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Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases
- Source :
- Histopathology, 78(3), 434-444. Wiley-Blackwell Publishing Ltd
- Publication Year :
- 2021
- Publisher :
- Wiley-Blackwell Publishing Ltd, 2021.
-
Abstract
- Aims: Mucinous adenocarcinoma arising in congenital pulmonary airway malformation (CPAM) is a rare complication, with little being known about its natural course. The aims of this article are to describe a series of mucinous adenocarcinomas arising from CPAMs, and present their clinicopathological features, genetics, and clinical outcome. Methods and results: Thirty-seven cases were collected within a 34-year period, and the subtype of adenocarcinoma and CPAM, tumour location, stage, growth patterns, molecular data and follow-up were recorded. The cohort comprised CPAM type 1 (n = 33) and CPAM type 2 (n = 4). Morphologically, 34 cases were mucinous adenocarcinomas (21 in situ; 13 invasive), and three were mixed mucinous and non-mucinous adenocarcinoma. Seventeen cases showed purely extracystic (intra-alveolar) adenocarcinoma, 15 were mixed intracystic and extracystic, and five showed purely intracystic proliferation. Genetically, nine of 10 cases tested positive for KRAS mutations, four with exon 2 G12V mutation and five with exon 2 G12D mutation. Residual disease on completion lobectomy was observed in two cases, and three cases recurred 7, 15 and 32 years after the original diagnosis. Two patients died of metastatic invasive mucinous adenocarcinoma. Conclusions: Most adenocarcinoma that arise in type 1 CPAMs, are purely mucinous, and are early-stage disease. Intracystic proliferation is associated with lepidic growth, an absence of invasion, and indolent behaviour, whereas extracystic proliferation may be associated with more aggressive behaviour and advanced stage. Most cases are cured by lobectomy, and recurrence/residual disease seems to be associated with limited surgery. Long-term follow-up is needed, as recurrence can occur decades later.
- Subjects :
- 0301 basic medicine
Adult
Male
Pathology
medicine.medical_specialty
Histology
Lung Neoplasms
Adolescent
Disease
medicine.disease_cause
Pathology and Forensic Medicine
Proto-Oncogene Proteins p21(ras)
03 medical and health sciences
0302 clinical medicine
Cystic Adenomatoid Malformation of Lung, Congenital
Medicine
Humans
Stage (cooking)
Child
Aged
Neoplasm Staging
Natural course
business.industry
Advanced stage
Infant, Newborn
Congenital pulmonary airway malformation
Infant
General Medicine
Middle Aged
medicine.disease
Prognosis
Adenocarcinoma, Mucinous
030104 developmental biology
030220 oncology & carcinogenesis
Child, Preschool
Mutation
Adenocarcinoma
Female
KRAS
Neoplasm Recurrence, Local
business
Complication
Subjects
Details
- Language :
- English
- ISSN :
- 13652559 and 03090167
- Volume :
- 78
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Histopathology
- Accession number :
- edsair.doi.dedup.....51e6db94d6bbdcdc6035f7d68495f0cb