Primary osteosarcoma of the kidney is an extremely rare phenomenon wit
h less than 20 previously reported cases in the English literature sin
ce 1936. Diagnosis usually is made in advanced stages of disease with
weight loss, palpable tumor, flank pain and gross hematuria being the
characteristic features of clinical presentation. Radiographically biz
arre renal calcifications may be suggestive of this uncommon neoplasm.
The atypic location is explained by metaplastic changes of originally
primitive embryonic mesenchymal tissue. Though the primary treatment
for sarcomas is surgical resection, because of their usual late and hi
gh stage presentation multimodal adjuvant therapy may be desirable. We
describe the clinical course of a 48-year-old male patient with a pri
mary renal osteosarcoma discovered by ultrasound. A marked reduction o
f vital tumor cells and an impressive increase of neoplastic bone form
ation following polychemotherapy is demonstrated histopathologically.
The principal clinical findings, differential diagnosis, etiology, pat
hogenesis and treatment modalities of this uncommon malignancy are dis
cussed.