Staging

Based on the common manifestations and metastatic patterns of PPB, the Registry recommends the following pre- and/or post-operative staging studies:

Type I PPB

  • Chest and abdominal CT (with attention to the possibility of ipsilateral and/or bilateral cystic disease) Abdominal CT is recommended because 20 examples of PPB associated with cystic nephroma have been observed. Most are Type I PPB Registry Publications Boman 2005

Types II and III PPB

  • Chest and abdomen CT, with and without IV contrast (with attention to the possibility of additional lung or pleural disease or the involvement of the diaphragm or liver)

  • Head MRI

  • Bone scan

Bone marrow aspiration or biopsy, although commonly used in the work-up of pediatric neoplasms, is probably not necessary. In an extensive review of literature and Registry cases, in which marrow aspiration and trephine exams were often performed, we are aware of only two cases of marrow metastasis [Kusafuka T et al. p53 gene mutations in pleuropulmonary blastomas. Pediatr Hematol Oncol 2002;19(2):117-28, and one Registry case].