SynthesisChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2018
Extracranial extra-CNS spread of embryonal tumor with multilayered rosettes (ETMR): case series and systematic review.
Synthesis in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Extra-Neural Metastases From Primary Intracranial Ependymomas: A Systematic Review.Frontiers in oncology · 2022Pooled it
- Timing of radiotherapy and autologous stem cell transplantation in embryonal tumor with multilayered rosettes: a critical narrative review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Review
- Freiburg Neuropathology Case Conference: A 2-Year-Old Child with Progressive Headache, Nausea, and Personality Change.Clinical neuroradiology · 2026Article
- Embryonal Tumors of the Central Nervous System with Multilayered Rosettes and Atypical Teratoid/Rhabdoid Tumors.Advances in experimental medicine and biology · 2023Article
- Embryonal tumors with multi-layered rosettes: a disease of dysregulated miRNAs.Journal of neuro-oncology · 2020Review
- ETMR: a tumor entity in its infancy.Acta neuropathologica · 2020Review
- Embryonal tumor with multilayered rosettes: illustrative case and review of the literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2018Review
Corrections and comments
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Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEmbryonal tumors with multilayered rosettes (ETMR) is a rare variant of embryonal tumor of infancy with nearly 200 cases reported in the literature. Leptomeningeal spread of this tumor is well known; however, extracranial metastasis has been sparsely reported in the literature.
methodsOur study was divided into two sections: (1) We conducted a retrospective review of our patient series of ETMR and screened for patients with evidence of ETMR over the last 10 years 2007-2017 at a single tertiary referral pediatric hospital, and (2) we conducted a systematic review according to PRISMA guidelines of all reported cases of ETMR to determine the incidence of extracranial metastasis and treatment paradigms.
resultsHere we report three cases of extracranial non-CNS spread of ETMR and conduct a systematic review of ETMR to improve our understanding of ETMR metastases and treatment paradigms. In our systematic review (n = 204), median overall survival was less than 1 year with 44.1% children surviving over 1 year. Previously, only five cases of extracranial metastasis of ETMR have been reported.
conclusionsOur case series (n = 3) and review demonstrate that these tumors may behave like soft tissue sarcomas and may be susceptible to tumor seeding through surgical manipulation or by CSF (ventriculoperitoneal shunt). Surgery for tumor recurrence may offer an improved local disease control, but preventative measures such as meticulous surgical resection may be necessary to reduce intraoperative contamination.
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