Background & Purpose Registry Personnel What is the Registry? What the Registry provides? What the Registry Wants? Enroll Basic Facts about PPB PPB Types: I, II & III Signs and Symptoms Age at Diagnosis / Gender / Laterality Prognosis Metastasis PPB Critical Issues Constitutional / Syndrome & Familial Disease Registry Personnel Radiography Surgery Pathology PPB Types: I, II & III Progression of Type Type I Pathology Diagnosis and Differential Diagnosis Type II & III Pathology Diagnosis and Differential Diagnosis Differentiation from Adult Pulmonary Blastoma Immunohistochemistry Ultrastructural Findings Fine Needle Aspiration Cytology Pathology of Metastases Recommended Path Studies Special Studies/Tumor Cytogenetics Pathology Consultation Recommendations Staging Treatment Lung Cysts Type I Type II and III Recurrences & Metastases Stem Cell Therapy Surveillance Appendix/Data Resources Forms, Schemas, and Roadmaps Consultation Enroll Now Basics of PPB What Causes PPB? Different Types of PPB Treatment Surgery Chemotherapy Radiation Therapy Does PPB Spread? Recurrence/Relapse Family/Genetic Issues Who Should Treat PPB? What Can Families Do? Enroll Now Definitions/Glossary Genetic Study/Tissue Bank For Families For Pathologists For Pediatric Oncologists, Data Managers, and Other Physical Caregivers All Forms Pathology Review and Tissue Submission Forms Parental Authorization Chemotherapy Schemas and Roadmaps

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 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

Treatment of PPB Metastasis and Recurrences Treatment of PPB metastases must be highly individualized. Recurrent disease in the chest has been successfully treated with further surgery, additional chemotherapy and often 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. 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)

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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. Minneapolis Web Design by First Scribe.