title: 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.
authors: Department of Paediatric Neurosurgery, Department of Paediatric Oncology, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
authors: O
authors: O
authors: Fayeye
authors: M
authors: M
authors: Volpon
authors: J
authors: J
authors: George
authors: D
authors: D
authors: Hobin
authors: G
authors: G
authors: Solanki
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D018202:Pulmonary Blastoma / Q000150:complications / Q000473:pathology / Q000601:surgery*; D013117:Spinal Cord Compression / Q000209:etiology / Q000601:surgery*; D013125:Spinal Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D013904:Thoracic Vertebrae / Q000473:pathology / Q000601:surgery
keywords: Pleuropulmonary blastoma (PPB); Spinal cord compression; Spinal metastases