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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.
38 Search Results :
2010 Goel P, Panda S, Srinivas M, Kumar D, Seith A, Ahuja A, Sarkar C, Chowdhury S, Agarwala S.
Pleuropulmonary blastoma with intrabronchial extension.
Pediatr Blood Cancer
54(7):1026-1028.
Number of Cases: 1
Focus Categories: pathology discussion  imaging of PPB 
2010 Chen S, Wang S, Gao J, Zhang S.
Pleuropulmonary blastoma: a clinicopathological analysis.
Chin J Lung Cancer
13(5):550-53.
Number of Cases: 5
Focus Categories: pathology discussion 
2009 Schiavetti A, Indolfi P, Hill DA, Priest JR
Primary pulmonary rhabdomyosarcoma in childhood: clinico-biologic features in two cases with review of the literature--erratum
Pediatr Blood Cancer
52(1):146.
Number of Cases: 2
Focus Categories: pathology discussion  Registry publication 
2009 Dehner LP.
USCAP Specialty Conference, case 4: Sertoli cell tumor of the ovary.
Pediatr Dev Pathol
In Press
Number of Cases: 1
Focus Categories: familial/constitutional  pathology discussion  Registry publication 
2009 Chadli-Debbiche A, Ben Brahim E, Jouini R, Labbene N, Sayed S, Ben Ayed M, Kaabar N.
Pleuropulmonary blastoma: cystic tumour misdiagnosed as an emphysematous bulla.
RespMedCME
[in press]
Number of Cases: 1
Focus Categories: pathology discussion 
2008 Hill DA, Jarzembowski JA, 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
32(2):282-295
Number of Cases: 51
Focus Categories: Type I PPB  Registry publication  pathology discussion 
2008 Gupta K, Vankalakunti M, Das A, Marwaha RK.
An autopsy report of a rare pediatric lung tumor: pleuropulmonary blastoma.
Indian J Pathol Microbiol
51(2):225-7
Number of Cases: 1
Focus Categories: general PPB-related topic  pathology discussion 
2007 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
Mar.
Number of Cases: 57
Focus Categories: pathology discussion  Registry publication 
2007 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].
Mod Pathol
20:279-300.
Number of Cases: 4
Focus Categories: pathology discussion  Registry publication 
2007 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
Mar.
Number of Cases: 41
Focus Categories: pathology discussion  Registry publication 
2007 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
Mar;48(3):370-1.
Number of Cases: 1
Focus Categories: cyst management  lung cyst  pathology discussion  Registry publication  Type I PPB 
2007 Vargas SO, Legallo RD, Pascacio JM, Moore J, Qualman SJ.
Primary pediatric intrathoracic primitive sarcomas: critical reappraisal of the spectrum of pleuropulmonary blastoma based on review of 63 patients [Abstract].
Annual Mtg Society for Pediatr Pathol
Mar.
Number of Cases: 42
Focus Categories: pathology discussion 
2006 Vargas SO, Korpershoek E, Kozakewich HP, Krijger RR, Fletcher JA, Perez-Atayde AR.
Cytogenetic and p53 profiles in congenital cystic adenomatoid malformation: insights into its relationship with pleuropulmonary blastoma.
Pediatr Dev Pathol
9(3):190-5.
Number of Cases: 2
Focus Categories: Type I PPB  pathology discussion  molecular studies 
2006 Park II, Lee EB.
Pleuropulmonary blastoma in lung.
Korean J Thorac Cardiovasc Surg
39(9):722-24.
Number of Cases: 1
Focus Categories: imaging of PPB  pathology discussion  Type I PPB 
2006 Hill DA, Williams GM, Finkelstein MJ, Serrano MF, Dehner LP, Priest JR.
Significance of histologic type and anaplasia in pleuropulmonary blastoma [Abstract].
Lab Invest
86(Suppl):212-27.
