A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old

We present a unique and challenging case of a high-grade neuroepithelial tumor with echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase (EML4-ALK) fusion in a five-month-old child. This tumor was difficult to classify, with glial and ependymal features, reinforcing the impact...

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Autores principales: Mrowczynski, Oliver D, Payne, Russell, Pu, Cunfeng, Greiner, Robert, Rizk, Elias
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Neurosurgery
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7366042/
https://www.ncbi.nlm.nih.gov/pubmed/32685319
http://dx.doi.org/10.7759/cureus.8654
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author Mrowczynski, Oliver D
Payne, Russell
Pu, Cunfeng
Greiner, Robert
Rizk, Elias
author_facet Mrowczynski, Oliver D
Payne, Russell
Pu, Cunfeng
Greiner, Robert
Rizk, Elias
author_sort Mrowczynski, Oliver D
collection PubMed
description We present a unique and challenging case of a high-grade neuroepithelial tumor with echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase (EML4-ALK) fusion in a five-month-old child. This tumor was difficult to classify, with glial and ependymal features, reinforcing the impact of a molecular-based diagnosis in correct classification and management. The patient had two tumor resections and underwent chemotherapy following the Head Start trial treatment regimen. The patient remains well, with no residual disease on MRI 15 months after initial resection. Further studies are needed to determine the frequency of EML4-ALK fusions in these types of tumors and to optimize therapeutic protocols for children and adults, alike, suffering from this disease.
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spelling pubmed-73660422020-07-17 A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old Mrowczynski, Oliver D Payne, Russell Pu, Cunfeng Greiner, Robert Rizk, Elias Cureus Neurosurgery We present a unique and challenging case of a high-grade neuroepithelial tumor with echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase (EML4-ALK) fusion in a five-month-old child. This tumor was difficult to classify, with glial and ependymal features, reinforcing the impact of a molecular-based diagnosis in correct classification and management. The patient had two tumor resections and underwent chemotherapy following the Head Start trial treatment regimen. The patient remains well, with no residual disease on MRI 15 months after initial resection. Further studies are needed to determine the frequency of EML4-ALK fusions in these types of tumors and to optimize therapeutic protocols for children and adults, alike, suffering from this disease. Cureus 2020-06-16 /pmc/articles/PMC7366042/ /pubmed/32685319 http://dx.doi.org/10.7759/cureus.8654 Text en Copyright © 2020, Mrowczynski et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Neurosurgery
Mrowczynski, Oliver D
Payne, Russell
Pu, Cunfeng
Greiner, Robert
Rizk, Elias
A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old
title A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old
title_full A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old
title_fullStr A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old
title_full_unstemmed A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old
title_short A Unique Case of a High-Grade Neuroepithelial Tumor With EML4-ALK Fusion in a Five-Month-Old
title_sort unique case of a high-grade neuroepithelial tumor with eml4-alk fusion in a five-month-old
topic Neurosurgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7366042/
https://www.ncbi.nlm.nih.gov/pubmed/32685319
http://dx.doi.org/10.7759/cureus.8654
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