A 30–year–old woman presented with 7 days of intermittent fevers, sweats, and flank pain. She noted nausea, vomiting, and dysuria, and severe headaches for the prior five days, a history of uncomplicated urinary track infections, and a diagnosis of hypertension since age 27, for which she was taking lisinopril. Her temperature was 39.6°, heart rate was 162 bt/min, blood pressure was 200/100 mmHg and she was tachypneic. Her physical examination was normal except left costovertebral angle tenderness, and left lower quadrant tenderness. Her WBC was 24,000 cells/mm3; the urinalysis revealed leukocyte esterase, eight WBCs, and bacteria. When corrected for a low albumin of 3.0, she was also hypercalcemic. Pyelonephritis was presumed and antibiotics with intravenous fluids initiated. Renal ultrasound revealed a 10cm left adrenal mass and a 4 cm right adrenal mass. She continued to have episodes of headache, tachycardia, and hypertension. These spells, in addition to the bilateral adrenal masses, prompted a 24–h urine metanephrine and VMA collection, which were elevated. In preparation for surgery, an MIBG scan showed increased uptake in both adrenal masses. There was no evidence of invasion per imaging. She was started on a–blockade and surgery was scheduled. She remained hypophosphatemic, despite oral supplementation of phosphorous. PTH and calcitonin were elevated. An ultrasound of her thyroid revealed suspicious nodules, but biopsy was normal. A sestamibi scan showed no parathyroid adenomas. MEN genetics confirmed RET mutation. A left adrenalectomy and a right subtotal adrenalectomy were performed to avoid steroid dependence.
Hospitalists are much less likely to encounter MEN–2A than pyelonephritis, but the diagnosis and mangement of both is important for MEN–2A patients. Pressure to treat the admitting diagnosis and ensure quick hospital throughput, requires hospitalists to maintain the discipline necessary to interpret all of the historical, laboratory and physical examination data to ensure that all patient issues are adequately addressed. Admission for common diseases is usually how rare diagnoses are identified. To balance efficiency and thoroughness, hospitalists must look for historical, examination and lab clues for a second or more severe diagnosis. The clues in this case were the tachycardia, which was too fast to be merely a compensation for fever, and the hypertension too high to be due solely to pain.
Secondary causes of hypertension should be considered in any individual with early onset hypertension, especially before age of thirty. With hyperparathyroidism and pheochromocytoma diagnosed, genetic testing to identify the RET oncogene mutation and MEN2A is essential. Pheochromocytoma occurs in about 50% of cases of MEN2A. Adrenalectomy is required to remove the source of adrenergic surge to prevent recurrence of paroxysms of hypertension and tachycardia. Prophylactic thyroidectomy is required in all cases of MEN2A or MEN2B.