INTRASPINAL WILMS-TUMOR METASTASES

Background. Intraspinal Wilms' tumor metastasis is rare, and is associ ated with a high mortality rate. Methods. The authors reviewed the cli nical course of two patients with Wilms' tumor in whom extradural meta stasis developed. In addition, a review of the literature and of patie nts entered in the National Wilms' Tumor Studies was performed to dete rmine the clinical presentation, treatment, and outcome of other patie nts with Wilms' tumor with intraspinal metastases. Results. Both of th e patients initially had abdominal pain without neurologic deficits. D espite therapy, paraplegia secondary to cord compression from recurren t epidural metastases developed in one patient, although a third remis sion has been achieved with further chemotherapy. The second patient r emains in disease-free remission 25+ months after surgical resection o f the extradural spinal tumor, adjuvant chemotherapy and radiation the rapy, and autologous bone marrow transplantation. Review of the litera ture and of the patients entered in the National Wilms' Tumor Studies revealed an additional 27 patients with Wilms' tumor with this pattern of metastasis. Only four were disease-free at the time of this report . Conclusions. The authors' experience stresses the importance of earl y recognition and treatment of this complication of Wilms' tumor and d emonstrates that intensive multimodality therapy can result in long-te rm disease-free remission.