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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

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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.
79 Search Results :
2010 Priest JR, Jaffe A.
Increasing incidence of detection of congenital lung lesions [Reply].
Pediatr Pulmonol
45:104.
Number of Cases: 0
Focus Categories: lung cyst  Registry publication 
2010 Burge D, Wheeler R.
Increasing incidence of detection of congenital lung lesions [Letter to Ed].
Pediatr Pulmonol
45:103
Number of Cases: 0
Focus Categories: lung cyst 
2009 Corapcioglu F, Lima T, Aksu G, Inan N, Deveci M, Gurbuz Y, Topcu S.
A case report with type II pleuropulmonary blastoma: successful treatment with surgery and chemotherapy.
Turk J Pediatr
51:78-81.
Number of Cases: 1
Focus Categories: bilateral  lung cyst 
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 
2007 Nur S, Badr R, Sandoval C, Brudniki A, Yeh A.
Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation. A case report.
J Pediatr Surg
42(10):1772-5.
Number of Cases: 1
Focus Categories: ASCR - PBSCT  CNS metastasis  cyst management  lung cyst 
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 
2006 Al-Backer N, Puligandla PS, Su W, Anselmo M, Laberge JM.
Type I pleuropulmonary blastoma in a 3-year-old male with a cystic lung mass.
J Pediatr Surg
41:E13-E15.
Number of Cases: 1
Focus Categories: cyst management  lung cyst 
2006 Rubinas T, Manera R, Newman B, Picken MM.
Pneumothorax and pulmonary cyst in a 2-year-old child.
Arch Pathol Lab Med
130(4):e47-9.
Number of Cases: 1
Focus Categories: lung cyst 
2006 Miniati DN, Chintagumpala M, Langston C, Dishop MK, Olutoye OO, Nuchtern JG, Cass DL.
Prenatal presentation and outcome of children with pleuropulmonary blastoma.
J Pediatr Surg
41:66-71.
Number of Cases: 10
Focus Categories: bilateral  lung cyst  cyst management  Type I PPB 
2005 Laberge JM, Puligandla P, Flageole H.
Asymptomatic congenital lung malformations.
Semin Pediatr Surg
14:16-33.
Number of Cases: 0
Focus Categories: lung cyst  cyst management  general PPB-related topic 
2005 Kirsch S, Leuschner I, Int-Veen C, Dantonello T, Brecht IB, Schuck A, Bielack S, Klingebiel T, Koscielniak E, Treuner J.
Sixteen children with pleuropulmonary blastoma - results of the German Cooperative Soft Tissue Sarcoma Group [Abstract].
Sarcoma
;9:88
Number of Cases: 16
Focus Categories: lung cyst  CNS metastasis  bilateral  ASCR - PBSCT 
2005 Indolfi P, Casale F, Bisogno G, Cecchetto G, Ferrari A, Perilongo G, Martone A, Donofrio V, Santoro N, Mazzarino I, Riccardi R, DiMartino M, DiTullio MT.
Pleuropulmonary blastoma after cystic malformations: a better prognosis? [Abstract].
Pediatr Blood Cancer
45(4):452-3.
Number of Cases: 22
Focus Categories: lung cyst  cyst management 
2005 Piastra M, Ruggiero A, Caresta E, Chiaretti A, Pulitano S, Polidori G, Riccardi R.
Life-threatening presentation of mediastinal neoplasms: report on 7 consecutive pediatric patients.
Am J Emerg Med
23:76-82.
Number of Cases: 2
Focus Categories: lung cyst 
2005 Duan M, Wang L, Cao Y, Li Z, Yang W, Rao X, Xiao K.
Results of surgical treatment of congenital cystic lung disease.
Thorac Cardiov Surg
53:61-4.
Number of Cases: 0
Focus Categories: lung cyst  general PPB-related topic 
2005 Piastra M, Ruggiero A, Caresta E, Granone P, Chiaretti A, Polidori G, Riccardi R.
Critical presentation of pleuropulmonary blastoma.
Pediatr Surg Int
21(3):223-6.
Number of Cases: 2
Focus Categories: lung cyst 
2004 Dosios T, Stinios J, Nicolaides P, Spyrakos S, Androulakakis E, Constantopoulos A.
Pleuropulmonary blastoma in childhood. A malignant degeneration of pulmonary cysts.
