Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFRGS |
Texto Completo: | http://hdl.handle.net/10183/263096 |
Resumo: | Purpose: Central nervous system embryonal tumors, not otherwise specified (ETNOS), comprise a group of rare, poorly differentiated embryonal neoplasms of neuroectodermal origin (WHO grade IV) that lack the specific histopathological features and molecular alterations that define other embryonal tumors, such as medulloblas toma and embryonal tumors with multilayered rosettes, C19MC altered. ETNOS accounts for less than 0.5% of all brain tumors and predominantly affects children aged 0–14 years. Case report: The authors report an autopsy case of ETNOS in a female newborn of 22 weeks of gestational age. The brain weighed 68.0 g and measured 61.0 mm in occipitofrontal diameter. A white-gray, soft, infiltrative tumor measuring 0.9 cm in the largest dimension was found in the right lateral ventricle. Microscopic exami nation revealed a high-grade malignant neoplasm composed of small round blue cells, with marked atypia, solid pattern, and high mitotic index. Neoplastic cells showed positive immunostaining for synaptophysin, GFAP, NFP, CD99, INI1, vimentin, and EMA. The diagnosis of ETNOS was thus established. Differential diagnosis includes other embryonal tumors, such as medulloepithelioma, neuroblastoma, ganglioneuroblastoma, and atypical rhabdoid/teratoid tumor. Treatment is usually surgical resection combined with craniospinal irradiation and/or multimodality chemotherapy. Conclusions: Congenital ETNOS are rare high-grade pediatric tumors, probably originated from primitive neu roepithelial cells. Overall, neoplastic cells are positive for synaptophysin, GFAP, INI1, and CD99. Immunohis tochemistry panels are fundamental to establish the diagnosis. |
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Cambruzzi, EduardoPêgas, Karla LaisNascimento, Gabriella Bezerra CortêsSilva, Jose Nathan Andrade Muller daZandoná, Natália BrandelliMedeiros, Mateus Scarabelot2023-08-03T03:33:14Z20212214-7519http://hdl.handle.net/10183/263096001173377Purpose: Central nervous system embryonal tumors, not otherwise specified (ETNOS), comprise a group of rare, poorly differentiated embryonal neoplasms of neuroectodermal origin (WHO grade IV) that lack the specific histopathological features and molecular alterations that define other embryonal tumors, such as medulloblas toma and embryonal tumors with multilayered rosettes, C19MC altered. ETNOS accounts for less than 0.5% of all brain tumors and predominantly affects children aged 0–14 years. Case report: The authors report an autopsy case of ETNOS in a female newborn of 22 weeks of gestational age. The brain weighed 68.0 g and measured 61.0 mm in occipitofrontal diameter. A white-gray, soft, infiltrative tumor measuring 0.9 cm in the largest dimension was found in the right lateral ventricle. Microscopic exami nation revealed a high-grade malignant neoplasm composed of small round blue cells, with marked atypia, solid pattern, and high mitotic index. Neoplastic cells showed positive immunostaining for synaptophysin, GFAP, NFP, CD99, INI1, vimentin, and EMA. The diagnosis of ETNOS was thus established. Differential diagnosis includes other embryonal tumors, such as medulloepithelioma, neuroblastoma, ganglioneuroblastoma, and atypical rhabdoid/teratoid tumor. Treatment is usually surgical resection combined with craniospinal irradiation and/or multimodality chemotherapy. Conclusions: Congenital ETNOS are rare high-grade pediatric tumors, probably originated from primitive neu roepithelial cells. Overall, neoplastic cells are positive for synaptophysin, GFAP, INI1, and CD99. Immunohis tochemistry panels are fundamental to establish the diagnosis.application/pdfengInterdisciplinary neurosurgery. [Amsterdam]. Vol. 23 (Mar. 2021), 100913, 4 p.Neoplasias do sistema nervoso centralNeoplasias encefálicasImuno-histoquímicaEmbryonal tumor, not otherwise specifiedBrain tumorCentral nervous systemPathologyImmunohistochemistryPrognosisAutopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specifiedEstrangeiroinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSTEXT001173377.pdf.txt001173377.pdf.txtExtracted Texttext/plain0http://www.lume.ufrgs.br/bitstream/10183/263096/2/001173377.pdf.txtd41d8cd98f00b204e9800998ecf8427eMD52ORIGINAL001173377.pdfTexto completoapplication/pdf5160650http://www.lume.ufrgs.br/bitstream/10183/263096/1/001173377.pdff3472480ea62ee39ea809a0a81f269e6MD5110183/2630962023-08-04 03:32:37.085812oai:www.lume.ufrgs.br:10183/263096Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2023-08-04T06:32:37Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false |
