An unusual manifestation of a rare pleuropulmonary blastoma presenting with spinal cord compression and its neurosurgical implications

Abstract

Pleuropulmonary blastomas (PPB) are rare and biologically aggressive paediatric tumours. Although central nervous system metastatic dissemination is a recognised complication of PPB, to our knowledge, spinal cord compression has been described only in six patients. We report a 5-year-old boy with a diagnosis of recurrent type III PPB that was initially thought to be an empyema, who developed features of thoracic spinal cord compression secondary to local tumour infiltration. Although PPB demonstrate significant biologically aggressive behaviour, aggressive surgical resections together with adjuvant chemotherapy can help limit disease progression without impacting on the quality of life. Spinal metastatic disease should also be treated vigorously. In this paper we discuss the treatment strategies available in the management of PPB.