Abstract
We report the uncommon clinical course of a female with right-sided hemi-hyperplasia.
At the age of 2 years and 2 months, a small spherical lesion of the right kidney was
detected by ultrasound and magnetic resonance tomography. When the patient was 4 years
and 7 months, the very slowly growing tumor was removed completely and diagnosed as
intermediate risk stage I nephroblastoma. The case demonstrates that even small renal
lesions require diagnostic work-up and adequate treatment.
Zusammenfassung
Wir berichten den ungewöhnlichen Verlauf einer Patientin mit rechtsseitiger Hemihypertrophie.
Im Alter von 2 Jahren und 2 Monaten wurde erstmals eine sphärische Läsion der rechten
Niere in Ultraschall und Kernspintomografie nachgewiesen. Im Alter von 4 Jahren und
7 Monaten erfolgte die vollständige nierenerhaltende Resektion der gering progredienten
Raumforderung. Histologisch wurde ein Nephroblastom von intermediärer Histologie nachgewiesen.
Der Fall zeigt, dass auch kleine renale Läsionen eine komplette Diagnostik und adäquate
Behandlung erfordern.
Key words
Wilms' tumor - nephroblastoma - nephrogenic rest - hemi-hyperplasia
Schlüsselwörter
Wilms-Tumor - Nephroblastom - nephrogener Rest - Hemihypertrophie
References
- 1
Choyke PL, Siegel MJ, Craft AW. et al .
Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic
hemihypertrophy.
Med Pediatr Oncol.
1999;
32
196-200
- 2
Cooper WN, Luharia A, Evans GA. et al .
Molecular subtypes and phenotypic expression of Beckwith-Wiedemann syndrome.
Eur J Hum Genet.
2005;
13
1025-1032
- 3
Craft AW, Parker L, Stiller C. et al .
Screening for Wilms’ tumor in patients with aniridia, Beckwith syndrome, or hemihypertrophy.
Med Pediatr Oncol.
1995;
24
231-234
- 4
Graf N, Hoppe A, Georgiadi E. et al .
‘In silico’ oncology for clinical decision making in the context of nephroblastoma.
Klin Padiatr.
2009;
221
141-149
- 5
Graf N, Tournade MF, de Kraker J.
The role of preoperative chemotherapy in the management of Wilms’ tumor. The SIOP
studies. International Society of Pediatric Oncology.
Urol Clin North Am.
2000;
27
443-454
- 6
Green DM, Breslow NE, Beckwith JB. et al .
Screening of children with hemihypertrophy, aniridia, and Beckwith-Wiedemann syndrome
in patients with Wilms tumor: a report from the National Wilms Tumor Study.
Med Pediatr Oncol.
1993;
21
188-192
- 7
Gylys-Morin V, Hoffer FA, Kozakewich H. et al .
Wilms tumor and nephroblastomatosis: imaging characteristics at gadolinium-enhanced
MR imaging.
Radiology.
1993;
188
517-521
- 8
Kaste SC, Dome JS, Babyn PS. et al .
Wilms tumor: prognostic factors, staging, therapy and late effects.
Pediatr Radiol.
2008;
38
2-17
- 9
Owens CM, Brisse HJ, Olsen OE. et al .
Bilateral disease and new trends in Wilms tumor.
Pediatr Radiol.
2008;
38
30-39
- 10
Perlman EJ, Faria P, Soares A. et al .
Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients.
Pediatr Blood Cancer.
2006;
46
203-221
- 11
Tan TY, Amor DJ.
Tumor surveillance in Beckwith-Wiedemann syndrome and hemihyperplasia: a critical
review of the evidence and suggested guidelines for local practice.
J Paediatr Child Health.
2006;
42
486-490
- 12
Zils K, Furtwangler R, Reinhard H. et al .
Consultation within the nephroblastoma trial SIOP 2001/GPOH as part of the workload
in the trial office.
Klin Padiatr.
2008;
220
183-188
- 13
Zoubek A, Slavc I, Mann G. et al .
Natural course of a Wilms’ tumor.
Lancet.
1999;
354
344
Correspondence
PD. Dr. Thorsten Simon
Universitätsklinikum Köln
Klinik für Kinderheilkunde:
Hämatologie/Onkologie
Kerpener Straße 62
50924 Köln
Germany
Telefon: +49/221/478 4380
Fax: +49/221/478 6801
eMail: thorsten.simon@uk-koeln.de