Castleman disease. A rare diagnosis in childhood

Detalhes bibliográficos
Autor(a) principal: Cunha,Sara Monteiro
Data de Publicação: 2021
Outros Autores: Vasconcelos,Sofia, Neto,Cláudia, Oliva,Tereza, Salgado,Miguel
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542021000100052
Resumo: Abstract Introduction: Castleman Disease (CD) is a rare polyclonal lymphoproliferative disorder characterized by massive growth of lymphoid tissue. The most common sites of disease are the chest, abdomen, neck, and axilla. Excisional biopsy is mandatory for diagnosis, and complete surgical resection the gold-standard treatment in unicentric CD. Case report: A ten-year-old girl was observed at the Emergency Department with sore throat and fever. Oropharynx examination revealed inflamed tonsils, with no exudates. Enlarged lymphadenopathy was palpable in the right supraclavicular fossa. Ultrasound revealed right supraclavicular lymphadenopathy with loss of adipose hilum and histopathologic assessment established CD diagnosis. Discussion/Conclusion: Lymphadenopathy is a common presentation in children, usually benign and self-limited. But it may also be a sign of underlying malignancy. Any lymphadenopathy in the supraclavicular fossa is worrisome and requires prompt investigation. CD diagnosis may be challenging, due its rare nature in childhood and nonspecific symptoms.
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spelling Castleman disease. A rare diagnosis in childhoodCastleman diseasechildhoodlymphadenopathyAbstract Introduction: Castleman Disease (CD) is a rare polyclonal lymphoproliferative disorder characterized by massive growth of lymphoid tissue. The most common sites of disease are the chest, abdomen, neck, and axilla. Excisional biopsy is mandatory for diagnosis, and complete surgical resection the gold-standard treatment in unicentric CD. Case report: A ten-year-old girl was observed at the Emergency Department with sore throat and fever. Oropharynx examination revealed inflamed tonsils, with no exudates. Enlarged lymphadenopathy was palpable in the right supraclavicular fossa. Ultrasound revealed right supraclavicular lymphadenopathy with loss of adipose hilum and histopathologic assessment established CD diagnosis. Discussion/Conclusion: Lymphadenopathy is a common presentation in children, usually benign and self-limited. But it may also be a sign of underlying malignancy. Any lymphadenopathy in the supraclavicular fossa is worrisome and requires prompt investigation. CD diagnosis may be challenging, due its rare nature in childhood and nonspecific symptoms.Centro Hospitalar do Porto2021-03-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542021000100052Nascer e Crescer v.30 n.1 2021reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542021000100052Cunha,Sara MonteiroVasconcelos,SofiaNeto,CláudiaOliva,TerezaSalgado,Miguelinfo:eu-repo/semantics/openAccess2024-02-06T17:06:29Zoai:scielo:S0872-07542021000100052Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:19:49.358624Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Castleman disease. A rare diagnosis in childhood
title Castleman disease. A rare diagnosis in childhood
spellingShingle Castleman disease. A rare diagnosis in childhood
Cunha,Sara Monteiro
Castleman disease
childhood
lymphadenopathy
title_short Castleman disease. A rare diagnosis in childhood
title_full Castleman disease. A rare diagnosis in childhood
title_fullStr Castleman disease. A rare diagnosis in childhood
title_full_unstemmed Castleman disease. A rare diagnosis in childhood
title_sort Castleman disease. A rare diagnosis in childhood
author Cunha,Sara Monteiro
author_facet Cunha,Sara Monteiro
Vasconcelos,Sofia
Neto,Cláudia
Oliva,Tereza
Salgado,Miguel
author_role author
author2 Vasconcelos,Sofia
Neto,Cláudia
Oliva,Tereza
Salgado,Miguel
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Cunha,Sara Monteiro
Vasconcelos,Sofia
Neto,Cláudia
Oliva,Tereza
Salgado,Miguel
dc.subject.por.fl_str_mv Castleman disease
childhood
lymphadenopathy
topic Castleman disease
childhood
lymphadenopathy
description Abstract Introduction: Castleman Disease (CD) is a rare polyclonal lymphoproliferative disorder characterized by massive growth of lymphoid tissue. The most common sites of disease are the chest, abdomen, neck, and axilla. Excisional biopsy is mandatory for diagnosis, and complete surgical resection the gold-standard treatment in unicentric CD. Case report: A ten-year-old girl was observed at the Emergency Department with sore throat and fever. Oropharynx examination revealed inflamed tonsils, with no exudates. Enlarged lymphadenopathy was palpable in the right supraclavicular fossa. Ultrasound revealed right supraclavicular lymphadenopathy with loss of adipose hilum and histopathologic assessment established CD diagnosis. Discussion/Conclusion: Lymphadenopathy is a common presentation in children, usually benign and self-limited. But it may also be a sign of underlying malignancy. Any lymphadenopathy in the supraclavicular fossa is worrisome and requires prompt investigation. CD diagnosis may be challenging, due its rare nature in childhood and nonspecific symptoms.
publishDate 2021
dc.date.none.fl_str_mv 2021-03-01
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
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status_str publishedVersion
dc.identifier.uri.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542021000100052
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dc.language.iso.fl_str_mv eng
language eng
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dc.publisher.none.fl_str_mv Centro Hospitalar do Porto
publisher.none.fl_str_mv Centro Hospitalar do Porto
dc.source.none.fl_str_mv Nascer e Crescer v.30 n.1 2021
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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