Evaluation of the good tumor response of embryonal tumor with abundant neuropil and true rosettes (ETANTR)

Petra Mozes, Péter Hauser, Tibor Hortobágyi, Gábor Benyó, István Peták, Miklós Garami, Adrienne Cserháti, Katalin Bartyik, László Bognár, Zoltán Nagy, Eszter Turányi, Katalin Hideghéty

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5 Citations (Scopus)

Abstract

The embryonal tumor with abundant neuropil and true rosettes is a rare and highly malignant variant of embryonal brain tumors. It usually affects infants and young children under the age of 4 years and exhibits a very aggressive course with a dismal prognosis. For the 68 cases reported to date the mean age at diagnosis was 25.42 months (range 3–57 months). Survival data are available for 48 children (including our case): the median overall survival is 13.0 months, though 6 (9 %) of the children have had a relative long survival (>30 months). The aggressive combined treatment, involving primary surgical tumor removal, adjuvant polychemotherapy, including high-dose chemotherapy with stem cell transplantation, radiotherapy and radiochemotherapy, might play an important role in the longer survival. We have performed a literature review and we present here a multimodal-treated case of a 2- year-old girl with a long survival, who was reoperated when recurrence occurred. The residual tumor demonstrated a good response to temozolomide radiochemotherapy (craniospinal axis + boost) and followed by maintenance temozolomide. The described complex aggressive treatment option might be considered for future cases of this tumor entity.

Original languageEnglish
Pages (from-to)99-105
Number of pages7
JournalJournal of Neuro-Oncology
Volume126
Issue number1
DOIs
Publication statusPublished - Jan 1 2016

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Keywords

  • Craniospinal axis
  • ETANTR
  • Radiochemotherapy
  • Temozolomide

ASJC Scopus subject areas

  • Oncology
  • Neurology
  • Clinical Neurology
  • Cancer Research

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