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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry
The Genetics of PPB

Critical Issues


Constitutional & Familial Issues: Genetics of PPB:


A Very Rare Disease: Enrolling Patients in the Registry


Cerebral Metastases in PPB


Genetic Study of PPB


PPB Associated with Cystic Nephroma and Other Renal Tumors


Type I PPB: Treatment Issues


Type II and III THERAPY RECOMMENDATION CHANGE

Join Our PPB Study

Whats New

04/09/2010
2010 PPB FAMILY WEEKEND IN WASHINGTON DC

02/15/2007
THERAPY CHANGE: PPB Types II and III

11/15/2006
PPB, Cystic Nephroma & Small Bowel Polyps

10/01/2005
Genetic Study in PPB - PPB Tissue Bank

Recommendations
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 [Literature Sources Kusafuka et al and one Registry case].

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Data and recommendations on this site are conscientiously presented but some are unpublished observations and have not undergone peer review. Consultation with the Registry is encouraged to clarify any topics. The International PPB Registry advises caution in the citation of website information.
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