title: Perilobar nephroblastomatosis: natural history and management.
abstract: Nephroblastomatosis (NB) has been considered as a precursor of Wilms tumor (WT). The natural history of NB seems to present significant variation as some lesions may regress spontaneously, while others may grow and expand or relapse and develop into WT later in childhood. Although, most investigators suggest adjutant chemotherapy, the effect and duration of treatment are not well established. Children with diffuse perilobar NB, Beckwith-Wiedemann syndrome, and hemihypertrophy seem to particularly benefit from treatment. We discuss our experience on two cases of NB and we review the literature for the management of this rare condition.
authors.affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: S
authors.initials: S
authors.lastname: Stabouli
authors.affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: N
authors.initials: N
authors.lastname: Printza
authors.affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: J
authors.initials: J
authors.lastname: Dotis
authors.affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: A
authors.initials: A
authors.lastname: Matis
authors.affiliation: Pediatric Oncology Clinic, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: D
authors.initials: D
authors.lastname: Koliouskas
authors.affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: N
authors.initials: N
authors.lastname: Gombakis
authors.affiliation: 1st Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration Hospital of Thessaloniki, 49 Kostantinoupoleos Street, 54642 Thessaloniki, Greece.
authors.forename: F
authors.initials: F
authors.lastname: Papachristou
references.citation: Clin Dysmorphol. 2001 Jan;10(1):19-23
references.pmid: 11152142
references.citation: Pediatr Dev Pathol. 2005 May-Jun;8(3):320-38
references.pmid: 16010493
references.citation: Clin Genet. 1995 May;47(5):257-62
references.pmid: 7554352
references.citation: Pediatr Radiol. 2004 Aug;34(8):660-4
references.pmid: 15103425
references.citation: Med Pediatr Oncol. 2000 Oct;35(4):429-32; discussion 433
references.pmid: 11025476
references.citation: Pediatr Pathol Lab Med. 1996 Nov-Dec;16(6):961-72
references.pmid: 9025894
references.citation: Pediatr Blood Cancer. 2006 Feb;46(2):203-21
references.pmid: 15816029
references.citation: Am J Med Genet A. 2005 Aug 1;136A(4):373-6
references.pmid: 15988748
references.citation: J Pediatr Surg. 2011 May;46(5):983-8
references.pmid: 21616266
references.citation: Pediatr Nephrol. 2005 Jul;20(7):1007-10
references.pmid: 15856325
references.citation: Pediatr Dev Pathol. 2009 May-Jun;12(3):237-8
references.pmid: 18597570
references.citation: Eur J Cancer. 2001 Feb;37(3):385-91
references.pmid: 11239761
references.citation: Pediatr Dev Pathol. 2009 Jan-Feb;12(1):47-52
references.pmid: 18275253
references.citation: JBR-BTR. 2009 May-Jun;92(3):144-5
references.pmid: 19670570
references.citation: Pediatr Radiol. 1992;22(8):598-9
references.pmid: 1337203
references.citation: Virchows Arch. 2006 Feb;448(2):214-7
references.pmid: 16177880
references.citation: Pediatr Pathol. 1990;10(1-2):1-36
references.pmid: 2156243
references.citation: Radiographics. 2010 Sep;30(5):1421-5
references.pmid: 20833859
references.citation: J Pediatr Hematol Oncol. 2009 Apr;31(4):296-9
references.pmid: 19346886
references.citation: Med Pediatr Oncol. 1999 Mar;32(3):196-200
references.pmid: 10064187
references.citation: Pediatr Pathol. 1993 Nov-Dec;13(6):741-9
references.pmid: 8108294
references.citation: Nephrol Dial Transplant. 2004 Jan;19(1):223-6
references.pmid: 14671061
references.citation: Pediatr Dev Pathol. 2005 Jan-Feb;8(1):115-23
references.pmid: 15803218
references.citation: Am J Med Genet. 1999 Oct 29;86(5):439-46
references.pmid: 10508986
references.citation: Pediatr Pathol. 1994 May-Jun;14(3):421-32
references.pmid: 8066001
references.citation: J Urol. 2004 Sep;172(3):1066-9; discussion 1069-70
references.pmid: 15311039