Pleuropulmonary Blastoma - A Single Centre Experience

Abstract

Background: Pleuropulmonary blastoma is a primary mesenchymal origin tumor of childhood with its origin in the thorax. It is considered a highly aggressive tumor because of its late diagnosis, potential for metastasis and recurrence and resistance to treatment. With very few series reported in literature and no standardized protocol, this tumor has become hard to treat. Materials and Methods: This series aims to study the cases that presented at our institute in terms of clinical presentation, work up, management and identify factors that can be associated with prognosis. It is a retrospective case series from a single centre over a period of 5 years. Results: Type II and III tumors were encountered in all the 4 children, who were all less than 3 years of age. Three children underwent neoadjuvant chemotherapy followed by surgical resection, two of whom also completed adjuvant chemotherapy and are on close follow up. The other child relapsed during follow up and the fourth child died during neoadjuvant chemotherapy. Conclusion: The prognosis of this rare tumor has direct association with degree of resection and completion of adjuvant chemotherapy. However, this could be statistically significant only if we could extrapolate it to a study with larger sample size.