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

Type I PPB: Treatment Issues: Priest JR, Hill DA, Williams GM, Moertel CM, Messinger Y, Finkelstein MJ, Dehner LP. Type I pleuropulmonary blastoma: A report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 2006;24:4492-8. The International PPB Registry has collected a series of Type I PPB cases and analyzed outcome based on treatments of surgery with or without adjuvant chemotherapy. Recurrences in the surgery alone group were 8/20 (40%) and in the adjuvant chemotherapy group were 1/17 (6%). Recurrence-free survival is statistically greater with adjuvant chemotherapy. Overall survival is not significantly different. All recurrences were Type II or III PPB. Survival in the adjuvant chemotherapy group was approximately 90%. Survival in the surgery alone group was about 65%. There may be some cases of Type I PPB that can be safely followed by close monitoring, and the Registry is studying Type I PPB to try to identify prognostic factors that can predict who benefits from adjuvant chemotherapy. To learn more, see also For Doctors-Recommendations-Treatment- Type I PPB. Clinicians are encouraged to contact the Registry for the latest information.

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