Pediatr Surg Int
20:863-5.
Number of Cases: 2
Focus Categories: cyst management  lung cyst 
2004 Hasiotou M, Polyviou P, Strantzia CM, Pourtsidis A, Stinios I.
Pleuropulmonary blastoma in the area of a previously diagnosed congenital lung cyst: report of two cases.
Acta Radiol
45:289-292.
Number of Cases: 2
Focus Categories: lung cyst 
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 Pham TT, Benirschke K, Masliah E, Stocker JT, Yi ES.
Congenital pulmonary airway malformation (congenital cystic adenomatoid malformation) with multiple extrapulmonary anomalies: autopsy report of a fetus at 19 weeks of gestation.
Pediatr Dev Pathol
7(6):661-6.
Number of Cases: 1
Focus Categories: lung cyst  general PPB-related topic  cystic nephroma 
2004 Cardinalli IAC, Okamura HO, Paiva VPP, Aguiar SA, Brandalise SRB.
Pleuropulmonary blastoma in childhood [Abstract].
Intercontinental Congress of Pathology

Number of Cases: 1
Focus Categories: lung cyst 
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 Cizmar A, Kaiserova E, Kirnak J, Siman J, Plank L, Szepe P, Martanovic P, Lisy M.
Pleuropulmonary blastoma [Abstract].
8th Danube Symposium of Paediatric Surgery
287
Number of Cases: 1
Focus Categories: CNS metastasis  lung cyst 
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 Kusafuka T, Kuroda S, Inoue M, Ara T, Yoneda A, Oue T, Udatsu Y, Osugi Y, Okada A.
p53 gene mutations in pleuropulmonary blastomas.
Pediatr Hematol Oncol
19(2):117-28.
Number of Cases: 3
Focus Categories: ASCR - PBSCT  CNS metastasis  cyst management  lung cyst  molecular studies  neo-adjuvant chemotherapy 
2002 Papagiannopoulos K, Hughes S, Nicholson AG, Goldstraw P.
Cystic lung lesions in the pediatric and adult population: surgical experience at the Brompton Hospital.
Ann Thorac Surg
73(5):1594-8.
Number of Cases: 1
Focus Categories: bilateral  lung cyst 
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 Kotiloglu E, Kaya H, Kiyan G, Tuney D, Canpolat C.
A rare primary pulmonary tumor of childhood.
Turk J Pediatr
44:156-9.
Number of Cases: 1
Focus Categories: bilateral  lung cyst  macrovascular invasion/embolism  neo-adjuvant chemotherapy  SVC syndrome  type progression 
2001 Guler E, Tezer Kutluk M, Valcin B, Cila A, Kale G, Buyukpamukcu M.
Pleuropulmonary blastoma in a child presenting with pneumothorax.
Tumori
87: 340-2.
Number of Cases: 1
Focus Categories: lung cyst 
2001 Kuzucu A, Soysal O, Yakinci C, Aydin NE.
Pleuropulmonary blastoma: report of a case presenting with spontaneous pneumothorax.
Eur J Cardiothorac Surg
19(2):229-30.
Number of Cases: 1
Focus Categories: lung cyst 
2001 Roh MS, Seo JY, Huh GY, Choi PJ, Hong SH, Jeong JS.
Imprint cytologic feature of pleuropulmonary blastoma.
Korean J Cytopathol
12(1):39-43.
Number of Cases: 1
Focus Categories: lung cyst 
2001 Federici S, Domenichelli V, Tani G, Sciutti R, Burnelli R, Zanetti G, Domini R.
Pleuropulmonary blastoma in congenital cystic adenomatoid malformation: report of a case.
Eur J Pediatr Surg
11(3):196-9.
Number of Cases: 1
Focus Categories: cyst management  lung cyst 
2001 Mott BD, Canver CC, Nazeer T, Buchan A, Ilves R.
Staged resection of bilateral pleuropulmonary blastoma in a two-month old girl.
J Cardiovasc Surg
42(1):135-7.
