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The International Pleuropulmonary Blastoma Registry
The International Pleuropulmonary Blastoma Registry
The Genetics of PPB

Critical Issues


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

Join Our PPB Study

Whats New

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

Literature Sources
Please use the search field or click on a focus category to locate a specific literature source.
22 Search Results :
2009 Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A.
Pulmonary cysts in early childhood and the risk of malignancy.
Pediatr Pulmonol
44(1):14-30.
Number of Cases: 0
Focus Categories: Type I PPB  Registry publication  major review  lung cyst  imaging of PPB  familial/constitutional  cyst management 
2009 Hill DA, Ivanovich J, Priest JR, Gurnett CA, Dehner LP, Desruisseau D, Jarzembowski JA, Wikenheiser-Brokamp KA, Suarez BK, Whelan AJ, Williams G, Bracamontes D, Messinger Y, Goodfellow PJ.
DICER1 Mutations in Familial Pleuropulmonary Blastoma.
Science
325:965
Number of Cases: 11
Focus Categories: Registry publication  molecular studies  major review  familial/constitutional  DICER1 mutation 
2007 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
49(3):266-73.
Number of Cases: 39
Focus Categories: Registry publication  major review  CNS metastasis 
2007 Indolfi P, Bisogno G, Casale F, Cecchetto G, De Salvo G, Ferrari A, Donfrancesco A, Donofrio V, Martone A, Di Martino M, Di Tullio MT.
Prognostic factors in pleuro-pulmonary blastoma.
Pediatr Blood Cancer
48(3):318-23.
Number of Cases: 22
Focus Categories: major review 
2006 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
149(6):850-4.e2.
Number of Cases: 18
Focus Categories: Registry publication  major review  familial/constitutional  cystic nephroma 
2006 Yuksel S, Ekim M, Fitoz S, Soygur T, Sertcelik A, Percinel S, Acar B, Ozcakar ZB, Comba A, Yalcinkaya F.
The association of cystic nephroma with pulmonary sequestration: is it a coincidence or not?
Pediatr Nephrol
21:1041-44.
Number of Cases: 0
Focus Categories: cystic nephroma  general PPB-related topic  major review 
2006 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
24:4492-8.
Number of Cases: 38
Focus Categories: Type I PPB  Registry publication  major review  cyst management 
2005 Hill DA.
USCAP Specialty Conference, case 1: Type I Pleuropulmonary blastoma.
Pediatr Dev Pathol
8:77-84
Number of Cases: 1
Focus Categories: cystic nephroma  major review  pathology discussion  Registry publication 
2005 Li H, Sun GQ, Zeng Q, He LJ.
CT features of pleuropulmonary blastoma in children.
Chin J Radiol
39(5):513-6.
Number of Cases: 10
Focus Categories: imaging of PPB  major review 
2003 Langston C.
New concepts in the pathology of congenital lung malformations.
Semin Pediatr Surg
12(1):17-37.
Number of Cases: 0
Focus Categories: major review  pathology discussion 
2003 MacSweeney F, Papagiannopoulos K, Goldstraw P, Sheppard MN, Corrin B, Nicholson AG.
An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation.
Am J Surg Pathol
27(8):1139-46.
Number of Cases: 1
Focus Categories: pathology discussion  major review  lung cyst  cyst management 
2002 Stocker JT.
Congenital pulmonary airway malformation - a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung.
Histopathology
41(S2):424-31.
Number of Cases: 0
Focus Categories: pathology discussion  major review  lung cyst  general PPB-related topic 
2001 Markman M.
Intraperitoneal chemotherapy in the management of malignant disease.
Expert Rev Anticancer Ther
1(1): 142-8.
Number of Cases: 0
Focus Categories: general PPB-related topic  major review 
2000 Drebov R, Brankov O, Mikhaylova V, Kamenova M, Christozova I.
Pleuropulmonary blastoma in children: diagnosis, surgical and complex treatment.
Khirurgiia
56:12-5.
Number of Cases: 10
Focus Categories: major review 
2000 Indolfi P, Casale F, Carli M, Bisogno G, Ninfo V, Cecchetto G, Bagnulo S, Santoro N, Giuliano M, Di Tullio MT.
Pleuropulmonary blastoma: management and prognosis of 11 cases.
Cancer
89(6):1396-1401.
Number of Cases: 11
Focus Categories: CNS metastasis  major review  neo-adjuvant chemotherapy 
1998 McDermott MB, Ponder TB, Dehner LP.
Nasal chondromesenchymal hamartoma: an upper respiratory tract analogue of the chest wall mesenchymal hamartoma.
Am J Surg Pathol
22(4):425-33.
Number of Cases: 1
Focus Categories: Registry publication  pathology discussion  major review  general PPB-related topic 
1997 Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP.
Pleuropulmonary blastoma: a clinicopathologic study of 50 cases.
Cancer
80(1):147-61.
Number of Cases: 50
Focus Categories: Registry publication  major review  cyst management  bilateral 
1996 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
128(2):220-4.
Number of Cases: 12
Focus Categories: Registry publication  major review  familial/constitutional  cytogenetics  cystic nephroma 
1995 Dehner LP, Watterson J, Priest J.
Pleuropulmonary blastoma. A unique intrathoracic-pulmonary neoplasm of childhood.
Perspect Pediatr Pathol
18:214-26.
Number of Cases: 38
Focus Categories: major review  pathology discussion  Registry publication 
1994 Dehner LP.
Pleuropulmonary blastoma is THE pulmonary blastoma of childhood.
Semin Diagn Pathol
11(2):144-51.
Number of Cases: 0
Focus Categories: major review  pathology discussion  Registry publication 
1994 Lopez-Andreu JA, Ferris J, Esquembre C, Verdeguer A, Esteban MJ, Paradis A, Costa E, Castel V.
Blastoma pulmonar pediatrico. Revision de la literatura y aportacion de un caso.
Oncologia
17(49):65-74.
Number of Cases: 1
Focus Categories: bilateral  familial/constitutional  major review 
1988 Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR, Dehner LP.
Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood.
Cancer
62(8):1516-26.
Number of Cases: 11
Focus Categories: major review  pathology discussion  Registry publication 

Keyword Search:



Focus Categories:

adult
ASCR - PBSCT
bilateral
CNS metastasis
cyst management
cystic nephroma
cytogenetics
DICER1 mutation
familial/constitutional
fine needle aspiration
general PPB-related topic
imaging of PPB
lung cyst
macrovascular invasion/embolism
major review
molecular studies
neo-adjuvant chemotherapy
pathology discussion
Registry publication
survival of CNS metastasis
SVC syndrome
Type I PPB
type progression


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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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