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  • Imaging Casebook
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Imaging Case Book

Neonatal pleuropulmonary blastoma, type 1

Journal of Perinatology volume 28, pages 82–84 (2008)Cite this article

The patient was taken to the operating room where the cystic mass was found to arise from the peripheral basilar portion of the right lower lobe. This was resected without a lobectomy, leaving most of the right lower lobe in place. Histological examination demonstrated hypercellular septae in this cystic mass. There were primitive mesenchymal elements with rare nodules of mature cartilage, consistent with pleuropulmonary blastoma (PPB). Because of this diagnosis, a repeat thoracotomy for complete lower lobectomy was performed.

PPB is a rare intrathoracic dysembryonic tumor occurring primarily in children less than 5 years of age, although it has been reported in an adult of 36 years.1, 2 The tumor can arise from the lung, pleural or both.3 Histologically, the lesion is different from the adult-type pulmonary blastoma, which contains epithelial elements. PPB does not have the epithelial elements occurring in the adult-type pulmonary blastoma.4 PPB rather has a primitive blastema along with a malignant mesenchymal sarcomatous component, most often embryonal rhabdomyosarcoma or chondrosarcoma.5 Interestingly, however, PPB shares features with pulmonary blastoma and with pulmonary rhabdomyosarcoma an intriguing association with congenital lung cysts.6 The exact nature of this association is not understood. But it is widely believed that the lesion arises from congenital lung cysts. Unfortunately, the most common cystic lesions of the lung, congenital cystic adenomatoid malformation of the lung and bronchogenic cyst, can only histologically be differentiated from this are cystic lesion with a neoplastic potential. This latter lesion has been given the name cystic mesenchymal hamartoma or type 1 PPB. Three types of PPB are now recognized. Type 1 is an entirely cystic lesion; type 2 is mixed cystic and solid; and type 3 is entirely solid.7, 8 The cystic lesion, as occurred in this case, is usually located in the periphery of the lobe in which it arises or attached to the pleura, and in 70% of the cases it occurs in the right lung, particularly the right lower lobe.7, 9 It is believed that these three types are a continuum; the type 1 lesion progresses to type 2 and type 3 as the cysts are replaced by solid malignant tissue.10

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  1. Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Children's Hospital, Washington University School of Medicine, St Louis, MO, USA

    T E Herman & M J Siegel

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  1. T E Herman
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Correspondence to T E Herman.

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Herman, T., Siegel, M. Neonatal pleuropulmonary blastoma, type 1. J Perinatol 28, 82–84 (2008). https://doi.org/10.1038/sj.jp.7211863

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