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Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis

Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial tumor in the central nervous system. Definitive diagnosis of this neoplasm is based on histopathologic analysis evaluating distinctive findings such as the fibroblastic differentiation. Here we present a clinical case of DIG with a...

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Institution:Universidad EIA
Main Authors: Romero Rojas, Alfredo E., Diaz Perez, Julio A., Lozano Castillo, Alfonso
Format: Artículo de revista
Language:English
Published: 2013-12
Subjects:
Desmoplastic ganglioglioma
Radiology
Histopathology
Immunohistochemistry
Children's tumors
Online Access:https://repositorio.udes.edu.co/handle/001/3610
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id oai:repositorio.udes.edu.co:001-3610
recordtype dspace
spelling Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
2019-08-21T17:49:28Z
2019-08-21T17:49:28Z
2013-12
6 p.
Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial tumor in the central nervous system. Definitive diagnosis of this neoplasm is based on histopathologic analysis evaluating distinctive findings such as the fibroblastic differentiation. Here we present a clinical case of DIG with a long follow-up in an eight-year-old boy with a six-month history of recurrent emesis, psychomotor hyperactivity and generalized tonic-clonic seizures. Computed tomography scan and magnetic resonance imaging (MRI) showed a cystic, heterogeneous, mass on the right temporal uncus. A histopathological diagnosis of late presentation DIG was made. We documented the immunohistochemical expression of a molecular soft tissue / muscle differentiation marker (h-CaD) in addition to a low proliferative index (Ki-67) in this case. After surgical intervention, a control MRI showed changes of right frontal-temporal craniotomy and a persistent mass in the anterior and medial temporal lobe with basal extension. Further surgical intervention was performed, completely removing the tumor, which had the same characteristics. The patient is asymptomatic while receiving anticonvulsant therapy (phenytoin) with no evidence of tumor recurrence on MRI after a follow-up of five years. The low grade and soft tissue appearance in images are correlated with the histopathologic and immunohistochemical profile of this tumor, but the rarity of this tumor makes a presumptive diagnosis by images a challenge. The above-mentioned molecular markers or new ones could be used as molecular targets for molecular imaging studies to increase the probability of a pre-operative diagnosis based on molecular features through images.
application/pdf
10.1177/197140091302600607
2385-1996
1971-4009
https://repositorio.udes.edu.co/handle/001/3610
eng
The Neuroradiology Journal
Neuroradiology Journal;Vol. 26 (6), pp. 649-654. 2013
Derechos Reservados - Universidad de Santander, 2013
info:eu-repo/semantics/openAccess
Atribución-NoComercial 4.0 Internacional (CC BY-NC 4.0)
https://creativecommons.org/licenses/by-nc/4.0/
https://journals.sagepub.com/doi/10.1177/197140091302600607
Desmoplastic ganglioglioma
Radiology
Histopathology
Immunohistochemistry
Children's tumors
Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
Artículo de revista
http://purl.org/coar/resource_type/c_6501
Text
info:eu-repo/semantics/article
http://purl.org/redcol/resource_type/ART
info:eu-repo/semantics/publishedVersion
Publication
http://purl.org/coar/access_right/c_abf2
http://purl.org/coar/version/c_970fb48d4fbd8a85
institution Universidad EIA
collection d_repositorio.udes.edu.co-DSPACE
title Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
spellingShingle Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
Desmoplastic ganglioglioma
Radiology
Histopathology
Immunohistochemistry
Children's tumors
title_short Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
title_full Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
title_fullStr Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
title_full_unstemmed Desmoplastic infantile ganglioglioma with late presentation : A clinical, radiological and histopathological analysis
title_sort desmoplastic infantile ganglioglioma with late presentation : a clinical, radiological and histopathological analysis
author Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
author_facet Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
Romero Rojas, Alfredo E.
Diaz Perez, Julio A.
Lozano Castillo, Alfonso
building Repositorio digital
topic Desmoplastic ganglioglioma
Radiology
Histopathology
Immunohistochemistry
Children's tumors
topic_facet Desmoplastic ganglioglioma
Radiology
Histopathology
Immunohistochemistry
Children's tumors
publishDate 2013-12
language English
format Artículo de revista
description Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial tumor in the central nervous system. Definitive diagnosis of this neoplasm is based on histopathologic analysis evaluating distinctive findings such as the fibroblastic differentiation. Here we present a clinical case of DIG with a long follow-up in an eight-year-old boy with a six-month history of recurrent emesis, psychomotor hyperactivity and generalized tonic-clonic seizures. Computed tomography scan and magnetic resonance imaging (MRI) showed a cystic, heterogeneous, mass on the right temporal uncus. A histopathological diagnosis of late presentation DIG was made. We documented the immunohistochemical expression of a molecular soft tissue / muscle differentiation marker (h-CaD) in addition to a low proliferative index (Ki-67) in this case. After surgical intervention, a control MRI showed changes of right frontal-temporal craniotomy and a persistent mass in the anterior and medial temporal lobe with basal extension. Further surgical intervention was performed, completely removing the tumor, which had the same characteristics. The patient is asymptomatic while receiving anticonvulsant therapy (phenytoin) with no evidence of tumor recurrence on MRI after a follow-up of five years. The low grade and soft tissue appearance in images are correlated with the histopathologic and immunohistochemical profile of this tumor, but the rarity of this tumor makes a presumptive diagnosis by images a challenge. The above-mentioned molecular markers or new ones could be used as molecular targets for molecular imaging studies to increase the probability of a pre-operative diagnosis based on molecular features through images.
issn 2385-1996
url https://repositorio.udes.edu.co/handle/001/3610
url_str_mv https://repositorio.udes.edu.co/handle/001/3610
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