Significance of mediastinal metastatic disease in Wilms' tumor of favorable histology

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Abstract
Although the results of the National Wilms' Tumor Study suggest poor outcome for children with metastases to intra‐abdominal, intracranial, skeletal, skin, diaphragm, or mediastinal sites, the incidence and course of mediastinal disease has not been described. Fifty‐four newly diagnosed Wilms' tumor patients at Children's Hospital of Michigan (1972‐1980) were studied for patterns of intrathoracic metastases. All had favorable histology; mean age (MA) at diagnosis was 4.5 years. Initial chemotherapy ranged from single agent to three‐drug combination (actinomycin, vincristine, adriamycin). Intrathoracic metastases occurred in 14 (26%) from 0‐28 months (median, 3.5 months) from diagnosis (MA, 5.8 years). Upon the discovery of intrathoracic metastases all received radiation (1,200‐2,000 rads) to the chest, some with supplementary doses to the mediastinum. Five children with parenchymal and one with parenchymal and hilar metastases appearing 0‐28 months after the time of diagnosis survive disease‐free 44‐127 months after metastases appeared (MA, 6.3 years). Eight children developed parenchymal and mediastinal metastases (MA, 5.4 years). Time from diagnosis to parenchymal metastases was 0‐7 months; time from diagnosis to mediastinal metastases was 0‐16 months; and in four, both sites appeared simultaneously. Death occurred in all eight patients 7‐25 months from diagnosis. Only one child had evidence of recurrence outside the chest. Potential risk factors and influence on survival are discussed.