Case report: a rare mediastinal neuroblastoma in an adult associated with syndrome of inappropriate secretion of antidiuretic hormone (SIADH)

ABSTRACT

Neuroblastoma is an embryonic cancer arising from neural crest stem cell and almost exclusively a pediatric neoplasm. In adults, neuroblastoma is rare and presents worse prognosis compared to children. The most common presentation of this neoplasia is a painless abdominal mass. Other signs and symptoms can be related to mass effect from the primary tumor or as a result of metastatic disease, or paraneoplastic syndromes. There are no well-established treatment guidelines for adults with neuroblastoma. In general, the principle of treatment is determined by risk assessment system, using a multimodal treatment (surgery, chemotherapy, and radiotherapy) based on treatment protocols for this neoplasm in children. This case report refers to the diagnosis of neuroblastoma in mediastinum in a 52-year-old woman with locally advanced disease and not able to be removed to diagnosis, accompanied by hyponatremia as paraneoplastic syndrome, which presented a good response to the treatment established.

RESUMO

O neuroblastoma é um câncer embrionário proveniente de células-tronco da crista neural e quase exclusivamente uma neoplasia pediátrica. Em adultos, o neuroblastoma é raro e apresenta pior prognóstico em relação às crianças. A apresentação mais comum dessa neoplasia é uma massa abdominal indolor. Outros sinais e sintomas podem estar relacionados ao efeito de massa do tumor primário ou como resultado de doença metastática ou síndromes paraneoplásicas. Não há diretrizes de tratamento bem estabelecidas para adultos com neuroblastoma. Em geral, o princípio do tratamento é determinado pelo sistema de avaliação de risco, utilizando um tratamento multimodal (cirurgia, quimioterapia e radioterapia) baseado em protocolos de tratamento dessa neoplasia em crianças. Este relato de caso refere-se ao diagnóstico de neuroblastoma no mediastino em uma mulher de 52 anos com doença localmente avançada e não passível de retirada ao diagnóstico, acompanhada de hiponatremia como síndrome paraneoplásica, que apresentou boa resposta ao tratamento instituído.

Keywords:

Neuroblastoma - Mediastinal neoplasms - Adult

Descritores:

Neuroblastoma - Neoplasias do mediastino - Adulto

Publikationsverlauf

Eingereicht: 19. Januar 2023

Angenommen: 21. Juni 2023

Artikel online veröffentlicht:
31. Juli 2023

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