71 patients with resectable osterosarcoma received chemotherapy for one year including high-dose methotrexate (18 x 200 mg/kg), adriamycin (5 x [2 x 45] mg/m2) and cyclophosphamide (6 x 1200 mg/m2). During the initial 15 weeks adriamycin was used preferentially and cytostatic agents were applied in a higher frequency than later on. 41/71 patients are continuously free of disease with a median follow up of 39 (24-54) months. The latest appearance of pulmonary metastases was observed at 28 months so far. 18/27 (67%) patients with extension of tumor lesion beyond 1/3 long bones length by x-ray examination relapsed in contrast to 12/43 (28%) patients with smaller lesions. 1 patient died from adriamycin induced cardiomyopathy. Generally therapy was well tolerated. An average of 70-80% of planned drug dosages could be realized without measurable influence of individual differences on outcome.
The disease of a four week old boy is described, which presented with papulous skin infiltrations, lethargy, anemia and bilateral testicular swelling. The investigation of bone marrow, cerebrospinal fluid and skin biopsy revealed infiltration by a leukemic process which could be identified by means of cytology and cytochemistry as erythroleukemia at the stage of erythremic myelosis. Death occurred 20 days after the onset of symptoms. Only one previous report of congenital erythroleukemia could be found in the literature. The findings are briefly discussed.
We describe the case history of a 13 years old boy who developed osteogenic sarcoma of the left thigh, six years after diagnosis of osteogenesis imperfecta tarda with a positive family history. Only four other patients with this disease combination are reported in the literature. Preoperative treatment with high-dose Methotrexate caused marked tumor regression, as shown at examination of amputation material. The inter-relationships between the two disorders are discussed and the literature is briefly reviewed.
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