Imaging Findings in a 3-year-old Girl with Type III Pleuropulmonary Blastoma

Abstract

Pleuropulmonary blastoma (PPB) is a rare dysembryonic intrathoracic neoplasm in children. It is a malignant tumour originating from the mesenchyme with a poor prognosis. We report on a 3-year-old girl who presented with respiratory symptoms and was diagnosed as having a type III PPB according to histological results attained by open biopsy. Imaging by CT and MRI revealed the exact size of the tumour involving the left lower lobe with displacement of the mediastinum and the diaphragm. Additional FDG-PET was important to evaluate tumour vitality and to decide the time of surgery, which was performed after 12 weeks of chemotherapy with the CWS2002P protocol. After R0 resection without complications and postoperative chemotherapy, the child continues to be in complete remission. This case underlines the importance of radical surgery of the aggressive neoplasm in combination with chemotherapy and the usefulness of multimodal imaging for the optimal planning of local therapy.