Background: Intraspinal Wilms' tumor metastasis is rare, and is associated with a high mortality rate.
Methods: The authors reviewed the clinical course of two patients with Wilms' tumor in whom extradural metastasis developed. In addition, a review of the literature and of patients entered in the National Wilms' Tumor Studies was performed to determine the clinical presentation, treatment, and outcome of other patients with Wilms' tumor with intraspinal metastases.
Results: Both of the patients initially had abdominal pain without neurologic deficits. Despite therapy, paraplegia secondary to cord compression from recurrent epidural metastases developed in one patient, although a third remission has been achieved with further chemotherapy. the second patient remains in disease-free remission 25+ months after surgical resection of the extradural spinal tumor, adjuvant chemotherapy and radiation therapy, and autologous bone marrow transplantation. Review of the literature 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 early recognition and treatment of this complication of Wilms' tumor and demonstrates that intensive multimodality therapy can result in long-term disease-free remission.