title: 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.
authors.affiliation: Children's Hospital Los Angeles, Los Angeles, California akaslow17@gmail.com cgayer@chla.usc.edu.
authors.forename: Abraham M
authors.initials: AM
authors.lastname: Kaslow
authors.affiliation: Keck School of Medicine of USC, Los Angeles, California.
authors.forename: Anne
authors.initials: A
authors.lastname: Riquier-Brison
authors.affiliation: Keck School of Medicine of USC, Los Angeles, California.
authors.forename: Janos
authors.initials: J
authors.lastname: Peti-Peterdi
authors.affiliation: Children's Hospital Los Angeles, Los Angeles, California.
authors.forename: Nick
authors.initials: N
authors.lastname: Shillingford
authors.affiliation: Children's Hospital Los Angeles, Los Angeles, California.
authors.forename: Josephine
authors.initials: J
authors.lastname: HaDuong
authors.affiliation: Baylor College of Medicine, Houston, Texas.
authors.forename: Rajkumar
authors.initials: R
authors.lastname: Venkatramani
authors.affiliation: Children's Hospital Los Angeles, Los Angeles, California Keck School of Medicine of USC, Los Angeles, California.
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Gayer
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D018246:Adrenocortical Adenoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D002648:Child; D006801:Humans; D006974:Hypertension, Malignant / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D008297:Male; D012083:Renin / Q000378:metabolism*
chemical_list: D012083:Renin
keywords: Adrenal mass; aldosterone; ectopic renin; malignant hypertension; pheochromocytoma