Pleuropulmonary blastoma, a distinctive neoplasm of childhood: report of three cases.
BACKGROUND
Pleuropulmonary blastoma (PPB) is a rare, aggressive dysontogenetic neoplasm affecting children. It was identified as a distinct entity by Manivel in 1988 and later subdivided into three types on the basis of the histological pattern, with increasing malignancy from type I (cystic) through type II (solid/cystic) to type III (solid).


OBJECTIVE
To report on the imaging findings, clinical presentation, and differential diagnosis, mainly cystic malformations.


METHODS
We evaluated three children, age 2-4 years, with PPB.


RESULTS
One patient presented with unresolving pneumothorax and a multicystic mass, another with a mixed fluid/solid lesion, and the last with a solid heterogeneous mass.


CONCLUSIONS
Despite its rarity, PPB should be considered in the evaluation of cystic or solid masses in children with respiratory distress. Plain film radiography alone is unable to distinguish between PPB and cystic malformations. CT represents the gold standard, although MRI can show the imaging features of solid enhancing nodules inside fluid-filled cavities, a mass causing lung compression, mediastinal shift, frequent pleural effusion, and no chest wall invasion. No preoperative imaging can reliably differentiate between congenital cystic lesions and PPB type I.
Orazi
Cinzia
C
Department of Diagnostic Imaging, I.R.C.C.S. Bambino Gesù Pediatric Hospital, P.za S. Onofrio 4, 00165, Rome, Italy.
Inserra
Alessandro
A
Schingo
Paolo M S
PM
De Sio
Luigi
L
Cutrera
Renato
R
Boldrini
Renata
R
Malena
Saverio
S
D002675:Child, Preschool; D003937:Diagnosis, Differential; D018450:Disease Progression; D004417:Dyspnea / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D010996:Pleural Effusion / Q000175:diagnosis; D010997:Pleural Neoplasms / Q000175:diagnosis*; D011030:Pneumothorax / Q000175:diagnosis; D001261:Pulmonary Atelectasis / Q000175:diagnosis; D018202:Pulmonary Blastoma / Q000175:diagnosis*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed