Clinical presentation of rhabdoid tumors of the kidney

Purpose: We designed this study to differentiate the clinical presentation. particularly the incidence of hematuria, of a rhabdoid tumor of the kidney (RTK), a rare but highly malignant tumor. From a Wilms tumor. Patients and Methods: We reviewed patient flow charts from the National Wil ms Tumor Study Group and queried participating hospitals to obtain addition al information regarding presenting symptoms and laboratory data for fifty patients. patient ages ranged from ? days to 3.5 years with a mean of Il mo nths. We documented the presence of gross and microscopic hematuria, fever, and hypercalcemia. Results: Whereas 75% of children with rhabdoid tumor of the kidney (RTK) ha d stage III (44%). IV (27%), or V (4%) tumors. 67% of children with Wilms t umors had stage I (41%) or II (26%) tumors. Either gross or microscopic hem aturia was present in 84.4% (27/32) of the patients with RTK. Gross hematur ia was present in 59% (22/37) of children with RTK compared with 18% previo usly reported with Wilms tumor. Microscopic hematuria was present in 76% (2 2/29) of children with RTK compared with 24% previously reported with Wilms tumor. Fever was found in 44% (16/36) of children with RTK. compared with 22% of children previously reported with Wilms tumor. Hypercalcemia was see n 26% (6/23) of children with RTK. Conclusion: Although diagnosis of any renal mass still must be confirmed wi th histopathologic features, a distinct clinical presentation with fever, h ematuria, a young age, and high-tumor stage at presentation suggests the di agnosis of RTK.