Number of Cases: 116
Focus Categories: pathology discussion  Registry publication 
2005 Boldrini R, Devito R, Diomedi-Camassei F, Francalanci P, Inserra A, Boglino C, Donfrancesco A, Jenkner A, Callea F.
Pulmonary blastomas of childhood: histologic, immunohistochemical, ultrastructural aspects and therapeutic considerations.
Ultrastruct Pathol
29(6):493-501.
Number of Cases: 6
Focus Categories: molecular studies  pathology discussion 
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 Dehner LP.
Beware of "degenerating" congenital pulmonary cysts [Letter to the Editor].
Pediatr Surg Int
21:123-4
Number of Cases: 4
Focus Categories: cyst management  cystic nephroma  pathology discussion  Registry publication 
2005 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
18:303.
Number of Cases: 35
Focus Categories: survival of CNS metastasis  Registry publication  pathology discussion  CNS metastasis 
2004 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 :99-100.
Number of Cases: 0
Focus Categories: general PPB-related topic  pathology discussion  Registry publication 
2004 MacSweeney F, Papagiannopoulos K, Goldstraw P, Sheppard MN, Corrin B, Nicholson AG.
A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4 [Reply].
Am J Surg Pathol
28(4):555.
Number of Cases: 0
Focus Categories: cyst management  lung cyst  pathology discussion 
2004 Hill DA, Dehner LP.
A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4 [Letter].
Am J Surg Pathol
28(4):554-5.
Number of Cases: 0
Focus Categories: cyst management  lung cyst  pathology discussion  Registry publication 
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 
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 
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 
2002 Essary LR, Vargas SO, Fletcher CDM.
Primary pleuropulmonary synovial sarcoma.
Cancer
94(2):459-69.
Number of Cases: 0
Focus Categories: general PPB-related topic  pathology discussion 
2000 Vujanic GM, Jenney MEM, Adams H, Meyrick SM.
Juxtaposed cystic nephroma and Wilms’ tumor.
Pediatr Dev Pathol
3:91-4.
Number of Cases: 1
Focus Categories: pathology discussion  cystic nephroma 
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 
1996 McDermott MB, Dehner LP, Priest JR.
Pleuropulmonary blastoma and congenital cystic malformations [Letter to the editor].
J Peds
129(5): 774-5.
Number of Cases: 0
Focus Categories: cyst management  pathology discussion  Registry publication 
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 
1993 Hachitanda Y, Aoyama C, Sato JK, Shimada H.
Pleuropulmonary blastoma in childhood. A tumor of divergent differentiation.
Am J Surg Pathol
17(4):382-91.
Number of Cases: 7
Focus Categories: pathology discussion 
1991 Cohen M, Emms M, Kaschula RO.
Childhood pulmonary blastoma: a pleuropulmonary variant of the adult-type pulmonary blastoma.
Pediatr Pathol
11(5):737-49.
Number of Cases: 2
Focus Categories: CNS metastasis  pathology discussion 
1990 Domizio P, Liesner RJ, Dicks-Mireaux C, Risdon RA.
Malignant mesenchymoma associated with a congenital lung cyst in a child: case report and review of the literature.
Pediatr Pathol
10:785-97.
Number of Cases: 1
Focus Categories: lung cyst  pathology discussion 
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 
1987 Manivel C, Wick M, Dehner L.
Thoracopulmonary blastoma - reassessment of an embryonal neoplasm [Abstract].
Lab Invest
56:46A.
Number of Cases: 9
Focus Categories: pathology discussion  Registry publication 
1986 Mark E.
Mesenchymal cystic hamartoma of the lung.
N Engl J Med
315:1255-9.
Number of Cases: 1
Focus Categories: general PPB-related topic  pathology discussion 
1983 Ashworth TG.
Pulmonary blastoma - a true congenital neoplasm.
Histopathol
7:585-94.
Number of Cases: 1
Focus Categories: pathology discussion 

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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