Number of Cases: 1
Focus Categories: bilateral  cyst management  lung cyst 
2001 Leung JLY, Tsao JPY, Lai AKH, Fan TW, Chan SCH.
Pleuropulmonary blastoma in an infant presenting with tension pneumothorax.
J HK Coll Radiol
4: 219-21.
Number of Cases: 1
Focus Categories: lung cyst 
2001 Papagiannopoulos KA, Sheppard M, Bush AP, Goldstraw P.
Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective?
Ann Thorac Surg
72(2):604-5.
Number of Cases: 1
Focus Categories: bilateral  cyst management  lung cyst 
2001 Ozcan C, Celik A, Ural Z, Veral A, Kandiloglu G, Balik E.
Primary pulmonary rhabdomyosarcoma arising within cystic adenomatoid malformation: a case report and review of the literature.
J Pediatr Surg
36(7):1062-5.
Number of Cases: 1
Focus Categories: lung cyst 
2000 Wright JR Jr.
Pleuropulmonary blastoma. A case report documenting transition from type I (cystic) to type III (solid).
Cancer
88(12):2853-8.
Number of Cases: 1
Focus Categories: cyst management  lung cyst  type progression 
2000 Kukkady A, Upadhyay V, Pease PW/B, Chan YF.
Pleuropulmonary blastoma: four cases.
Pediatr Surg Int
16(8):595-8.
Number of Cases: 4
Focus Categories: cyst management  cytogenetics  lung cyst 
2000 Nicol KK, Geisinger KR.
The cytomorphology of pleuropulmonary blastoma.
Arch Pathol Lab Med
124(3):416-8.
Number of Cases: 1
Focus Categories: cyst management  fine needle aspiration  lung cyst 
2000 Picaud JC, Levrey H, Bouvier R, Chappuis JP, Louis D, Frappaz D, Claris O, Bellon G.
Bilateral cystic pleuropulmonary blastoma in early infancy.
J Pediatr
136(6):834-6
Number of Cases: 2
Focus Categories: bilateral  cyst management  familial/constitutional  lung cyst 
1999 Pinter A, Kalman A, Karsza L, Verebely T, Szemledy F.
Long-term outcome of congenital cystic adenomatoid malformation.
Pediatr Surg Int
15(5-6):332-5.
Number of Cases: 0
Focus Categories: lung cyst  general PPB-related topic 
1999 Lallier M, Bouchard S, Di Lorenzo M, Youssef S, Blanchard H, Lapierre JG, Vischoff D, Tucci M, Brochu P.
Pleuropulmonary blastoma: a rare pathology with an even rarer presentation.
J Pediatr Surg
34(7):1057-9.
Number of Cases: 1
Focus Categories: bilateral  cystic nephroma  familial/constitutional  lung cyst 
1999 Aubin P, Vischoff D, Haig M, Villeneuve E, Charest J, Lallier M, Di Lorenzo M.
Management of an infant with duffuse bulbous pulmonary lesions using high-frequency oscillatory ventilation.
Can J Anesth
46(10):970-4.
Number of Cases: 1
Focus Categories: cyst management  lung cyst 
1998 Kaslovsky RA.
Concerning CCAM in children [Letter to the Editor].
Pediatr Pulmonol
26(3):230.
Number of Cases: 1
Focus Categories: lung cyst 
1998 Yusuf U, Dufour D, Jenrette JM 3rd, Abboud MR, Laver J, Barredo JC.
Survival with combined modality therapy after intracerebral recurrence of pleuropulmonary blastoma.
Med Pediatr Oncol
30(1):63-6.
Number of Cases: 1
Focus Categories: bilateral  CNS metastasis  lung cyst 
1997 d'Agostino S, Bonoldi E, Dante S, Meli S, Cappellari F, Musi L.
Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature.
J Pediatr Surg
32(9):1381-3.
Number of Cases: 1
Focus Categories: lung cyst 
1997 Adirim TA, King R, Klein BL.
Radiological case of the month.
Arch Pediatr Adolesc Med
151(10):1053-4.
Number of Cases: 1
Focus Categories: imaging of PPB  lung cyst 
1997 Gelven PL, Hopkins MA, Green CA, Harley RA, Wilson MM.
Fine-needle aspiration cytology of pleuropulmonary blastoma: case report and review of the literature.
