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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry
Recommendations

Treatment of PPB Metastasis and Recurrences

Treatment of PPB metastases must be highly individualized.

High dose chemotherapy and autologous stem cell reconstitution has been used in several children with recurrent or metastatic PPB. Despite this relatively small number of cases, HDCT + ASCR has been successful in approximately 50% of cases, which suggests that this treatment should be considered. (See Compilation of HDCT + ASCR Cases.)

Recurrent disease in the chest has been successfully treated with further surgery, additional chemotherapy and often with focal radiation therapy.

Metastasis to the cerebral parenchyma (the most common site of hematogenous metastasis) has usually involved surgery and radiation therapy, surgery and chemotherapy, or combinations of three modalities. Cerebral metastases have been cured and the Registry recommends attempting curative therapy. Clinicians are urged to contact the Registry for details of CNS treatments and outcomes.

The treating physician must ultimately determine therapy for these and other metastatic sites.

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Data and recommendations on this site are conscientiously presented but some are unpublished observations and have not undergone peer review.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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