Atypical teratoid rhabdoid tumor: long-term survival after chemoradiotherapy

Abstract

Purpose

Atypical teratoid rhabdoid tumors (ATRTs) arise from the central nervous system largely in the pediatric population. They portend a very poor prognosis with few long-term survivors. We describe a series of five cases at our institution.

Methods

We conducted a retrospective chart review and clinical follow-up.

Results

Three patients underwent chemoradiation after surgical resection; the two patients whose caretakers declined this therapy passed away soon after diagnosis. Chemoradiation included intravenous and intrathecal chemotherapy as well as intensity-modulated radiotherapy after resection. Of the patients receiving chemoradiation, two patients had infratentorial tumors, two had gross residual tumor after resection, and two were under the age of 3 years. The three patients receiving trimodality therapy remain clinically and symptomatically disease-free with follow-up times of 44, 46, and 55 months. Two of the patients have mild neuropsychiatric sequelae after therapy.

Conclusions

Long-term, high-volume trials of ATRT are currently not published. We offer experience in successful long-term survival of this tumor treated with chemoradiotherapy.

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Ethical approval

For this type of study, formal consent is not required. This article does not contain any studies with human participants or animals performed by any of the authors.

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The authors all declare that conflicts of interest do not exist.

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Correspondence to Chi Lin.

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Verma, V., Johnson, C.P., Bennion, N.R. et al. Atypical teratoid rhabdoid tumor: long-term survival after chemoradiotherapy. Childs Nerv Syst 31, 1393–1399 (2015). https://doi.org/10.1007/s00381-015-2723-5

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Keywords

  • Atypical teratoid rhabdoid tumor
  • Pediatric brain tumors
  • Radiotherapy
  • Chemotherapy