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The clinical features and management of severe hypertension (HT) (blood pressure >99th percentile+5 mm Hg) have been rarely described in pediatric oncology.Retrospective descriptive study of the case files of 31 patients followed in the Institut Curie Department of Pediatric Oncology between 1999 and 2004 and presenting severe HT at the time of diagnosis of their tumor.The median age was 2 years 1 month (range: 3 mo to 6 y 8 mo). Median blood pressure was 99th percentile+30 mm Hg (range: 99th percentile+7 mm Hg to 99th percentile+62 mm Hg). The tumors presented by these children were: Wilms tumor (n=17, ie, 20% of all Wilms tumors treated during this period), neuroblastoma (n=12, ie, 10% of all neuroblastomas treated during this period) or other tumors (n=2). HT was asymptomatic in all children. Initial management consisted of etiologic treatment by primary chemotherapy and/or surgical resection of the tumor, associated with antihypertensive therapy, initially administered by intravenous injection for 12 children (nicardipine, labetalol) and then orally in all children (calcium channel blockers, n=23; angiotensin-converting enzyme inhibitor, n=16; β-blockers, n=4; α/β-blockers, n=2; diuretics, n=1). Dual therapy was necessary in 7 cases and triple therapy was necessary in 1 case. The median duration of antihypertensive therapy was 40 days (range: 9 to 195). No child developed a serious complication of HT.Initial HT is a frequent complication of Wilms tumor and neuroblastoma and affects young children (<2.5 y). It is often severe, asymptomatic, but needs specific treatment and resolves after treatment of the tumor.