Definition
Pleuropulmonary blastoma (PPB) is a rare embryonal tumor occurring in young children. The majority of children affected are less than 6 years old, although cases in older children and a 36 year old have been reported. Hereditary predisposition to PPB is approximately 25% with patients or their family members reported to have conditions such as lung cysts, cystic nephroma, nasal chondromesenchymal hamartoma, intestinal polyps, Sertoli-Leydig cell ovarian tumor, Wilms’ tumor, and rhabdomyosarcoma.
Three types of PPB have been described, type I, II, and III, and may represent the stages of disease progression over time. The characteristic features of type I PPB are air-filled cysts with the epithelium and underlying mesenchymal cells appearing benign. In type II, the mesenchymal cells overgrow, forming cystic and solid areas. Type III PPB shows solid areas only.
The median times of presentation in types I, II, and III PPB is 9, 36, and 42 months, respectively, and survival...
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References and Further Reading
Hill, D., Jarzembowski, J., Priest, J., et al. (2008). Type I pleuropulmonary blastoma: Pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry. American Journal of Surgical Pathology, 32, 282–285.
Manivel, C., Priest, J., Watterson, J., et al. (1988). Pleuropulmonary blastoma the so-called pulmonary blastoma of childhood. Cancer, 62, 1516–1526.
Priest, J., McDermott, M., Bhatia, S., et al. (1997). Plauropulmonary blastoma a clinicopathologic study of 50 cases. Cancer, 80, 147–161.
Stocker, J. T. (2011). Respiratory tract. In J. T. Stocker & L. P. Dehner (Eds.), Pediatric pathology (p. 497). Philadelphia: Lippincott Williams and Wilkins.
Venkatramani, R., Malagolowkin, M., Wang, L., et al. (2012). Pleuropulmonary blastoma: A single institution experience. Journal of Pediatric Hematology/Oncology, 34, 182–185.
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Cruz, J.F. (2018). Pleuropulmonary Blastoma. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_374
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DOI: https://doi.org/10.1007/978-3-319-66796-6_374
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