Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report

Detalhes bibliográficos
Autor(a) principal: Borba, Flávia Marcella Sena Gonçalves
Data de Publicação: 2022
Outros Autores: Almeida, Andressa de, Tomaz, Emanuelle Scarlath, Silva, Nágella Samara Eleutério da, Louzada, Cibelle Ferreira
Tipo de documento: Artigo
Idioma: por
eng
Título da fonte: Revista Brasileira de Cancerologia (Online)
Texto Completo: https://rbc.inca.gov.br/index.php/revista/article/view/2515
Resumo: Introduction: Neuroblastoma is an extracranial tumor originated from neural crest cell failures. It’s the most common malignant neoplasm among infants and it presents a very heterogeneous clinical profile. The objective of the article is to report the case of a patient with neuroblastoma in advanced stage and satisfactory survival, despite the poor prognostic factors. Case report: Female patient, 1 year and 4 months old who presented irritability, abdominal distention and intermittent fever with three months of evolution. In physical exam, she presented alopecia suggestive of trichotillomania, cervical, inguinal and axillary lymph node enlargement and abdominal distention. Abdominal computed tomography evidenced expansive mass, measuring 6.8 x 5.8 x 4.0 cm, located in the left anterior pararenal space. Bone scintigraphy and nuclear magnetic resonance showed images suggestive of distant secondary implants. After total tumor excision, it was confirmed poorly differentiated neuroblastoma, with non-amplified MYCN gene and unfavorable histology. Bilateral iliac crest biopsy revealed bone marrow areas occupied by the neoplasm, suggesting bone marrow infiltration. As this was a stage IV neuroblastoma, multimodal therapy was established with adjuvant chemotherapy after surgery, followed by autologous bone marrow transplantation, radiotherapy at the primary site of the lesion and at the sites of bone metastases and use of 13-cis-retinoic acid. Conclusion: Despite presenting several indicators of poor prognosis (age, stage IV, bone metastases, bone marrow infiltration), the patient has been in complete remission of the disease for 39 months.
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spelling Clinical Aspects and Prognostic Factors of Neuroblastoma: Case ReportAspectos Clínicos y Factores Pronósticos del Neuroblastoma: Reporte de CasoAspectos Clínicos e Fatores Prognósticos do Neuroblastoma: Relato de Casoneuroblastomaprognósticoneoplasiasrelatos de casosneuroblastomaprognosismedical neoplasmscase reportsneuroblastomapronósticoneoplasiasinformes de casosIntroduction: Neuroblastoma is an extracranial tumor originated from neural crest cell failures. It’s the most common malignant neoplasm among infants and it presents a very heterogeneous clinical profile. The objective of the article is to report the case of a patient with neuroblastoma in advanced stage and satisfactory survival, despite the poor prognostic factors. Case report: Female patient, 1 year and 4 months old who presented irritability, abdominal distention and intermittent fever with three months of evolution. In physical exam, she presented alopecia suggestive of trichotillomania, cervical, inguinal and axillary lymph node enlargement and abdominal distention. Abdominal computed tomography evidenced expansive mass, measuring 6.8 x 5.8 x 4.0 cm, located in the left anterior pararenal space. Bone scintigraphy and nuclear magnetic resonance showed images suggestive of distant secondary implants. After total tumor excision, it was confirmed poorly differentiated neuroblastoma, with non-amplified MYCN gene and unfavorable histology. Bilateral iliac crest biopsy revealed bone marrow areas occupied by the neoplasm, suggesting bone marrow infiltration. As this was a stage IV neuroblastoma, multimodal therapy was established with adjuvant chemotherapy after surgery, followed by autologous bone marrow transplantation, radiotherapy at the primary site of the lesion and at the sites of bone metastases and use of 13-cis-retinoic acid. Conclusion: Despite presenting several indicators of poor prognosis (age, stage IV, bone metastases, bone marrow infiltration), the patient has been in complete remission of the disease for 39 months.Introducción: El neuroblastoma es un tumor extracraneal originado a partir de fallas de células de cresta neural. Es la neoplasia maligna más común de los bebés y tiene un perfil clínico muy heterogéneo. El objetivo de este artículo es informar un caso de un paciente con neuroblastoma avanzado y supervivencia satisfactoria, a pesar de los factores de mal pronóstico. Relato del caso: Paciente femenina, de 1 año y 4 meses, comenzó con irritabilidad, distensión abdominal y fiebre intermitente con tres meses de evolución. En examen físico, presentó