A case report of an unusual non-mucinous papillary variant of CPAM type 1 with KRAS mutations.
BMC Pulm Med
; 20(1): 52, 2020 Feb 24.
Article
em En
| MEDLINE
| ID: mdl-32093717
ABSTRACT
BACKGROUND:
congenital pulmonary airway malformation (CPAM) is the most frequent congenital lung disorder. CPAM type 1 is the most common subtype, typically having a cystic radiological and histological appearance. Mucinous clusters in CPAM type 1 have been identified as premalignant precursors for mucinous adenocarcinoma. These mucinous adenocarcinomas and the mucinous clusters in CPAM commonly harbor a specific KRAS mutation. CASE PRESENTATION we present a case of a 6-weeks-old girl with CPAM type 1 where evaluation after lobectomy revealed a highly unusual complex non-mucinous papillary architecture in all cystic parts, in which both mucinous clusters and non-mucinous papillary areas harbored the known KRAS mutation.CONCLUSIONS:
we found that a KRAS mutation thought to be premalignant in mucinous clusters only, was also present in the other cyst lining epithelial cells of this unusual non-mucinous papillary variant of CPAM type 1, warranting clinical follow-up because of uncertain malignant potential.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Malformação Adenomatoide Cística Congênita do Pulmão
/
Proteínas Proto-Oncogênicas p21(ras)
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Mutação
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Female
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Humans
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Infant
Idioma:
En
Revista:
BMC Pulm Med
Ano de publicação:
2020
Tipo de documento:
Article
País de afiliação:
Holanda