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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

From the International Pleuropulmonary Blastoma Registry; Department of Pediatric Hematology/Oncology, Children's Hospitals and Clinics of Minnesota, St Paul; Center for Care Innovation and Research, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN; and the Department of Pathology and Immunology, Barnes-Jewish and St Louis Children’s Hospitals at Washington University, St Louis, MO

Address reprint requests to John R. Priest, MD, International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, 345 N Smith Avenue, Mailstop 70-301, St Paul, Minnesota 55102; e-mail: jprst{at}prodigy.net

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.

Supported by the Pine Tree Apple Tennis Classic.

Presented in part at the Annual Meeting of the American Society of Pediatric Hematology and Oncology, Orlando, FL, May 14, 2005.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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