Evaluation of the good tumor response of embryonal tumor with abundant neuropil and true rosettes (ETANTR).
J Neurooncol
; 126(1): 99-105, 2016 Jan.
Article
em En
| MEDLINE
| ID: mdl-26373296
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Encefálicas
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Neoplasias Embrionárias de Células Germinativas
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Neurópilo
Limite:
Child, preschool
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Female
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Humans
Idioma:
En
Revista:
J Neurooncol
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Hungria