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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry

Welcome

This website provides information from the International Pleuropulmonary Blastoma (PPB) Registry for Families and for Physicians/Caregivers on the rare childhood chest tumor known as “pleuropulmonary blastoma” or “PPB”. This tumor has occurred for years, but because of its rarity, it has come under concerted medical scrutiny only since the mid-1980’s. With the cooperation of families and physicians all over the world, we have been collecting cases of PPB for 20+ years. Through this accumulation of information, we have begun to understand some of the biological and treatment issues of this disease and have published several articles (see Registry Publications). Of course, many others have also made important observations and published information (see Literature Sources ). We continue to collect cases and ask for help in this regard from all families and all caregivers.

Our GoalsWhat Can The PPB Registry Provide?

CONFIRMATION OF PPB DIAGNOSIS: An important Registry service is diagnostic confirmation. Chest tumors in children are unusual and not all are PPB*. We receive numerous cases which we do not believe are PPB. We strongly encourage early diagnostic review. There is no cost for review or shipping, and real-time reviews can be completed in days.

*See Types II-III Dx and Differential Dx for Registry non-PPB diagnoses.

We provide a central collection of information on PPB for all interested parties. The most well-documented conclusions about PPB are in published reports of ours and others (Registry Publications and Literature Sources ). However, as we continue to collect data, we will share it with caregivers in as up-to-date a fashion as possible. We make some recommendations regarding chemotherapy and radiation therapy, but because of the limited numbers of PPB patients, such recommendations must be considered “educated guesses”. The Registry recommends chemotherapy for all Types of PPB. Types II and III PPB in older children is an aggressive cancer, and aggressive therapy is necessary. We recommend less aggressive therapy for Type I (but we recognize that not all pediatric oncologists will agree with or follow this recommendation). We collect data on chemotherapy effectiveness and will share what we can with caregivers.

The Registry will answer reasonable inquiries from family members especially to direct them to sources of information, but we will continue to turn families back to their local treating physicians for all decision making. Pediatric cancer treatment is a long-term process, which requires dedicated local attention. We cannot and do not provide that from a long distance. We can provide data on this rare disease, but we do not believe we are any better at treating PPB than caregivers in any experienced pediatric cancer facility. Furthermore, individual treating physicians will have their own sensible ideas about what each patient and family needs, and we are no substitute for that.

Enrolling in the Registry

Both families and physicians may initiate the enrollment process. Physicians can access the protocol and forms from the Registry Information Portal. Families should email the Registry, info@ppbregistry.org for consents.

 

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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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