Clinical analysis of pleuropulmonary blastoma in four larger Chinese pediatric hematology and oncology center

Backgroud: Pleuropulmonary blastoma (PPB) is a very rare malignant tumor of the pleuropulmonary mesenchyme in childhood. This report retrospectively analyzed PPB in four larger Chinese pediatric oncology centers in recent years. And provide a basis for multicenter collaborative treatment of PPB in China. Methods: This analysis concerns patients aged 0-18 years with confirmed to have PPB by pathology review, registered from 1999 to 2011 in Beijing Children’s Hospital, Sun Yat-Sen University Cancer Center, The Children’s Hospital of Chongqing Medical University and Shanghai children’s medical center. Lesions were classified as type I, type II or type III according to Dehner’s classification. Results: The series included 38 patients (2 type I, 15 type II and 21 type III), 14 males, 24 females with a median age of 37.5 months (19~204 months), a median of 28 days (2-180 days) from onset to diagnosis. Most Tumors were large (97%>5 cm) and invaded the parietal pleura and pericardium. In seven patients developed with distant metastasis. 9/38 (23.7%) of the patients had lost to follow up at the time of initial diagnosis because they gave up further treatment or got treatment by a local hospital. The remaining 29/38 (76.3%) patients had follow-up information. 2 patients died of respiratory failure after biopsy. 27 patients had treated by chemotherapy/surgery or both among them, 13 patients had total resection, 6 had partial resection. And 7 patients received no further treatment after surgery, but 5 patients relapsed. Total 22 patients had chemotherapy, 1 patient had chemotherapy only. The median follow-up was 43.0 months, 6 patients had recurrences and died, For the 6 recurrent cases, 5 cases had surgery only at initial treatment, and 1 case had chemotherapy only at initial treatment. 10-year overall survival rate was 71.1±8.7%. The relations between outcome and treatment were not significantly associated (P=0.196), but treatment with combined chemotherapy and surgery may suggest better prognosis (P=0.06). Conclusions: Our study showed that achieving total resection of the tumor and combined chemotherapy at any time of treatment may resulted in a better prognosis. The patient’s characteristics and pathologic features were different from the worldwide data, for the higher prevalence of type II and type III, also the higher prevalence of stage III and IV in our patients. Presumably the problem was diagnosed late for PPB in China.