Pleuropulmonary Blastoma: Outcomes of Patients Treated with Curative Intent Protocols

Pleuropulmonary blastoma (PPB) is a rare and aggressive type of intrathoracic malignancy arising from pleuropulmonary mesenchyme predominantly seen in childhood. We evaluated the clinico-patho-radiological profile, treatment response, and overall outcomes for patients with PPB treated with curative intent protocols at the Tata Memorial Hospital (TMH). All patients with histologically confirmed PPB were retrospectively analyzed. Between December 2004 to June 2023, 26 patients (15 males) with histologically proven PPB in the age group of 2 to 22 years (Median 4 years) were evaluated at TMH. Clinico-radiological and treatment details were available for 19 patients. The mean tumor size in this cohort was 11.45cm. The presence of pleural effusion and intrathoracic nodes was observed in 10 (53%) and 5 (26%) patients, respectively. All of them presented with locally extensive and type II/III disease. Curative intent treatment was offered to 17 patients and 14 of them received upfront intensive multi-agent chemotherapy. Three patients underwent upfront surgery. Four out of fourteen (29%) patients progressed on induction chemotherapy and hence, did not undergo local treatment. Post neoadjuvant chemotherapy , out of the 9 patients, local treatment in the form of surgery was offered to 7 (64%) patients followed by adjuvant radiotherapy to 6 patients (38%), whereas 2 patients received definitive radiotherapy. At the time of last follow up only 5 out of 17 (29%) patients were alive and disease free. All patients who had progressive disease on chemotherapy and did not receive local treatment died of the disease. Of the 12 patients receiving local treatment, 4 died of local relapse. The 2-year and 3-year overall survival of the entire cohort were 47% and 35% respectively. PPB is associated with dismal outcomes. Patients responding to induction chemotherapy and receiving local treatment have better survival.