An ectopic renin-secreting adrenal corticoadenoma in a child with malignant hypertension

Abstract

A previously healthy 7-year-old male presented with hypertensive emergency, hypokalemia, and elevated plasma renin activity and aldosterone levels. There was no evidence of virilization or cushingoid features. MRI of the abdomen revealed a large (5 × 5 × 3 cm) peripherally enhancing, heterogeneous mass arising from the left adrenal gland. The patient was treated for a suspected pheochromocytoma. However, his blood pressure was not responsive to alpha-blockade. Blood pressure was controlled with a calcium channel blocker and an angiotensin-converting enzyme (ACE) inhibitor. A complete surgical resection of the mass was performed. Postoperatively, his blood pressure normalized and he did not require antihypertensives. On pathological examination, the tumor tissue stained negative for chromogranin and positive for renin. The final diagnosis was renin-secreting adrenal corticoadenoma, an extremely rare adrenal tumor not previously reported in a pediatric patient. Malignant hypertension due to a renin-secreting tumor may need to be distinguished from a pheochromocytoma if alpha-adrenergic blockade is ineffective.

Keywords: Adrenal mass; aldosterone; ectopic renin; malignant hypertension; pheochromocytoma.

Figure 1

T2‐weighted MRI of abdomen, coronal view. The arrow indicates a 5‐cm lesion above the left kidney consistent with an adrenal mass.

Figure 2

Histochemical analysis of adrenal mass. (A) H&E stain, 200× magnification. This section shows a monotonous population of neoplastic cells with abundant clear cytoplasm and small bland appearing nuclei. (B) 200×. Renin immunolabeling is demonstrated in red and is diffusely present in the tumor section. Normal adrenal tissue displays green autofluorescence.