Pleuropulmonary blastoma in an adult : An initial case report

✍ Scribed by D. Ashley Hill; Saeed Sadeghi; Michelle Z. Schultz; John S. Burr; Louis P. Dehner

• Publisher: John Wiley and Sons
• Year: 1999
• Tongue: English
• Weight: 528 KB
• Volume: 85
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/(sici)1097-0142(19990601)85:11<2368::aid-cncr11>3.0.co;2-9

✦ Synopsis

BACKGROUND.

Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood. Its primitive, sarcomatous features are analogous to those of other dysembryonic or dysontogenetic tumors, such as Wilms tumor, hepatoblastoma, neuroblastoma, and embryonal rhabdomyosarcoma. PPB typically presents in young children, most younger than 5 years, as a pulmonary and/or pleuralbased tumor with cystic, solid, or combined cystic and solid features. These neoplasms are characterized histologically by primitive mesenchymal or a mixture of primitive and sarcomatous components and generally have an unfavorable clinical outcome: death occurs within 1-2 years after diagnosis.