PPB Associated with Cystic Nephroma and Other Renal Tumors

"Boman F, Hill DA, Williams GM, Chauvenet A, Fournet JC, BouronDalSoglio D, Messinger Y, Priest JR. Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the International Pleuropulmonary Blastoma/DICER1 Registry. J Pediatr 2006; 149:850-854. The International PPB Registry has identified 18 patients with PPB associated with 20 renal tumors (15 CN) either in themselves or family members. Clinicians are encouraged to contact the Registry for the latest information. A particular subset of PPB/CN patients also has small bowel polyps, usually with intussuception. Six such children have been identified. All ""syndromic"" PPB patients are encouraged to be enrolled in the PPB Genetic Study.