review : Atypical teratoid/rhabdoid tumor. In: Neuro-Oncology Practice. 2019 ; Vol. 6, No. 3. pp. 163-178.
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