Type I pleuropulmonary blastoma: A report from the international pleuropulmonary blastoma registry

John R. Priest, D. Ashley Hill, Gretchen M. Williams, Christopher L. Moertel, Yoav Messinger, Marsha J. Finkelstein, Louis P. Dehner

Research output: Contribution to journalArticle

81 Scopus citations


Purpose: Type I pleuropulmonary blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. Patients and Methods: Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). Results: Thirty-eight type I PPB cases were identified: Registry (n = 30) and literature (n = 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen children had surgery and adjuvant chemotherapy; one experienced recurrence and died. All recurrences were type II or III PPB. Recurrence-free survival was higher in the surgery + chemotherapy group (P= .01); overall survival did not differ (P = .18). The improved recurrence-free survival was found only in males. Four of nine children with recurrence survived. Conclusion: Adjuvant chemotherapy appears to benefit type I PPB patients. Benefit limited to males requires broader substantiation. Salvage after types II and III recurrence is poor (four of nine; 44%). A rigorous surveillance schedule after type I PPB diagnosis might detect early recurrence and be an acceptable alternative to adjuvant chemotherapy.

Original languageEnglish (US)
Pages (from-to)4492-4498
Number of pages7
JournalJournal of Clinical Oncology
Issue number27
StatePublished - Sep 20 2006
Externally publishedYes


Cite this

Priest, J. R., Hill, D. A., Williams, G. M., Moertel, C. L., Messinger, Y., Finkelstein, M. J., & Dehner, L. P. (2006). Type I pleuropulmonary blastoma: A report from the international pleuropulmonary blastoma registry. Journal of Clinical Oncology, 24(27), 4492-4498. https://doi.org/10.1200/JCO.2005.05.3595