Embryonal tumors with multilayered rosettes: A tertiary care centre experience.
Chemotherapy
Embryonal tumors with multilayered rosettes (ETMR)
Radiotherapy
Surgery
Journal
Clinical neurology and neurosurgery
ISSN: 1872-6968
Titre abrégé: Clin Neurol Neurosurg
Pays: Netherlands
ID NLM: 7502039
Informations de publication
Date de publication:
Mar 2021
Mar 2021
Historique:
received:
03
12
2020
revised:
15
01
2021
accepted:
16
01
2021
pubmed:
9
2
2021
medline:
18
1
2022
entrez:
8
2
2021
Statut:
ppublish
Résumé
Embryonal tumors with multilayered rosettes (ETMR) is an extremely rare and highly aggressive tumor. It includes three distinct entities i.e, embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL) and medulloepithelioma (MEPL). Here, we present our institutional experience of seven ETMR cases treated over a period of five years. Patients' records from 2015 to 2019 were reviewed manually and electronically to retrieve the data. Clinicopathological and outcome details of ETMR cases were entered in a predesigned proforma. A total of seven cases of ETMR were registered from 2015 to 2019 with a median age at presentation of four years (range 3-7 years). All patients underwent surgery. However, only three patients completed the planned adjuvant treatment, comprising of focal radiotherapy (RT) alone, craniospinal irradiation (CSI) alone and CSI followed by six cycles of chemotherapy in one patient each respectively. Two patients commenced CSI but deteriorated during RT and thereafter needed best supportive care. Two patients could not be started on any adjuvant treatment. Unfortunately, six patients succumbed to their disease within one year of their diagnosis. Only one patient who received both CSI and adjuvant chemotherapy is alive at 15 months of diagnosis. ETMR is a rare and aggressive entity. Majority of the patients die within one year of the diagnosis despite multimodality treatment.
Sections du résumé
BACKGROUND
BACKGROUND
Embryonal tumors with multilayered rosettes (ETMR) is an extremely rare and highly aggressive tumor. It includes three distinct entities i.e, embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL) and medulloepithelioma (MEPL). Here, we present our institutional experience of seven ETMR cases treated over a period of five years.
MATERIALS AND METHODS
METHODS
Patients' records from 2015 to 2019 were reviewed manually and electronically to retrieve the data. Clinicopathological and outcome details of ETMR cases were entered in a predesigned proforma.
RESULTS
RESULTS
A total of seven cases of ETMR were registered from 2015 to 2019 with a median age at presentation of four years (range 3-7 years). All patients underwent surgery. However, only three patients completed the planned adjuvant treatment, comprising of focal radiotherapy (RT) alone, craniospinal irradiation (CSI) alone and CSI followed by six cycles of chemotherapy in one patient each respectively. Two patients commenced CSI but deteriorated during RT and thereafter needed best supportive care. Two patients could not be started on any adjuvant treatment. Unfortunately, six patients succumbed to their disease within one year of their diagnosis. Only one patient who received both CSI and adjuvant chemotherapy is alive at 15 months of diagnosis.
CONCLUSION
CONCLUSIONS
ETMR is a rare and aggressive entity. Majority of the patients die within one year of the diagnosis despite multimodality treatment.
Identifiants
pubmed: 33556852
pii: S0303-8467(21)00035-4
doi: 10.1016/j.clineuro.2021.106508
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
106508Informations de copyright
Copyright © 2021 Elsevier B.V. All rights reserved.