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The International Pleuropulmonary Blastoma Registry
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Critical Issues


Constitutional & Familial Issues: Genetics of PPB:


A Very Rare Disease: Enrolling Patients in the Registry


Cerebral Metastases in PPB - Surveillance Recommended


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

Join Our PPB Study

Whats New

04/02/2008
2008 PPB FAMILY WEEKEND--The brochures were mailed today!

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

Registry Publications
The following is a list of publications and abstracts by members of the International PPB Registry, most recent first. To obtain any of these publications, please contact Gretchen Williams at 612-813-7115 or at gretchen.williams@childrensmn.org.
  • Hill DA, Jarzembowski JA, Lennerz JK, Priest JR, Williams G, Schoettler P, Dehner LP. Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry. Am J Surg Pathol 2008; 32(2):282-295
    Number of Cases: 51

  • Hill DA, Dishop MK, McKay EM, Langston C, Priest JR, Dehner LP. Immature interstitial mesenchymal tumor (IIMT): a distinctive new entity of the infant lung [Abstract]. Annual Meeting of the Society for Pediatric Pathology 2007; Mar.
    Number of Cases: 4

  • Hill DA, Jarzembowski JA, Lennerz JK, Spence D, Schoettler P, Priest JR, Williams G, Dehner LP. Histologic features of Type I pleuropulmonary blastoma (PPB) [Abstract]. Annual Meeting of the Society for Pediatric Pathology 2007; Mar.
    Number of Cases: 41

  • Lennerz JK, Jarzembowski JA, Spence D, Schoettler P, Dehner LP, Hill DA. Diagnostic features of pleuropulmonary blastoma (PPB) are absent in congenital cystic adenomatoid malformation [Abstract]. Annual Meeting of the Society for Pediatric Pathology 2007; Mar.
    Number of Cases: 57

  • Pai S, Eng HL, Lee SY, Hsaio CC, Huang WT, Huang SC, Hill DA, Dehner LP, Priest JR. Correction: Pleuropulmonary blastoma, not rhabdomyosarcoma in a congenital lung cyst [Letter to the Editor]. Pediatr Blood Cancer 2007; Mar;48(3):370-1.
    Number of Cases: 1

  • 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 Registry. J Pediatr 2006; 149(6):850-4.e2.
    Number of Cases: 18

  • Hill DA, Williams GM, Finkelstein MJ, Serrano MF, Dehner LP, Priest JR. Significance of histologic type and anaplasia in pleuropulmonary blastoma [Abstract]. Lab Invest 2006; 86(Suppl):212-27.
    Number of Cases: 116

  • Stefanaki K, Boman F, Stassinopoulou A, Williams GM, Fournet JC, Bouron-Dal Soglio D, Chauvenet A, Hill A, Messinger Y, Priest JR. Association of pleuropulmonary blastoma with cystic nephroma and small bowel polyp: case report and data from the International Pleuropulmonary Blastoma Registry [Abstract]. Virchows Arch 2006; 448:692.
    Number of Cases: 18

  • Priest JR, Magnuson J, Williams GM, Abromowitch M, Byrd R, Sprinz P, Finkelstein M, Moertel CL, Hill DA. Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma. Pediatr Blood Cancer 2006; 49(3):266-73.
    Number of Cases: 39

  • 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.
    Number of Cases: 38

  • Serrano MF, Dehner LP, Williams GM, Priest JR, Hill DA. Histologic predictors of central nervous system metastasis in pleuropulmonary blastoma: a pilot study [Abstract]. Mod Pathol 2005; 18:303.
    Number of Cases: 35

  • Dehner LP. Beware of "degenerating" congenital pulmonary cysts [Letter to the Editor]. Pediatr Surg Int 2005; 21:123-4
    Number of Cases: 4

  • Priest JR, Messinger Y, Finkelstein MJ, Williams GM, Hill DA, Dehner LP, Moertel CL. Treatment modality vs outcome in Type I pleuropulmonary blastoma (PPB) - Results from The PPB Registry [abstract]. Pediatr Blood Cancer 2005; 44(6):544
    Number of Cases: 33