alopecia sugerente de tricotilomanía, ganglio linfático megaly en las cadenas cervicales, inguinales y axilares y distensión abdominal. La tomografía computarizada del abdomen mostró una masa expansiva, que mide 6,8 x 5,8 x 4,0 cm, situada en el espacio pararrenal anterior izquierdo. La centellografía ósea y la resonancia magnética, se visualizaron imágenes sugestivas de implantes secundarios a distancia. Después de la escisión total del tumor, se confirmó neuroblastoma poco diferenciado, con un gen MYCN no amplificado e histología desfavorable. La biopsia de la cresta ilíaca bilateral reveló áreas de la médula ocupadas por neoplasia compatibles con infiltración medular. Debido a que se trata de neuroblastoma en estadio IV, se estableció terapia multimodal, con quimioterapia adyuvante después de la cirugía, seguida de trasplante autólogo de médula ósea, radioterapia en el sitio primario de la lesión y en los sitios de metástasis óseas y uso de ácido 13-cis-retinoico. Conclusión: A pesar de presentar varios indicadores de mal pronóstico (edad, metástasis óseas, estadio IV, infiltración medular), el paciente ha permanecido en remisión completa de la enfermedad durante casi 39 meses.Introdução: O neuroblastoma é um tumor extracraniano originado de falhas das células da crista neural. É a neoplasia maligna mais comum dos lactentes e apresenta perfil clínico bastante heterogêneo. O objetivo deste artigo é relatar o caso de um neuroblastoma em estádio avançado e sobrevida satisfatória, a despeito dos fatores prognósticos desfavoráveis. Relato do caso: Paciente feminina, 1 ano e 4 meses, iniciou quadro de irritabilidade, distensão abdominal e febre intermitente com três meses de evolução. Ao exame físico, apresentou alopecia sugestiva de tricotilomania, linfonodomegalia cervical, inguinal e axilar e distensão abdominal. A tomografia computadorizada de abdome evidenciou massa expansiva, medindo 6,8 x 5,8 x 4,0 cm, localizada no espaço pararrenal anterior esquerdo. À cintilografia óssea e ressonância nuclear magnética, foram visualizadas imagens sugestivas de implantes secundários a distância. Após exérese tumoral total, foi confirmado neuroblastoma pouco diferenciado, com gene MYCN não amplificado e histologia desfavorável. Biópsia da crista ilíaca bilateral revelou áreas compatíveis com infiltração medular. Por se tratar de neuroblastoma estádio IV, estabeleceu-se terapêutica multimodal, com quimioterapia adjuvante após cirurgia, seguida de transplante autólogo de medula óssea, radioterapia no sítio primário da lesão e nos locais de metástases ósseas e uso de ácido 13-cis-retinoico. Conclusão: Apesar de apresentar diversos indicadores de mau prognóstico (idade, metástases ósseas, estadiamento IV, infiltração medular), a paciente permanece em remissão completa da doença há 39 meses.INCA2022-09-16info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionRelato de Casoapplication/pdfapplication/pdftext/htmlhttps://rbc.inca.gov.br/index.php/revista/article/view/251510.32635/2176-9745.RBC.2022v68n3.2515Revista Brasileira de Cancerologia; Vol. 68 No. 3 (2022): July/Aug./Sept.; e-212515Revista Brasileira de Cancerologia; Vol. 68 Núm. 3 (2022): jul./ago./sept; e-212515Revista Brasileira de Cancerologia; v. 68 n. 3 (2022): jul./ago./set.; e-2125152176-9745reponame:Revista Brasileira de Cancerologia (Online)instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)instacron:INCAporenghttps://rbc.inca.gov.br/index.php/revista/article/view/2515/2178https://rbc.inca.gov.br/index.php/revista/article/view/2515/2752https://rbc.inca.gov.br/index.php/revista/article/view/2515/2284Copyright (c) 2022 Revista Brasileira de Cancerologiahttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessBorba, Flávia Marcella Sena GonçalvesAlmeida, Andressa deTomaz, Emanuelle ScarlathSilva, Nágella Samara Eleutério daLouzada, Cibelle Ferreira2023-03-29T13:18:30Zoai:rbc.inca.gov.br:article/2515Revistahttps://rbc.inca.gov.br/index.php/revistaPUBhttps://rbc.inca.gov.br/index.php/revista/oairbc@inca.gov.br0034-71162176-9745opendoar:2023-03-29T13:18:30Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)false
dc.title.none.fl_str_mv Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
Aspectos Clínicos y Factores Pronósticos del Neuroblastoma: Reporte de Caso
Aspectos Clínicos e Fatores Prognósticos do Neuroblastoma: Relato de Caso
title Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
spellingShingle Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
Borba, Flávia Marcella Sena Gonçalves
neuroblastoma
prognóstico
neoplasias
relatos de casos
neuroblastoma
prognosis
medical neoplasms
case reports
neuroblastoma
pronóstico
neoplasias
informes de casos
title_short Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
