Massive bilateral nephroblastomatosis in a 13-year-old girl View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1982-05

AUTHORS

E. Pichler, O. A. Jürgenssen, E. Balzar, W. F. Pinggera, A. Wolf, O. Wagner, G. Reinartz, H. Czembirek, G. Syré

ABSTRACT

A 13-year-old girl on chronic hemodialysis with renal failure thought to be due to polycystic renal disease, underwent bilateral nephrectomy as a pretransplant procedure. Microscopic examination of the grossly enlarged nodular kidneys revealed a bilateral diffuse tumor infiltration which was not Wilms tumor. Eventually the diagnosis of bilateral nephroblastomatosis was established. This is apparently unique at this age without coexistent Wilms tumor. Four months after nephrectomy metastases-exceedingly rare in nephroblastomatosis-developed. Local radiation and cytostatic therapy with Actinomycin D, Vincristine and Adriamycin were initiated. All drugs were administered 24 h before the beginning of hemodialysis; only Actinomycin D was reduced to 70% of the usual dosage. Therapeutic side effects remained within the usual limits. Renal transplantation was performed 34 months after metastases had developed, i.e. 10 months after cessation of cytostatic therapy.Massive bilateral nephroblastomatosis can also occur in older children and can easily be mistaken for polycystic renal discase. It's early clinical and microscopic recognition could enable appropriate management, which should consist of open biopsy, chemotherapy and scrupulous follow-up procedures, rather than aggressive therapy. The latter probably had to be administered in this patient, initially uremic and on chronic hemodialysis. More... »

PAGES

231-236

References to SciGraph publications

  • 1975-06. Bilateral nephroblastomatosis in PEDIATRIC RADIOLOGY
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/bf00441208

    DOI

    http://dx.doi.org/10.1007/bf00441208

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1008743092

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/6288384


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