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: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://doi.org/10.25753/BirthGrowthMJ.v30.i1.18640
Resumo: 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 childhoodDoença de Castleman - Um diagnóstico raro na infânciaCase ReportsIntroduction: 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.Introdução: A doença de Castleman (DC) é um distúrbio linfoproliferativo policlonal raro caracterizado por crescimento anormal de tecido linfóide. Os locais mais comummente afetados são o tórax, abdómen, pescoço e axila. A biópsia excisional é mandatória para o diagnóstico e a resseção cirúrgica é o tratamento de eleição na forma unicêntrica.            Caso clínico: Uma criança de dez anos de idade, do sexo feminino, foi observada no Serviço de Urgência por odinofagia e febre. Ao exame físico, apresentava rubor amigdalino sem exsudados e adenomegalia palpável na região supraclavicular direita. A ecografia cervical confirmou linfoadenopatia com perda do centro adiposo e o exame histopatológico foi compatível com DC. Discussão/Conclusão: As adenomegalias são uma apresentação comum na infância e geralmente benignas e auto-limitadas. Contudo, poderão ser um sinal de neoplasia. Uma adenopatia na região supraclavicular é preocupante e requer investigação atempada. O diagnóstico de DC é desafiante, devido à sua raridade em idade pediátrica e sintomas inespecíficos.Unidade Local de Saúde de Santo António2021-04-06info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.25753/BirthGrowthMJ.v30.i1.18640eng2183-9417Cunha, Sara MonteiroVasconcelos, SofiaNeto, CláudiaOliva, TerezaSalgado, Miguelinfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-05-07T09:45:11Zoai:ojs.revistas.rcaap.pt:article/18640Portal AgregadorONGhttps://www.rcaap.pt/oai/openairemluisa.alvim@gmail.comopendoar:71602024-05-07T09:45:11Repositório 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
Doença de Castleman - Um diagnóstico raro na infância
title Castleman disease. A rare diagnosis in childhood
spellingShingle Castleman disease. A rare diagnosis in childhood
Cunha, Sara Monteiro
Case Reports
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 Case Reports
topic Case Reports
description 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 2021-04-06
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://doi.org/10.25753/BirthGrowthMJ.v30.i1.18640
url https://doi.org/10.25753/BirthGrowthMJ.v30.i1.18640
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 2183-9417
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv Unidade Local de Saúde de Santo António
publisher.none.fl_str_mv Unidade Local de Saúde de Santo António
dc.source.none.fl_str_mv 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
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv mluisa.alvim@gmail.com
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