Pleuropulmonary blastoma: a report from the TREP (Tumori Rari in Età Pediatrica) Project.

INTRODUCTION: Pleuropulmonary blastoma (PPB) is a rare, aggressive mesenchymal tumor of childhood. The Italian Tumori Rari in Età Pediatrica (TREP) Registry was the first in Europe dedicated to prospective data collection on rare pediatric tumors. We analyzed data from an Italian series of patients with PPB, focusing on the role of the TREP Project. METHODS: We considered patients aged 0-14 with histologically confirmed diagnosis, registered in population-based cancer registries (before 2000) or the TREP Registry (2000 to 2014), and analyzed data on clinical characteristics, treatment, and outcome. Event-free survival (EFS) and overall survival (OS) were estimated. Relevant prognostic factors were identified performing a univariate analysis. RESULTS: Thirty-seven cases were included (7 type I, 13 type II, 17 type III). The average diagnosis rate rose from 1.10 to 1.73 cases/year after the TREP Project started. All patients underwent surgery, 33 received chemotherapy, and 9 had radiotherapy. The median follow-up was 8.7 years. For type I, II, and III, respectively, the 5-year OS was 85.7% (33.4-97.9), 52.7% (23.4-75.5), and 57.8% (31.1-77.3); the 5-year EFS was 85.7% (33.4-97.9), 52.7% (23.4-75.5), and 52.9% (27.6-73.0). Favorable prognostic factors for EFS were Intergroup Rhabdomyosarcoma Study (IRS) stage I (p = 0.03) and T1 tumor (p = 0.05). A total of 78.3% of patients who had chemotherapy after 2000 received a standardized treatment. CONCLUSIONS: The TREP Registry showed an excellent capacity for registering cases of PPB. Patients received homogeneous treatment after the TREP Project started. Long-term outcomes were excellent for type I and unsatisfactory for type II and III. Tumor invasiveness and IRS stage were of prognostic value.

Intrauterine smoke exposure: a new risk factor for bronchopulmonary dysplasia?

OBJECTIVE: To evaluate the role of intrauterine smoke exposure and other variables on the development of bronchopulmonary dysplasia (BPD) in infants with birth weight < 1500 g (VLBW). METHODS: This case-control study investigated 277 VLBW infants (141 cases, 136 controls) admitted at birth to neonatal intensive care unit and survived to discharge. A retrospective telephone interview provided detailed parental information supplementing clinical data. Logistic regression assessed the effects of birth weight < 1000 g, gestational age < 30 weeks (GA<30), respiratory distress syndrome (RDS), neonatal mechanical ventilation > 7 days (MV>7), patent ductus arteriosus (PDA), intrauterine smoke exposure > or = 3 months (ISE), and of parental history of asthma on BPD (oxygen dependency at 28 days with characteristic radiographic abnormalities) occurrence. RESULTS: Including all variables, only GA<30, RDS and MV>7 were significantly associated with BPD. ISE did not contribute significantly to this model (odds ratio [OR] 1.94; 95% confidence interval 0.88-4.26). Excluding iatrogenic variable MV>7, GA<30, RDS, PDA and ISE (OR 2.21; 95% confidence interval 1.03-4.76) were significantly associated with BPD. Analyzing GA as a continuous variable, the OR was 0.63 for each additional week. CONCLUSIONS: Prolonged mechanical ventilation, RDS and low gestational age were the major BPD determinants. Intrauterine smoke exposure seems to influence independently BPD development.