dc.title.pt_BR.fl_str_mv |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
title |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
spellingShingle |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified Cambruzzi, Eduardo Neoplasias do sistema nervoso central Neoplasias encefálicas Imuno-histoquímica Embryonal tumor, not otherwise specified Brain tumor Central nervous system Pathology Immunohistochemistry Prognosis |
title_short |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
title_full |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
title_fullStr |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
title_full_unstemmed |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
title_sort |
Autopsy report and review of the 2016 WHO classification of congenital supratentorial embryonal tumors, not otherwise specified |
author |
Cambruzzi, Eduardo |
author_facet |
Cambruzzi, Eduardo Pêgas, Karla Lais Nascimento, Gabriella Bezerra Cortês Silva, Jose Nathan Andrade Muller da Zandoná, Natália Brandelli Medeiros, Mateus Scarabelot |
author_role |
author |
author2 |
Pêgas, Karla Lais Nascimento, Gabriella Bezerra Cortês Silva, Jose Nathan Andrade Muller da Zandoná, Natália Brandelli Medeiros, Mateus Scarabelot |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Cambruzzi, Eduardo Pêgas, Karla Lais Nascimento, Gabriella Bezerra Cortês Silva, Jose Nathan Andrade Muller da Zandoná, Natália Brandelli Medeiros, Mateus Scarabelot |
dc.subject.por.fl_str_mv |
Neoplasias do sistema nervoso central Neoplasias encefálicas Imuno-histoquímica |
topic |
Neoplasias do sistema nervoso central Neoplasias encefálicas Imuno-histoquímica Embryonal tumor, not otherwise specified Brain tumor Central nervous system Pathology Immunohistochemistry Prognosis |
dc.subject.eng.fl_str_mv |
Embryonal tumor, not otherwise specified Brain tumor Central nervous system Pathology Immunohistochemistry Prognosis |
description |
Purpose: Central nervous system embryonal tumors, not otherwise specified (ETNOS), comprise a group of rare, poorly differentiated embryonal neoplasms of neuroectodermal origin (WHO grade IV) that lack the specific histopathological features and molecular alterations that define other embryonal tumors, such as medulloblas toma and embryonal tumors with multilayered rosettes, C19MC altered. ETNOS accounts for less than 0.5% of all brain tumors and predominantly affects children aged 0–14 years. Case report: The authors report an autopsy case of ETNOS in a female newborn of 22 weeks of gestational age. The brain weighed 68.0 g and measured 61.0 mm in occipitofrontal diameter. A white-gray, soft, infiltrative tumor measuring 0.9 cm in the largest dimension was found in the right lateral ventricle. Microscopic exami nation revealed a high-grade malignant neoplasm composed of small round blue cells, with marked atypia, solid pattern, and high mitotic index. Neoplastic cells showed positive immunostaining for synaptophysin, GFAP, NFP, CD99, INI1, vimentin, and EMA. The diagnosis of ETNOS was thus established. Differential diagnosis includes other embryonal tumors, such as medulloepithelioma, neuroblastoma, ganglioneuroblastoma, and atypical rhabdoid/teratoid tumor. Treatment is usually surgical resection combined with craniospinal irradiation and/or multimodality chemotherapy. Conclusions: Congenital ETNOS are rare high-grade pediatric tumors, probably originated from primitive neu roepithelial cells. Overall, neoplastic cells are positive for synaptophysin, GFAP, INI1, and CD99. Immunohis tochemistry panels are fundamental to establish the diagnosis. |
publishDate |
2021 |
dc.date.issued.fl_str_mv |
2021 |
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2023-08-03T03:33:14Z |
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001173377 |
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dc.relation.ispartof.pt_BR.fl_str_mv |
Interdisciplinary neurosurgery. [Amsterdam]. Vol. 23 (Mar. 2021), 100913, 4 p. |
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