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

Mozes, P ✉ [Mózes, Petra (Sugárterápia), author] Department of Oncotherapy (USZ / ASZMS); Hauser, P [Hauser, Péter (Gyermekkori közpo...), author] II. Department of Pediatrics (SU / FM / C); Hortobagyi, T [Hortobágyi, Tibor (Neuropathológia), author] Department of Pathology (UD); Benyo, G [Benyó, Gábor (Gyermekgyógyászat...), author]; Petak, I [Peták, István (farmakológia), author] MTA-SE Pathobiokémiai Kutatócsoport (2006-ig: M... (SU / FM / I / BMBI / MBT); Garami, M [Garami, Miklós (Gyermekgyógyászat), author] II. Department of Pediatrics (SU / FM / C); Cserhati, A [Cserháti, Adrienn (radiológia), author] Department of Oncotherapy (USZ / ASZMS); Bartyik, K [Bartyik, Katalin (gyermekgyógyászat), author] Department of Paediatrics and Paediatric Health... (USZ / ASZMS); Bognar, L [Bognár, László (Idegsebészet), author]; Nagy, Z [Nagy, Zoltán (Fizika), author] Department of Oncotherapy (USZ / ASZMS); Turanyi, E [Turányi, Eszter (Neuropatológia), author] I. Department of Pathology and experimental Can... (SU / FM / I); Hideghety, K [Hideghéty, Katalin (Sugárterápia), author] Department of Oncotherapy (USZ / ASZMS)

English Scientific Article (Journal Article)
Published: JOURNAL OF NEURO-ONCOLOGY 0167-594X 1573-7373 126 (1) pp. 99-105 2016
  • SJR Scopus - Cancer Research: Q2
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    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.
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    2021-12-01 13:13