title: Evaluation of the good tumor response of embryonal tumor with abundant neuropil and true rosettes (ETANTR).
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.
authors.affiliation: Department of Oncotherapy, Faculty of Medicine, University of Szeged, Szeged, Hungary. mozespetra@gmail.com.|Department of Radiation Oncology, Klinikum rechts der Isar, Technische Universität München, Ismaninger Str. 22, 81675, Munich, Germany. mozespetra@gmail.com.
authors.forename: Petra
authors.initials: P
authors.lastname: Mozes
authors.affiliation: 2nd Department of Paediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: Péter
authors.initials: P
authors.lastname: Hauser
authors.affiliation: Department of Pathology, Medical Center, University of Debrecen, Debrecen, Hungary.
authors.forename: Tibor
authors.initials: T
authors.lastname: Hortobágyi
authors.affiliation: Paediatric Haematology and Stem Cell Transplantation Unit, St. István and St. László Hospital, Budapest, Hungary.
authors.forename: Gábor
authors.initials: G
authors.lastname: Benyó
authors.affiliation: MTA-SE Pathobiochemistry Research Group, Department of Medical Chemistry, Semmelweis University, Budapest, Hungary.
authors.forename: István
authors.initials: I
authors.lastname: Peták
authors.affiliation: 2nd Department of Paediatrics, Semmelweis University, Budapest, Hungary.
authors.forename: Miklós
authors.initials: M
authors.lastname: Garami
authors.affiliation: Department of Oncotherapy, Faculty of Medicine, University of Szeged, Szeged, Hungary.
authors.forename: Adrienne
authors.initials: A
authors.lastname: Cserháti
authors.affiliation: Department of Paediatrics and Paediatric Health Care Center, Faculty of Medicine, University of Szeged, Szeged, Hungary.
authors.forename: Katalin
authors.initials: K
authors.lastname: Bartyik
authors.affiliation: Department of Neurosurgery, Medical Center, University of Debrecen, Debrecen, Hungary.
authors.forename: László
authors.initials: L
authors.lastname: Bognár
authors.affiliation: Department of Oncotherapy, Faculty of Medicine, University of Szeged, Szeged, Hungary.
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Nagy
authors.affiliation: 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary.
authors.forename: Eszter
authors.initials: E
authors.lastname: Turányi
authors.affiliation: Department of Oncotherapy, Faculty of Medicine, University of Szeged, Szeged, Hungary.
authors.forename: Katalin
authors.initials: K
authors.lastname: Hideghéty
mesh_terms: D001932:Brain Neoplasms / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D019394:Ki-67 Antigen / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D009373:Neoplasms, Germ Cell and Embryonal / Q000473:pathology*; D019581:Neuropil / Q000473:pathology*
chemical_list: D019394:Ki-67 Antigen
keywords: Craniospinal axis; ETANTR; Radiochemotherapy; Temozolomide