Skip to main content
SpringerLink
Log in

Medullary sponge kidneys and unilateral Wilms tumour in a child with Beckwith-Wiedemann syndrome

  • Medical Genetics
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

The occurrence of a Wilms tumour in a 4-year-old girl with bilateral medullary sponge kidney, Beckwith-Wiedemann syndrome and congenital hemihypertrophy demonstrates the close relationship between these disorders. Another six cases from the literature with congenital hemihypertrophy and with medullary sponge kidney are discussed, two of them also developed intra-abdominal neoplasm.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Abbreviations

BWS:

Beckwith-Wiedemann syndrome

CH:

congenital hemihypertrophy

MSK:

medullary sponge kidney

References

  1. Beckwith JB (1963) Extreme cytomegaly of the adrenal fetal cortex, omphalocele, hyperplasia of kidneys and pancreas and leydig cell hyperplasia: another syndrome? Proceedings of the Annual Meeting of the Western Society for Pediatric Research, Los Angeles, 11 November 1963. In: Cohen MM, Gorlin RJ, Feingold Murray, ten Bensel RW (1971) The Beckwith-Wiedemann syndrome. Am J Dis Child 122:515–519

    Google Scholar 

  2. Bronk JB, Parker BR (1987) Pyelocalyceal diverticula in the Beckwith-Widemann syndrome. Pediatr Radiol 17:80–81

    PubMed  Google Scholar 

  3. Charro Salgado AL, Lopez Macia A, Freire A, Paramo P, Fernandez-Cruz A (1977) Beckwith-Wiedemann's syndrome with medullary sponge kidneys, agonadism and persistent testosterone production. Eur Urol 3:108–110

    PubMed  Google Scholar 

  4. Cohen MM Jr (1989) A comprehensive and critical assessment of overgrowth and overgrowth syndromes. Adv Hum Genet 18:181–376

    PubMed  Google Scholar 

  5. Dinkel E, Ertel M, Peters H, Berres M, Schulte-Wissermann H (1985) Kidney size in childhood: sonographical growth charts for kidney length and volume. Pediatr Radiol 15:38–43

    PubMed  Google Scholar 

  6. Duncan PA, Sagel I, Farnsworth PB (1979) Medullary sponge kidney and partial Beckwith-Wiedemann syndrome associated with congenital asymmetry. NY State J Med 79:1222–1225

    Google Scholar 

  7. Eisenberg RL, Pfister RC (1972) Medullary sponge kidney associated with congenital hemihypertrophy (asymmetry). Am J Roentgenol 116:773–777

    Google Scholar 

  8. Engström W, Lindham S, Schofield P (1988) Wiedemann-Beckwith syndrome. Eur J Pediatr 147:450–457

    Article  PubMed  Google Scholar 

  9. Gutjahr P, Kaatsch P, Spaar HJ, Niethammer D, Göbel U, Henze G, Ludwig R, Kuhl J, Erttmann R, Harms D, Hohenfellner R (1990) Klinik, Therapie und Prognose bei 373 Kindern mit Wilms-Tumoren — Ergebnisse der bundesweiten Studie 1980–1988. Akt Urol 21:132–141

    Google Scholar 

  10. Gutjahr P, Daum R (1985) Wilms-Tumor (Nephroblastom). Dtsch Ärztebl 82:3358–3364

    Google Scholar 

  11. Gutjahr P, Schwenger M, Spaar HJ, Michaelis J, Niethammer D, Jürgens H, Kühl J, Ludwig R, Kutzner J, Schmidt D, Hohenfellner R (1990) Bedeutung der präoperativen Chemotherapie und der Radiotherapie bei 373 Kindern mit Wilms-Tumor. Dtsch Med Wochenschr 115:248–253

    PubMed  Google Scholar 

  12. Haas OA, Zoubeck A, Grümayer ER, Ferst G, Gadner H (1987) Das Wiedemann-Beckwith-Syndrom: klinische Charakteristik, konstitutionelle Chromosomenanomalien und Tumorinzidenz. Klin Padiatr 199:283–291

    PubMed  Google Scholar 

  13. Harms O, Schmidt D (1986) Classification of solid tumors in children. The Kiel pediatric tumor registry. Monogr pediatr 18:1–18

    Google Scholar 

  14. Harris RE, Fuchs EF, Kaempf MJ (1981) Medullary sponge kidney and congenital hemihypertrophy: case report and literature review. J Urol 126:676–678

    PubMed  Google Scholar 

  15. Harrison AR, Williams JP (1971) Medullary sponge kidney and congenital hemihypertrophy. Br J Urol 43:552–561

    PubMed  Google Scholar 

  16. Heikel PE, Parkkulainen KV (1966) Vesicoureteral reflux in children. A classification and results of conservative treatment. Ann Radiol 9:37–40

    PubMed  Google Scholar 

  17. Hennessy WT, Cromie WJ, Duckett JW (1981) Congenital hemihypertrophy and associated abdominal lesions. Urology 27:576–579

