Pediatric Lung tumors
2023-04-17 Paola Dal Cin, PhD , Rita Alaggio, MD Affiliation1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
2.IRCCS Ospedale Bambino Gesú , Roma (Italy)
Classification
Definition
Lung malignancies common in adults are exceptionally rare or non-existing in children. The most common histotype include pleuropulmonary blastoma. 1 Bronchial carcinoids may occur. Among the mesenchymal neoplasms, specific pediatric entities are represented by congenital peribronchial myofibroblastic tumor (CPMT) and fetal lung interstitial tumor FLIT. EBV related smooth muscle tumors may arise in the context of primary or acquired immunodeficiencies.
| Pediatric lung tumors | |
|---|---|
| Fetal lung interstitial tumor (FLIT) | Few cases reported, benign iproliferation of spindle cells in lung interstitium. A single case with t(2;12)(p23;p13) associated with A2M:: ALK fusion. 2 |
| Congenital peribronchial myofibroblastic tumor (CPMT) | Morphologically, congenital peribronchial myofibroblastic tumor (CPMT) is reminiscent of congenital fibrosarcoma, with ETV6:: NTRK3 fusion detected in some cases. 3,4 |
| Pleuropulmonary blastoma (PPB) | Pleuropulmonary blastoma (PPB ) is a cystic/solid embryonal neoplasm show loss of function of DICER1 related to germline mutation and second somatic hit in the majority of cases. 1 In a subset a biallelic somatic mutation is identified in absence of germline alterations. 5 Trisomy 8 is the most frequent recurrent aberration, follow by loss 17p 6 |
Article Bibliography
| Reference Number | Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|---|
| 1 | 31527943 | 2019 | Pleuropulmonary Blastoma: More Than a Lung Neoplasm of Childhood. | Dehner LP et al |
| 2 | 24965693 | 2014 | Identification of novel ALK rearrangement A2M-ALK in a neonate with fetal lung interstitial tumor. | Onoda T et al |
| 3 | 21843073 | 2011 | Unusual presentation of congenital infantile fibrosarcoma in seven infants with molecular-genetic analysis. | Steelman C et al |
| 4 | 24117734 | 2014 | Congenital peribronchial myofibroblastic tumour with features of maturation in the older infant: report of two cases with a literature review. | Chang C et al |
| 5 | 26925222 | 2015 | Temporal order of RNase IIIb and loss-of-function mutations during development determines phenotype in pleuropulmonary blastoma / DICER1 syndrome: a unique variant of the two-hit tumor suppression model. | Brenneman M et al |
| 6 | 28382840 | 2017 | Strong Expression and Amplification of IGF1R in Pleuropulmonary Blastomas. | Vokuhl C et al |
Citation
Paola Dal Cin, PhD ; Rita Alaggio, MD
Pediatric Lung tumors
Atlas Genet Cytogenet Oncol Haematol. 2023-04-17
Online version: http://atlasgeneticsoncology.org/solid-tumor/209112/pediatric-lung-tumors
