Successful treatment of metastatic cerebral recurrence of pleuropulmonary blastoma

Pediatr Blood Cancer. 2019 May;66(5):e27628. doi: 10.1002/pbc.27628. Epub 2019 Jan 24.


Pleuropulmonary blastoma (PPB) is a rare pediatric tumor. The central nervous system (CNS) is the most common site of extrathoracic metastasis. The prognosis of PPB patients with CNS metastases is dismal: most patients die within one year after recurrence. Here, we describe two patients diagnosed with PPB who developed intracranial recurrences shortly after the end of their initial treatment and were successfully treated by gross total resection, radiotherapy, and chemotherapy. Both patients are in complete remission four and three years after recurrence. Although an optimal regimen remains to be determined, these cases demonstrate that PPB with CNS metastases is potentially curable.

Keywords: CNS relapse; metronomic therapy; pleuropulmonary blastoma; treatment.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain Neoplasms / secondary
  • Brain Neoplasms / therapy*
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Humans
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / therapy*
  • Prognosis
  • Pulmonary Blastoma / pathology
  • Pulmonary Blastoma / therapy*

Supplementary concepts

  • Pleuropulmonary blastoma