Diagn Cytopathol
16(4):336-40.
Number of Cases: 1
Focus Categories: bilateral  fine needle aspiration  lung cyst 
1996 Park HR, Sohn JH, Hong KW.
A case report of pleuropulmonary blastoma in childhood.
Korean J Pathol
30:351-4.
Number of Cases: 1
Focus Categories: lung cyst 
1996 Lopez-Andreu JA, Ferris-Tortajada J, Gomez J.
Pleuropulmonary blastoma and congenital cystic malformations [Letter].
J Peds
129(5):773-5.
Number of Cases: 0
Focus Categories: lung cyst  cyst management 
1996 Tagge EP, Mulvihill D, Chandler JC, Richardson M, Uflacker R, Othersen HB.
Childhood pleuropulmonary blastoma: caution against nonoperative management of congenital lung cysts.
J Pediatr Surg
31(1):187-90.
Number of Cases: 1
Focus Categories: bilateral  cyst management  lung cyst 
1993 Calabria R, Srikanth MS, Chamberlin K, Bloch J, Atkinson JB.
Management of pulmonary blastoma in children.
Am Surg
59:192-6.
Number of Cases: 5
Focus Categories: lung cyst 
1993 McDermott VGM, Mackenzie S, Hendry GMA.
Case report: primary intrathoracic rhabdomyosarcoma: a rare childhood malignancy.
Br J Radiol
66:937-41.
Number of Cases: 2
Focus Categories: CNS metastasis  lung cyst 
1993 Micallef-Eynaud PD, Goulden NT, Langdale-Brown B, Eden TB, Steers JW.
Intracerebral recurrence of primary intrathoracic rhabdomyosarcoma.
Med Pediatr Oncol
21:132-6.
Number of Cases: 2
Focus Categories: CNS metastasis  lung cyst  neo-adjuvant chemotherapy 
1992 Murphy JJ, Blair GK, Fraser GC, Ashmore PG, LeBlanc JG, Sett SS, Rogers P, Magee JF, Taylor GP, Dimmick J.
Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.
J Pediatr Surg
27(10):1364-7.
Number of Cases: 3
Focus Categories: lung cyst 
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 
1990 Drut R, Drut RM.
Blastoma pleuropulmonar.
Patologia
23:197-200.
Number of Cases: 1
Focus Categories: bilateral  familial/constitutional  lung cyst 
1990 Cairoli G, Bertana S, Giuntoli M, Battisti C.
La malformazione adenomatoide cistica del polmone: esperienza in 4 casi operati.
Ped Med Chir
12:681-6.
Number of Cases: 1
Focus Categories: lung cyst 
1989 Shimokama T, Watanabe T.
Multilocular renal cyst. Scanning and transmission electron microscopic observations.
Path Res Pract
184:255-59.
Number of Cases: 0
Focus Categories: cystic nephroma  general PPB-related topic  lung cyst 
1989 Hedlund GL, Bisset GS, Bove KE.
Malignant neoplasms arising in cystic hamartomas of the lung in childhood.
Radiology
173: 77-9.
Number of Cases: 2
Focus Categories: lung cyst 
1989 Bove KE.
Sarcoma arising in pulmonary cystic mesenchymal hamartoma.
Pediatr Pathol
9:785-92.
Number of Cases: 1
Focus Categories: lung cyst 
1988 Shariff S, Thomas JA, Shetty N, D'Cunha S.
Primary pulmonary rhabdomyosarcoma in a child, with a review of literature.
J Surg Oncol
38:261-4.
Number of Cases: 1
Focus Categories: lung cyst 
1987 Becroft DMO, Jagusch MF.
Pulmonary sarcomas arising in mesenchymal cystic hamartomas [Abstract].
Pediatr Pathol
7(4):478.
Number of Cases: 3
Focus Categories: lung cyst 
1987 Allan BT, Day DL, Dehner LP.
Primary pulmonary rhabdomyosarcoma of the lung in children. Report of two cases presenting with spontaneous pneumothorax.
Cancer
59(5):1005-11.
Number of Cases: 2
Focus Categories: lung cyst  Registry publication 
1987 Selle B, Dorffel W, Wit J.
Das pulmonale blastom im kindesalter-eine kasuistik und eine ubersicht uber die literatur
Z Kinderchir
42(6):373-7.