  • Hill DA. USCAP Specialty Conference, case 1: Type I Pleuropulmonary blastoma. Pediatr Dev Pathol 2005; 8:77-84
    Number of Cases: 1

  • Hill DA, Dehner LP. A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4 [Letter]. Am J Surg Pathol 2004; 28(4):554-5.
    Number of Cases: 0

  • Priest J, Williams G. Incidence, timing, and outcome of central nervous system metastasis in pleuropulmonary blastoma [Abstract]. 11th International Symposium on Pediatric Neuro-Oncology 2004; EPI30:67.
    Number of Cases: 24

  • Messinger YH, Dehner LP, Hill DA, Williams G, Priest JR. Pleuropulmonary blastoma and its association with other childhood neoplasms [abstract]. J Clin Oncol 2004; 22(14S):8542
    Number of Cases: 82

  • Dehner LP, Tazelaar HD, Manabe T. Pleuropulmonary blastoma. In Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC (eds): World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. Lyon, IARC Press 2004; 99-100.
    Number of Cases: 0

  • Hill DA, Sadeghi S, Schultz MZ, Burr JS, Dehner LP. Pleuropulmonary blastoma in an adult. Cancer 1999; 85(11):2368-74.
    Number of Cases: 1

  • McDermott MB, Ponder TB, Dehner LT. Nasal chondromesenchymal hamartoma: an upper respiratory tract analogue of the chest wall mesenchymal hamartoma. Am J Surg Pathol 1998; 22(4):425-33.
    Number of Cases: 1

  • Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP. Pleuropulmonary blastoma. A clinicopathologic study of 50 cases. Cancer 1997; 80(1):147-61.
    Number of Cases: 50

  • Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL, Friend SH, Newsham I, Amylon MD, Pappo A, Mahoney DH, Langston C, Heyn R, Kohut G, Freyer DR, Bostrom B, Richardson MS, Barredo J, Dehner LP.  Pleuropulmonary blastoma: a marker for familial disease. J Pediatr 1996; 128(2):220-4.
    Number of Cases: 12

  • McDermott MB, Dehner LP, Priest JR. Pleuropulmonary blastoma and congenital cystic malformations [Letter to the editor]. J Peds 1996; 129(5): 774-5.
    Number of Cases: 0

  • Dehner LP, Watterson J, Priest J. Pleuropulmonary blastoma. A unique intrathoracic-pulmonary neoplasm of childhood. Perspect Pediatr Pathol 1995; 18:214-26.
    Number of Cases: 38

  • Boyer MW, Moertel CL, Priest JR, Woods WG.  Use of intracavitary cisplatin for the treatment of childhood solid tumors in the chest or abdominal cavity. J Clin Oncol 1995; 13(3):631-6.
    Number of Cases: 2

  • Dehner LP. Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Semin Diagn Pathol 1994; 11(2):144-51.
    Number of Cases: 0

  • Priest J, Watterson J, Dehner L, Friend S, McIntyre J, Strong L, Hansen M, Byrd B, Feusner J, Amylon M, Bostrom B, Langston C, Pappo A.  Childhood pleuropulmonary blastoma (PPB) as a unique marker for familial childhood neoplasms. [Abstract]. Proceedings of ASCO 1994; 13:186
    Number of Cases: 0

  • Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR, Dehner LP. Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood. Cancer 1988; 62(8):1516-26.
    Number of Cases: 11

  • Manivel C, Wick M, Dehner L. Thoracopulmonary blastoma - reassessment of an embryonal neoplasm [Abstract]. Lab Invest 1987; 56:46A.
    Number of Cases: 9

  • Allan BT, Day DL, Dehner LP. Primary pulmonary rhabdomyosarcoma of the lung in children. Report of two cases presenting with spontaneous pneumothorax. Cancer 1987; 59(5):1005-11.
    Number of Cases: 2



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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.