(1.13) “Did my bleed cure me?”: Pheochromocytoma that self-resolved with a spontaneous hemorrhage

Pheochromocytomas are catecholamine-secreting adrenal medullary tumors. Due to their wide variety of clinical presentations, they are called ‘great mimics’.  Untreated, they typically cause progressive catecholamine excess, high output cardiac failure, and death.

Case(s) Description :

A 46-year-old woman presented to the emergency department with acute left-sided abdominal pain and vomiting. She was afebrile, but tachycardic and hypotensive and had left-sided abdominal tenderness and an elevated serum lactate.  Contrast-enhanced abdominal CT scan found no clear cause for pain but showed an indeterminate 4.2 cm left adrenal nodule, 18 Hounsfield units (HU) on virtual non-contrast images.  Hypotension and lactate improved with intravenous fluids.  Plasma and urine metanephrines were drawn to facilitate outpatient workup of the nodule.

One day later she developed an acute change in mental status, worsening abdominal pain, hypertensive urgency, and supraventricular tachycardia. Her blood pressure (BP) plummeted to 76 mmHg systolic, with a simultaneous two gram/dL hemoglobin (Hb) drop to 6.2 g/dl, requiring transfusion.  By day three, her pain improved dramatically, and BP began to stabilize. A new non-contrast CT to investigate the Hb drop showed a very different picture: the mass was larger, 4.6 cm, heterogenous with areas measuring 26 to 38 HU, and with surrounding fluid and inflammation consistent with spontaneous interval hemorrhage. The plasma metanephrines sent prior to the bleed resulted showing a free metanephrine of 215 pg/ml (ref < =57), free normetanephrine of 7677 pg/ml (ref < =148), for a total of 7892 pg/mL (ref < =205), greater than 30 times normal.  The 24-hour urine fractionated metanephrine came back at 1180 mcg (ref 58-203), normetanephrine of 5747 mcg (ref 88-649), for a total of 6927 mcg/24h (ref 182-739), almost ten times normal and highly suggestive of pheochromocytoma.

Five days later, her symptoms had mostly resolved, though she was found to have a cardiac ejection fraction (EF) < 25% with left ventricular wall motion consistent with stress-induced cardiomyopathy. Repeat plasma free metanephrine was only 83 pg/ml, normetanephrine 147 pg/ml, and total metanephrines 230 pg/ml. These were close to the normal range and suggested the rare possibility of a pheochromocytoma having cured itself.  Six months later, a follow up CT showed near complete resolution of the adrenal mass, and her EF had returned to normal, confirming a likely Takotsubo cardiomyopathy.

Discussion :

A scattering of case reports exist in the literature of spontaneous pheochromocytoma hemorrhage. Although rare, these can be highly catastrophic with outcomes from silent hemorrhage to shock and death. This case is yet another example of the importance of diagnosis of pheochromocytoma even when the classically described triad of episodic headaches, palpitations and sweating, along with paroxysmal hypertension are absent.  Being one of the most common causes of spontaneous adrenal bleeding, pheochromocytomas should always be considered in cases of adrenal hemorrhage.  Our patient represents a rare case as she not only survived, but had resolution of symptoms.