    Article  Google Scholar 

  18. Hoyme HE, Procopio F, Crooks W, Feingold M, Jones KL (1986) The incidence of neoplasia in children with isolated congenital hemihypertrophy. Presented at the D. W. Smith Meeting on Malformations and Morphogenesis, Burlington, Vermont, 11–14 August. In: Cohen MM Jr (1989) A comprehensive and critical assessment of overgrowth and overgrowth syndromes. Adv Hum Genet 18:181–376

  19. Irving IM (1970) The “E.M.G.” syndrome (exomphalos, macroglossia, gigantism). Prog Pediatr Surg 1:1–61

    Google Scholar 

  20. Kosseff AL, Herrmann J, Gilbert EF, Viseskul C, Lubinsky M, Opitz JM (1976) Studies of Malformation Syndromes of Man XXIX: The Wiedemann-Beckwith Syndrome. Eur J Pediatr 123:139–166

    Article  PubMed  Google Scholar 

  21. Kuiper JJ (1976) Medullary sponge kidney. In: Gardner KD Jr (ed) Cystic diseases of the kidney. Wiley, New York, pp 151–171

    Google Scholar 

  22. Mayall GF (1970) The incidence of medullary sponge kidney. Clin Radiol 21:171–174

    Article  PubMed  Google Scholar 

  23. Mulvihill DM, Mecado MG, Boineau FG (1989) Beckwith-Wiedemann syndrome and its association with type III polycystic kidney disease. Pediatr Nephrol 3:286–289

    Article  PubMed  Google Scholar 

  24. Parker DA, Skalko RG (1969) Congenital asymmetry: report of 10 cases with associated developmental abnormalities. Pediatrics 44:584–589

    PubMed  Google Scholar 

  25. Pettenati MJ, Haines JL, Higgins RR, Wappner RS, Palmer CG, Weaver DD (1986) Wiedemann-Beckwith syndrome: presentation of clinical and cytogenetic data on 22 new cases and review of the literature. Hum Genet 74:143–154

    Article  PubMed  Google Scholar 

  26. Pfister RC, Weber AL, Smith EH, Wilkinson RH, May DA (1975) Congenital asymmetry (hemihypertrophy) and abdominal disease. Radiology 116:685–691

    PubMed  Google Scholar 

  27. Saypol DC, Laudone VP (1983) Congenital hemihypertrophy with adrenal carcinoma and medullary sponge kidney. Urology 21:510–511

    Article  PubMed  Google Scholar 

  28. Sotelo-Avila C, Gonzales-Crussi F, Fowler JW (1980) Complete and incomplete forms of Beckwith-Wiedemann syndrome: their oncogenic potential. J Pediatr 96:47–50

    PubMed  Google Scholar 

  29. Sprayregen S, Strasberg Z, Neidich TP (1973) Medullary Sponge Kidney and Congenital Total Hemihypertrophy. NY State J Med 73:2768–2772

    Google Scholar 

  30. Steyn J, Logie NJ (1964) Medullary sponge kidney. Br J Urol 36:482–486

    PubMed  Google Scholar 

  31. Tomooka Y, Onitsuka H, Goya T, Hayashida Y, Kuroiwa T, Kudo S, Miyazaki S, Torisu M (1988) Congenital hemihypertrophy with adrenal adenoma and medullary sponge kidney. Br J Radiol 61:851–853

    PubMed  Google Scholar 

  32. Virdis R, Drayer JIM, Montoliu J, Levine LS, Laragh JH (1977) Hypertension and medullary sponge kidneys in an adolescent with Beckwith-Wiedemann syndrome. J Pediatr 91: 761–763

    PubMed  Google Scholar 

  33. Wiedemann H-R (1964) Complexe malformatif familial avec hernie ombilicale et macroglossie: Un “syndrome nouveau”. J Genét Hum 13:223–232

    Google Scholar 

  34. Wiedemann H-R (1983) Tumours and hemihypertrophy associated with Wiedemann-Beckwith syndrome. Eur J Pediatr 141:129

    Article  Google Scholar 

  35. Zerres K, Völpel M-C, Weiß H (1984) Cystic kidneys: Genetics, pathologic anatomy, clinical picture, and prenatal diagnosis. Hum Genet 68:104–135

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Paediatrics, University Hospital, Langenbeckstrasse 1, W-6500, Mainz 1, Germany

    R. Beetz, O. Schofer, R. Schumacher & P. Gutjahr

  2. Department of Urology, University Hospital, Langenbeckstrasse 1, W-6500, Mainz 1, Germany

    H. Riedmiller

Authors
  1. R. Beetz
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. O. Schofer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. H. Riedmiller
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R. Schumacher
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. P. Gutjahr
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Beetz, R., Schofer, O., Riedmiller, H. et al. Medullary sponge kidneys and unilateral Wilms tumour in a child with Beckwith-Wiedemann syndrome. Eur J Pediatr 150, 489–492 (1991). https://doi.org/10.1007/BF01958430

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01958430

Key words

Search

Navigation