SAT-497 - Pheochromocytoma Presenting with Hypotension and Cardiogenic Shock: A Diagnostic Paradox

Background:

Pheochromocytomas typically present with hypertension as a result of catecholamine excess.  Rare cases manifest with paradoxical hypotension due to concurrent stress induced cardiomyopathy which delays diagnosis and worsens prognosis.

Case Presentation:

A 47-year-old woman with a history of Takotsubo cardiomyopathy presented to the emergency department with dyspnea and cough, and was found to be tachypneic, tachycardic, and hypotensive. 6 weeks prior, she was admitted to a different hospital facility with cardiogenic shock, where a left adrenal mass was found. While on pressors, metanephrines were collected. Despite a concern for falsely elevated levels, a trial of doxazosin was initiated which caused hypotension. She was eventually discharged home on prazosin 1mg daily. 

In the current presentation, diagnostic evaluation revealed leukocytosis, elevated troponins, EF of 30%, and left ventricular dysfunction supportive of stress induced cardiomyopathy. Treatment was initiated with vasopressors. Endocrinology was consulted due to worsening hypotension. Plasma metanephrine was found to be 186 pg/ml (ULN < 57 pg/ml), normetanephrine 794 pg/ml (ULN < 148 pg/ml) and 24-hour urine metanephrine was 691 mcg/24h (ULN=203 mcg/24 h), normetanephrine 1440 mcg/24h (ULN=649 mcg/24 h). Non contrast CT abdomen/pelvis confirmed an avidly enhancing rounded 3.3 cm left adrenal mass. Due to the strong clinical concern for pheochromocytoma, she was started on low dose doxazosin and discharged with follow-up.

Due to hypotension doxazosin adjustments were limited, in the next couple of weeks she continued to alternate between hypertensive crisis followed by hypotensive episodes. 6 weeks later, repeat non contrast CT revealed growing adrenal mass to 4.1 cm with HU of 45. She was on Doxazosin for a total period of 16 weeks, and the maximum tolerated dose was 3 mg twice daily prior to undergoing an adrenalectomy with complete tumor resection. Microscopic assessment confirmed a well-differentiated pheochromocytoma.


Discussion

The diagnosis of pheochromocytoma presenting with hypotension and cardiogenic shock is challenging, considering metanephrines obtained during critical illness may be misleading. Social barriers—including intellectual disability, limited support, and poor follow-up—further delayed accurate diagnosis. In this patient, clinical instability and enlarging adrenal mass on imaging served as strong diagnostic indicators. This case illustrates a rare clinical scenario in which clinical presentation and adrenal imaging findings outweighed biochemical work-up given the known limitation of metanephrines interpretation during shock state.

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