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Pleuropulmonary blastoma, a distinctive neoplasm of childhood: report of three cases

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Abstract

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.

Materials and 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.

Conclusion

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.

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Authors and Affiliations

  1. Department of Diagnostic Imaging, I.R.C.C.S. Bambino Gesù Pediatric Hospital, P.za S. Onofrio 4, 00165, Rome, Italy

    Cinzia Orazi, Paolo M. S. Schingo & Saverio Malena

  2. Department of General and Thoracic Surgery, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy

    Alessandro Inserra

  3. Department of Oncology, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy

    Luigi De Sio

  4. Department of Paediatrics – Bronchopneumology Unit, I.R.C.C.S. Bambino Gesù, Rome, Italy

    Renato Cutrera

  5. Department of Anatomy and Histology, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy

    Renata Boldrini

  6. Via Luigi Ronzoni 65, 00165, Rome, Italy

    Paolo M. S. Schingo

Authors
  1. Cinzia Orazi
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  2. Alessandro Inserra
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  3. Paolo M. S. Schingo
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  4. Luigi De Sio
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  5. Renato Cutrera
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  6. Renata Boldrini
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  7. Saverio Malena
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Corresponding author

Correspondence to Paolo M. S. Schingo.

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Orazi, C., Inserra, A., Schingo, P.M.S. et al. Pleuropulmonary blastoma, a distinctive neoplasm of childhood: report of three cases. Pediatr Radiol 37, 337–344 (2007). https://doi.org/10.1007/s00247-006-0402-0

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  • DOI: https://doi.org/10.1007/s00247-006-0402-0

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