Catastrophic Presentation of a Ruptured Pheochromocytoma

Background: Pheochromocytoma is a catecholamine-secreting tumor, encountered in less than 0.5% of patients with hypertension and around 4% of patients with adrenal incidentaloma.(1) It classically presents with episodic headache, sweating, and hypertension but rarely can present with serious complications such as hypertensive, pheochromocytoma crisis, and hemorrhagic shock. Clinical Case: We report a 49-year-old male patient with a history of hypertension for five years on Amlodipine but not compliant. Presented to the Emergency Department with sudden onset left severe flank pain associated with one episode of vomiting for six hours. Vital signs were stable, and basic labs were within normal. Bedside ultrasound couldn’t appreciate any abnormality due to obesity, urinary tract CT-scan showed a large heterogeneous non-enhancing mass, possibly hematoma arising from left adrenal gland. Afterward, his condition deteriorated rapidly, he became hypotensive, and hemoglobin level dropped from 14 to 8gm/dl within a few hours. He became agitated with a worsening level of consciousness, so he was admitted under the medical intensive care unit (MICU) and required intubation and started on mechanical ventilation. Abdominal CT-angiography was done to look for any active bleeding, showed re-demonstration of the same lesion in the left adrenal without contrast enhancement or extravasation. He underwent urgent explorative laparotomy that showed ruptured large left adrenal hematoma, which was entirely evacuated with the adrenal tissue, his vital signs were maintained stable during the surgery. Postoperative course showed uncontrolled blood pressure requiring three anti-hypertensive medications; otherwise, he had gradual improvement until successful weaning, and returned to his baseline. The histopathology result was suggestive of pheochromocytoma with extensive bleeding. Conclusion: Ruptured pheochromocytoma is an extremely rare presentation of hemorrhagic shock and needs a high index of suspicion. In our patient, the lack of classical presentation and imaging features of pheochromocytoma combined with the condition rarity, made the diagnosis very challenging. Urgent surgical intervention may be warranted, and multidisciplinary perioperative preparation is the key to a favorable outcome. References: (1) Bravo EL. Pheochromocytoma: new concepts and future trends. Kidney Int. 1991 Sep;40(3):544–56. doi: 10.1038/ki.1991.244. PMID: 1787652.