Disseminated Cerebrospinal Embryonal Tumor in the Adult

Introduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient. Illust...

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Main Authors: Alessandro Caporlingua, Daniele Armocida, Federico Caporlingua, Gennaro Lapadula, Grazia Maria Elefante, Manila Antonelli, Maurizio Salvati
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2016/6785459
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author Alessandro Caporlingua
Daniele Armocida
Federico Caporlingua
Gennaro Lapadula
Grazia Maria Elefante
Manila Antonelli
Maurizio Salvati
author_facet Alessandro Caporlingua
Daniele Armocida
Federico Caporlingua
Gennaro Lapadula
Grazia Maria Elefante
Manila Antonelli
Maurizio Salvati
author_sort Alessandro Caporlingua
collection DOAJ
description Introduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient. Illustrative Case. A 49-year-old male presenting with low back pain, dysuria, and hypoesthesia of the lower extremities referred to our emergency department. Brain and whole spine contrast-enhanced MRI documented a diffusively disseminated heterogeneous neoplasm with intradural extra- and intramedullary involvement of the cervicothoracic tract and cauda equina. A primary biopsy of the lumbosacral localization was performed through L5 bilateral laminectomy. Histologic diagnosis was Embryonal Tumor Not Otherwise Specified. The patient underwent chemotherapy with postoperative adjuvant alternating Vincristine-Doxorubicin-Ifosfamide (VAI) and Ifosfamide-Etoposide (IE). Discussion. Spinal ETs are exceedingly rare especially when presenting in the adult patient. Neurosurgical and oncologic management is still unclear. When feasible, surgical removal should always be performed to obtain a histologic diagnosis. Postoperative adjuvant therapy might entail both chemo- and radiotherapy; however a consensus on this matter is still lacking.
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spelling doaj-art-b0578be8d23c41c2804b9210dcc456ee2025-02-03T01:22:32ZengWileyCase Reports in Pathology2090-67812090-679X2016-01-01201610.1155/2016/67854596785459Disseminated Cerebrospinal Embryonal Tumor in the AdultAlessandro Caporlingua0Daniele Armocida1Federico Caporlingua2Gennaro Lapadula3Grazia Maria Elefante4Manila Antonelli5Maurizio Salvati6Department of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, ItalyDepartment of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, ItalyDepartment of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, ItalyDepartment of Neurology and Psychiatry, Neurosurgery, Sapienza University of Rome, Rome, ItalyDepartment of Radiological, Oncological and Anatomo-Pathological Sciences, Sapienza University of Rome, Rome, ItalyDepartment of Radiological, Oncological and Anatomo-Pathological Sciences, Sapienza University of Rome, Rome, ItalyDepartment of Science and Medical Surgical Biotechnology, Sapienza University of Rome, Rome, ItalyIntroduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient. Illustrative Case. A 49-year-old male presenting with low back pain, dysuria, and hypoesthesia of the lower extremities referred to our emergency department. Brain and whole spine contrast-enhanced MRI documented a diffusively disseminated heterogeneous neoplasm with intradural extra- and intramedullary involvement of the cervicothoracic tract and cauda equina. A primary biopsy of the lumbosacral localization was performed through L5 bilateral laminectomy. Histologic diagnosis was Embryonal Tumor Not Otherwise Specified. The patient underwent chemotherapy with postoperative adjuvant alternating Vincristine-Doxorubicin-Ifosfamide (VAI) and Ifosfamide-Etoposide (IE). Discussion. Spinal ETs are exceedingly rare especially when presenting in the adult patient. Neurosurgical and oncologic management is still unclear. When feasible, surgical removal should always be performed to obtain a histologic diagnosis. Postoperative adjuvant therapy might entail both chemo- and radiotherapy; however a consensus on this matter is still lacking.http://dx.doi.org/10.1155/2016/6785459
spellingShingle Alessandro Caporlingua
Daniele Armocida
Federico Caporlingua
Gennaro Lapadula
Grazia Maria Elefante
Manila Antonelli
Maurizio Salvati
Disseminated Cerebrospinal Embryonal Tumor in the Adult
Case Reports in Pathology
title Disseminated Cerebrospinal Embryonal Tumor in the Adult
title_full Disseminated Cerebrospinal Embryonal Tumor in the Adult
title_fullStr Disseminated Cerebrospinal Embryonal Tumor in the Adult
title_full_unstemmed Disseminated Cerebrospinal Embryonal Tumor in the Adult
title_short Disseminated Cerebrospinal Embryonal Tumor in the Adult
title_sort disseminated cerebrospinal embryonal tumor in the adult
url http://dx.doi.org/10.1155/2016/6785459
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