Case Presentation: A 39-year-old active MMA fighter with hypertension and type 2 diabetes mellitus, both diagnosed in early adulthood, presented with 1 day of crampy, left-sided abdominal pain associated with multiple episodes of vomiting. He also reported episodic dizziness, flushing, headache, and rises in blood pressure (BP) over the two years prior. He denied recent injury to flank or abdomen. On admission, his blood pressure and glucose were both elevated. Physical exam was notable for tender left upper quadrant without palpable masses. A Computed Tomography (CT) scan of the abdomen and pelvis with intravenous contrast revealed a 6.5×6 cm mass in the left adrenal gland hemorrhaging into the retroperitoneum. Magnetic Resonance Imaging (MRI) confirmed a large intra-adrenal hemorrhage measuring 8.5x7x8.4 cm extending to the retroperitoneum. Urine and serum metanephrines and normetanephrines were elevated. Adrenal Surgery was consulted, and a plan was made for resection of the tumor. The patient’s BP medication was transitioned to phenoxybenzamine prior to surgery. After BP was controlled with alpha-blockade, he underwent laparoscopic left adrenalectomy without complications. Pathology confirmed pheochromocytoma. One month after the surgery, the patient’s BP and diabetes were controlled without medication. Repeat blood metanephrines and normetanephrine normalized. Given the patient’s large tumor and young age, he was referred to Medical Genetics. He was offered a personalized hereditary cancer gene panel that includes 34 standard cancer panels, and 13 additional genes associated with pheochromocytomas, which came back negative.
Discussion: Spontaneous hemorrhage within a pheochromocytoma, resulting in retroperitoneal or intra-peritoneal hemorrhage, is a rare and life-threatening event with a mortality rate of 30%. Here, we present a rare case of a spontaneously hemorrhaging pheochromocytoma in a young adult with early-onset hypertension and diabetes who presented with abdominal pain. For diagnosis, both CT and MRI can be helpful, and all patients with hemorrhagic adrenal masses should undergo a hormonal assessment to exclude hormonally active tumors. Patients with massive bleeding or hemodynamic instability require emergency surgery, which carries a high mortality rate of 40-45%. In our case, the patient was hemodynamically stable, allowing for elective surgery and an uneventful recovery. In the preoperative management of pheochromocytoma, alpha-adrenergic receptor blockers are the first-choice medication. Approximately 40% of affected individuals have a hereditary predisposition, and many have bilateral tumors; however, genetic testing in our patient was negative. Establishing potential risk factors for hemorrhage remains challenging, as demonstrated in our case. After the operation for hemorrhagic pheochromocytoma, careful lifelong follow-up is recommended for optimal patient care.
Conclusions: This case emphasizes the importance of lowering the suspicion threshold for pheochromocytoma in young patients with hypertension and diabetes who present with abdominal pain and vomiting. It is important to maintain a broad differential to avoid premature closure on a diagnosis. Although uncommon, pheochromocytoma is a critical diagnosis to consider, as missing it can result in severe complications and a high risk of mortality.