title_full Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
title_fullStr Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
title_full_unstemmed Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
title_sort Clinical Aspects and Prognostic Factors of Neuroblastoma: Case Report
author Borba, Flávia Marcella Sena Gonçalves
author_facet Borba, Flávia Marcella Sena Gonçalves
Almeida, Andressa de
Tomaz, Emanuelle Scarlath
Silva, Nágella Samara Eleutério da
Louzada, Cibelle Ferreira
author_role author
author2 Almeida, Andressa de
Tomaz, Emanuelle Scarlath
Silva, Nágella Samara Eleutério da
Louzada, Cibelle Ferreira
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Borba, Flávia Marcella Sena Gonçalves
Almeida, Andressa de
Tomaz, Emanuelle Scarlath
Silva, Nágella Samara Eleutério da
Louzada, Cibelle Ferreira
dc.subject.por.fl_str_mv neuroblastoma
prognóstico
neoplasias
relatos de casos
neuroblastoma
prognosis
medical neoplasms
case reports
neuroblastoma
pronóstico
neoplasias
informes de casos
topic neuroblastoma
prognóstico
neoplasias
relatos de casos
neuroblastoma
prognosis
medical neoplasms
case reports
neuroblastoma
pronóstico
neoplasias
informes de casos
description Introduction: Neuroblastoma is an extracranial tumor originated from neural crest cell failures. It’s the most common malignant neoplasm among infants and it presents a very heterogeneous clinical profile. The objective of the article is to report the case of a patient with neuroblastoma in advanced stage and satisfactory survival, despite the poor prognostic factors. Case report: Female patient, 1 year and 4 months old who presented irritability, abdominal distention and intermittent fever with three months of evolution. In physical exam, she presented alopecia suggestive of trichotillomania, cervical, inguinal and axillary lymph node enlargement and abdominal distention. Abdominal computed tomography evidenced expansive mass, measuring 6.8 x 5.8 x 4.0 cm, located in the left anterior pararenal space. Bone scintigraphy and nuclear magnetic resonance showed images suggestive of distant secondary implants. After total tumor excision, it was confirmed poorly differentiated neuroblastoma, with non-amplified MYCN gene and unfavorable histology. Bilateral iliac crest biopsy revealed bone marrow areas occupied by the neoplasm, suggesting bone marrow infiltration. As this was a stage IV neuroblastoma, multimodal therapy was established with adjuvant chemotherapy after surgery, followed by autologous bone marrow transplantation, radiotherapy at the primary site of the lesion and at the sites of bone metastases and use of 13-cis-retinoic acid. Conclusion: Despite presenting several indicators of poor prognosis (age, stage IV, bone metastases, bone marrow infiltration), the patient has been in complete remission of the disease for 39 months.
publishDate 2022
dc.date.none.fl_str_mv 2022-09-16
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
Relato de Caso
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status_str publishedVersion
dc.identifier.uri.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/2515
10.32635/2176-9745.RBC.2022v68n3.2515
url https://rbc.inca.gov.br/index.php/revista/article/view/2515
identifier_str_mv 10.32635/2176-9745.RBC.2022v68n3.2515
dc.language.iso.fl_str_mv por
eng
language por
eng
dc.relation.none.fl_str_mv https://rbc.inca.gov.br/index.php/revista/article/view/2515/2178
https://rbc.inca.gov.br/index.php/revista/article/view/2515/2752
https://rbc.inca.gov.br/index.php/revista/article/view/2515/2284
dc.rights.driver.fl_str_mv Copyright (c) 2022 Revista Brasileira de Cancerologia
https://creativecommons.org/licenses/by/4.0
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Copyright (c) 2022 Revista Brasileira de Cancerologia
https://creativecommons.org/licenses/by/4.0
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
application/pdf
text/html
dc.publisher.none.fl_str_mv INCA
publisher.none.fl_str_mv INCA
dc.source.none.fl_str_mv Revista Brasileira de Cancerologia; Vol. 68 No. 3 (2022): July/Aug./Sept.; e-212515
Revista Brasileira de Cancerologia; Vol. 68 Núm. 3 (2022): jul./ago./sept; e-212515
Revista Brasileira de Cancerologia; v. 68 n. 3 (2022): jul./ago./set.; e-212515
2176-9745
reponame:Revista Brasileira de Cancerologia (Online)
instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron:INCA
instname_str Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
instacron_str INCA
institution INCA
reponame_str Revista Brasileira de Cancerologia (Online)
collection Revista Brasileira de Cancerologia (Online)
repository.name.fl_str_mv Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)
repository.mail.fl_str_mv rbc@inca.gov.br
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