Carcinoma bronquíolo-alveolar associado a malformação congénita das vias aéreas pulmonares em adolescente assintomático

 

Bronchioloalveolar carcinoma associated with congenital pulmonary airway malformation in an asymptomatic adolescent

 

Francisco Abecasis¹

Maria Gomes Ferreira²

Ana Oliveira³

Henrique Vaz Velho⁴

 

Resumo

A malformação congénita das vias aéreas pulmonares (CPAM) é uma entidade rara com potencial de transformação maligna. Relata-se o caso de um rapaz de 14 anos, assintomático, referenciado à consulta após detecção de imagem nodular na base do pulmão direito num radiograma de tórax. A tomografia computorizada (TC) mostrou, no pulmão direito, formação redonda de média densidade com centro cavitado.

Após um ano de seguimento, mantinha-se assintomático, sendo a imagem radiológica sobreponível. Em conjunto com a equipa de cirurgia cardiotorácica foi decidido proceder a biópsia excisional. O exame histológico revelou um carcinoma bronquíolo-alveolar mucinoso associado a CPAM tipo 1. Perante este resultado, o doente foi submetido a lobectomia inferior direita. No exame histológico do restante lobo, não se identificou tumor ou malformação residuais. Mantém-se assintomático e sem complicações passados dois anos. Tanto quanto é do conhecimento dos autores, este é o primeiro caso desta rara associação em Portugal. Discute-se a abordagem de lesões quísticas em doentes assintomáticos.

Palavras-chave:Carcinoma bronquíolo-alveolar, criança, malformação adenomatóide quística do pulmão, malformação congénita das vias aéreas pulmonare

 

Abstract

Congenital pulmonary airway malformation (CPAM) is a rare entity with potential for malignant transformation.

We describe the case of a fourteen-year-old boy evaluated for the presence of a nodular image on the right lung on the chest x-ray. Computerized Tomography (CT) showed a round lesion of medium density with cavitation on the right lung. After one year of follow-up the patient was still asymptomatic and the image was similar. With the agreement of the cardiothoracic surgeons an excisional biopsy was performed.

The histological examination revealed a mucinous bronchioloalveolar carcinoma associated with a type 1 CPAM. The patient was then submitted to right inferior lobectomy. After two years follow-up he is asymptomatic and free of complications. To the authors best knowledge this is the first case reported in Portugal of this rare association. The approach to cystic lesions in asymptomatic patients is discussed.

Key-words: Bronchioloalveolar carcinoma, children, cystic adenomatoid malformation of lung, congenital pulmonary airway malformation.

 

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¹ Interno do Internato Complementar de Pediatria, Hospital Garcia de Orta, Almada

² Assistente Hospitalar Graduada de Pediatria, Hospital Garcia de Orta, Almada

³ Assistente Hospitalar Graduada de Anatomia Patológica do Hospital Garcia de Orta, Almada

⁴ Chefe do Serviço de Cirurgia Cardiotorácica do Hospital de Santa Marta

Serviço de Pediatria (Directora: Dr.ª Ana Jorge)

Serviço de Anatomia Patológica (Director: Dr.ª Maria José Brito)

Hospital Garcia de Orta, Avenida Torrado da Silva, 2805-267 Almada, Portugal

Serviço de Cirurgia Cardiotorácica (Director: Prof. Doutor José Roquette)

Hospital de Santa Maria, Rua de Santa Maria, 50, 1169-024 Lisboa, Portugal

 

Recebido para publicação/Received for publication: 07.09.21

Aceite para publicação/accepted for publication: 07.11.02