title: Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma.
abstract: BACKGROUND
  Pleuropulmonary blastoma (PPB) is a rare tumor of pleura and lung in young children. Central nervous system (CNS) complications, particularly cerebral parenchymal metastases, occur in aggressive forms of PPB: Types II and III PPB. This article evaluates cerebral and meningeal metastases, cerebrovascular events (CVA) caused by tumor emboli, spinal cord complications, and intracranial second malignancies in PPB.
  
  
  METHODS
  International PPB Registry and literature cases were evaluated for CNS events. Cerebral metastasis patients were evaluated for gender, side of origin of PPB, PPB Type, interval from diagnosis to metastasis, status of chest disease, treatment, and outcome. Standard statistical methods were used to calculate the cumulative probability of cerebral metastasis and survival following metastasis.
  
  
  RESULTS
  Thirty-nine cases of cerebral metastasis were identified in 5/53 Registry Type II cases, 15/44 Registry Type III cases, and 19/143 literature Type II/III cases. Metastases occurred 1-60, median 11.5 months after diagnosis. Chest disease was controlled in 50% of children at time of metastasis. The cumulative probability of cerebral metastasis by 5 years from diagnosis was 11% for Type II patients (95%CI (confidence interval): 2-20%) and 54% for Type III patients (95%CI: 31-76%). Seven children survive cerebral metastasis. Other CNS complications were post-operative CVA (five cases), spinal cord invasion or compression (six), leptomeningeal disease (three), and second intracranial malignancies (two).
  
  
  CONCLUSIONS
  Cerebral metastasis is more frequent in PPB than in other childhood sarcomas. Clinicians should screen for this complication. Diverse other CNS complications are less common and require careful diagnosis.
authors.affiliation: International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, St. Paul, Minnesota 55102, and Washington University Medical Center, Division of Anatomic Pathology, St. Louis, MO, USA. jprst@prodigy.net
authors.forename: John R
authors.initials: JR
authors.lastname: Priest
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Magnuson
authors.forename: Gretchen M
authors.initials: GM
authors.lastname: Williams
authors.forename: Minnie
authors.initials: M
authors.lastname: Abromowitch
authors.forename: Rebecca
authors.initials: R
authors.lastname: Byrd
authors.forename: Philippa
authors.initials: P
authors.lastname: Sprinz
authors.forename: Marsha
authors.initials: M
authors.lastname: Finkelstein
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Moertel
authors.forename: D Ashley
authors.initials: DA
authors.lastname: Hill
mesh_terms: D001932:Brain Neoplasms / Q000453:epidemiology / Q000473:pathology / Q000556:secondary*; D016543:Central Nervous System Neoplasms / Q000453:epidemiology / Q000473:pathology / Q000556:secondary*; D002540:Cerebral Cortex; D002561:Cerebrovascular Disorders / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000453:epidemiology / Q000473:pathology; D009360:Neoplastic Cells, Circulating; D010997:Pleural Neoplasms / Q000473:pathology*; D011379:Prognosis; D016016:Proportional Hazards Models; D018202:Pulmonary Blastoma / Q000150:complications* / Q000453:epidemiology / Q000473:pathology / Q000556:secondary*; D012042:Registries / Q000706:statistics & numerical data; D016019:Survival Analysis