Evaluation of the good tumor response of embryonal tumor with abundant neuropil and true rosettes (ETANTR)
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 m...
Elmentve itt :
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| Dokumentumtípus: | Cikk |
| Megjelent: |
2016
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| Sorozat: | JOURNAL OF NEURO-ONCOLOGY
126 No. 1 |
| doi: | 10.1007/s11060-015-1938-3 |
| mtmt: | 2946462 |
| Online Access: | http://publicatio.bibl.u-szeged.hu/12218 |
| Tartalmi kivonat: | 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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| Terjedelem/Fizikai jellemzők: | 99-105 |
| ISSN: | 0167-594X |