High therapeutic effectiveness of postoperative irinotecan chemotherapy in a typical case of radiographically and pathologically diagnosed pleuropulmonary blastoma.

A 2-year 9-month-old girl with a large mass in the right chest underwent middle and inferior lobectomy, after which the mass was pathologically diagnosed as a pleuropulmonary blastoma (PPB). The clinical, radiographic, and pathologic findings were typical. Three courses of postoperative chemotherapy with 2 different regimens were ineffective in preventing multiple metastases of the lung. We then instituted a protocol with irinotecan (CPT-11) and vincristine. After the third course of this protocol the metastases disappeared and there were no recurrences. PPB is a rare pediatric malignant tumor, and no adequate therapy has been defined. This is the only case reported to have been treated with vincristine/irinotecan therapy for the treatment of PPB.