Abstract

Background: In patients with system based diseases, multiple pathologies coexisting in the same organ can go undiagnosed. We present a case of a patient with long standing hypertension recently diagnosed with Primary hyperaldosteronism in the setting of known Pheochromocytoma. Case: A 43 year old male with a history of Von Hippel Lindau (VHL), Pheochromocytoma status post right subtotal adrenalectomy 18 years ago, renal cell carcinoma, prediabetes, bilateral renal cysts, and hypertension presented to the clinic for evaluation of suspected hyperaldosteronism. The patient had a history of medication refractory hypertension associated with hypokalemia requiring potassium supplementation. The patient’s VHL disease involved the pancreas, adrenal, brain and spine which required surgery to remove tumors in these areas. His Pheochromocytoma resolved status post subtotal adrenalectomy. Recent imaging and laboratory work up showed a stable left adrenal nodule (unchanged over the years), unremarkable right adrenal status post subtotal adrenalectomy with normal serum and urine metanephrines. In September 2019 he was seen by his Cardiologist for persistent Atrial Fibrillation and subsequent workup for suspected hyperaldosteronism showed a morning plasma aldosterone of 44.4ng/dL (normal range 4–21 ng/dL), plasma renin activity of <0.6 ng/ml/hr (normal range 0.6–4.3 ng/mL), 24 hour urine aldosterone of 21mcg/24hr (normal range 3–20 mcg/24hr), Urine sodium excretion of 236 mmol/24hr (normal range 40-217mmol/24hr), and potassium of 3.5mmol/L (normal range 3.6–5.0 mmol/L). The diagnosis of Primary hyperaldosteronism was confirmed and the patient was subsequently scheduled for adrenal venous sampling. Conclusion: This case demonstrates how multiple adrenal pathologies can present in the same individual. There is paucity of data in the literature as to the existence of both Pheochromocytoma and primary hyperaldosteronism in the same individual or the pathophysiological connection between them. When the diagnosis of a disease affecting a specific organ has already been made, the discovery of a secondary pathologic process affecting such organ may end up getting delayed. Clinicians should be aware of this possibility.

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