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[Cardiac failure by major arterial hypertension secondary to nephroblastoma]
Insights
A rare case of severe hypertension in an infant was linked to Wilms tumor, a type of kidney cancer. This finding suggests primary hyperreninism from the tumor may cause high blood pressure in some children.
Area of Science:
- Pediatric Nephrology
- Pediatric Oncology
- Cardiology
Background:
- Systemic arterial hypertension is a rare but serious complication in pediatric oncology.
- Wilms tumor, a common pediatric kidney malignancy, can be associated with hypertension, often attributed to renal vein compression and hyperreninism.
Observation:
- A 7-week-old boy presented with general symptoms, rapidly developing cardiac failure due to severe systemic arterial hypertension.
- Hypertension management required intensive care, mechanical ventilation, and antihypertensive medications (captopril, frusemide).
- Diagnostic investigations confirmed a left-sided Wilms tumor.
Findings:
- Elevated renin levels were detected in the left renal vein during nephrectomy.
- Analysis of tumoral tissue indicated renin production within the Wilms tumor itself, suggesting primary hyperreninism.
- This contrasts with mesoblastic nephroma, where renin is found in compressed, non-tumoral tissue.
Implications:
- This case highlights that Wilms tumors can directly secrete renin, leading to severe hypertension through primary hyperreninism.
- Further research is warranted to determine if renin secretion is a characteristic of all or only specific subtypes of Wilms tumors.
- Understanding the source of renin is crucial for accurate diagnosis and management of hypertension in pediatric kidney cancer patients.
Abstract:
The authors report on the case of a 7 week-old boy, in whom a renal mass was discovered after general symptoms were observed. Within 48 h, cardiac failure secondary to systemic arterial hypertension occurred, requiring intensive care. After a few days of mechanical ventilation and alternating elevated and low blood pressure, improvement was obtained with captopril and frusemide enabling further investigations to be carried out which lead to the diagnosis of Wilms tumor. During left-sided nephrectomy, elevated renin from the left renal vein was found. The post surgical course was excellent. Several authors have reported on the association between arterial hypertension and nephroblastoma as being the result of hyperreninism due to hilar compression; however severe hypertension was uncommon. Renin activity determination from the tumoral tissue had led to a different interpretation, ie primary hyperreninism: in the case of mesoblastic nephroma, only the non tumoral but compressed tissue contains a large quantity of renin; in the case of nephroblastomas, only the tumoral tissue contains renin. The question now is whether all or only certain nephroblastomas secrete renin.