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Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue

Stephan Wälti, Laurent Garel, Dorothée Dal Soglio, Françoise Rypens, Michael Messerli & Josée Dubois

abstract

BACKGROUND
The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging.

OBJECTIVE
The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors.

MATERIALS AND METHODS
We conducted a retrospective study of 135 congenital lung lesions at a university mother and child center over a period of 10 years (2005-2015). During this time, we noted four tumors (two cystic PPBs and two FLITs) and 131 malformations. We recorded the following parameters: timing of conspicuity in utero (mid-second trimester, third trimester, or not seen prenatally), presence of symptoms at birth, prenatal and perinatal radiologic findings, and either histological diagnoses by pathology or follow-up imaging in non-operated cases.

RESULTS
All lesions except the four tumors were detected during mid-second-trimester ultrasound. In none of the tumors was any pulmonary abnormality found on the mid-second-trimester sonogram, contrary to the developmental pulmonary abnormalities.

CONCLUSION
The timing of conspicuity in utero appears to be a key feature for the differentiation between malformations and tumors. Lesions that were not visible at the mid-second-trimester ultrasound should be considered as tumor. A cystic lung lesion in the context of a normal mid-second-trimester ultrasound is highly suggestive of a cystic PPB. Differentiating the types of solid congenital lung tumors based upon imaging features is not yet feasible.
   
citation Wälti S, Garel L, Soglio D D, Rypens F, Messerli M, Dubois J. Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue. Pediatr Radiol 2017; 47:1766-1775.
   
type journal paper/review (English)
date of publishing 07-09-2017
journal title Pediatr Radiol (47/13)
ISSN electronic 1432-1998
pages 1766-1775
PubMed 28884206
DOI 10.1007/s00247-017-3953-3