Adult Wilms' tumor. Report of two cases and review of the literature

Abstract

Background: Wilms' tumor is rare in adults. The recommended treatments for patients with Stage II adult Wilms' tumor with favorable histology (FH) nephroblastomas are conflicting.

Methods: Two patients with Stage II, favorable histology, adult nephroblastomas are described. Current treatment modalities are discussed and the literature is reviewed.

Results: The first patient, a 52-year-old woman, probably had a late local relapse of a Wilms' tumor 21 years after nephrectomy because of a renal tumor originally diagnosed as reticular sarcoma. In this case, a recurring or an extrarenal Wilms' tumor should have been considered. After the tumor was removed, the patient received adjuvant chemotherapy with dactinomycin and vincristine and was disease free 44 months after diagnosis. The Wilms' tumor in the second patient, a 33-year-old woman, was discovered accidentally and classified as Stage II/FH based on preoperative biopsy. She was treated with radical nephrectomy and adjuvant chemotherapy with dactinomycin and vincristine. This patient was disease free 24 months after surgery.

Conclusions: Surgery and two-drug chemotherapy with dactinomycin and vincristine is suggested for patients with Stage II adult Wilms' tumor with FH nephroblastomas.