Number of Cases: 1
Focus Categories: bilateral  CNS metastasis  lung cyst 
1987 Graham JM, Boyle W, Troxell J, Cullity GJ, Sprague PL, Beckwith JB.
Cystic hamartomata of lung and kidney: a spectrum of developmental abnormalities
Am J Med Genetics
27:45-59
Number of Cases: 0
Focus Categories: lung cyst  cystic nephroma  familial/constitutional  general PPB-related topic 
1986 Fisher JE, Khan AR, Jewett TC, Allen J, Afshani E.
Childhood pneumoblastoma (pulmonary blastoma) and adult chondroid hamartoma of lung--a unifying concept [Abstract].
Pediatr Pathol
5:94-95.
Number of Cases: 1
Focus Categories: lung cyst  general PPB-related topic 
1986 Morales L, Julia V, Tardio E, Cardesa A, Cruz M.
Blastome pulmonaire sur kyste pulmonaire congenital
Chir Pediatr
27(1):53-6.
Number of Cases: 1
Focus Categories: lung cyst 
1986 Williams RA.
Embryonal rhabdomyosarcoma occurring in cystic adenomatoid malformation [Abstract].
Pediatr Pathol
5:118-9.
Number of Cases: 1
Focus Categories: lung cyst 
1984 Holland-Moritz RM, Heyn RM.
Pulmonary blastoma associated with cystic lesions in children.
Med Pediatr Oncol
12(2):85-8.
Number of Cases: 1
Focus Categories: cyst management  cystic nephroma  lung cyst 
1983 Ohtomo K, Araki T, Yashiro N, Iio M.
Pulmonary blastoma in children.
Radiology
147: 101-4.
Number of Cases: 2
Focus Categories: lung cyst 
1982 Weinblatt ME, Siegel SE, Isaacs H.
Pulmonary blastoma associated with cystic lung disease.
Cancer
49(4):669-71.
Number of Cases: 1
Focus Categories: lung cyst 
1981 Krous HF, Sexauer CL.
Embryonal rhabdomyosarcoma arising within a congenital bronchogenic cyst in a child.
J Pediatr Surg
16: 506-8.
Number of Cases: 1
Focus Categories: CNS metastasis  familial/constitutional  lung cyst 
1980 Weinberg AG, Currarino G, Moore GC, Votteler TP.
Mesenchymal neoplasia and congenital pulmonary cysts.
Pediatr Radiol
9:179-82.
Number of Cases: 1
Focus Categories: lung cyst 
1979 Abt AB, Demers LM, Shochat SJ.
Cystic nephroma: an ultrastructural and biochemical study.
J Urol
122:539-41.
Number of Cases: 0
Focus Categories: cystic nephroma  general PPB-related topic  lung cyst 
1979 Sumner TE, Phelps CR 2nd, Crowe JE, Poolos SP, Shaffner LD.
Pulmonary blastoma in a child.
Am J Roentgenol
133(1):147-8.
Number of Cases: 1
Focus Categories: cyst management  lung cyst 
1978 Valderrama E, Saluja G, Shende A, Lanzkowsky P, Berkman J.
Pulmonary blastoma, report of two cases in children.
Am J Surg Pathol
2:415-22.
Number of Cases: 2
Focus Categories: lung cyst 
1977 Ueda K, Gruppo R, Unger F, Martin L, Bove K.
Rhabdomyosarcoma of lung arising in congenital cystic adenomaoid malformation.
Cancer
40:383-8.
Number of Cases: 1
Focus Categories: lung cyst 
1964 Williams DI.
Nephroblastoma. Clinical picture and diagnosis [Fig 155, p252]. In: Riches E (Ed) Tumors of the kidney and ureter.
Riches E (Ed) Tumors of the kidney and ureter.Edinburgh & London, Livingstone.
235-54 [Fig 155, p252]
Number of Cases: 0
Focus Categories: cystic nephroma  general PPB-related topic  lung cyst 
1963 Stephanopoulos C, Catsaras H.
Myxosarcoma complicating a cystic hamartoma of the lung.
Thorax
18:144.
Number of Cases: 1
Focus Categories: lung cyst 

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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