MON-537 - Menacing Metastatic Pheochromocytoma & Forsaken Follow-Up

Pheochromocytomas (PCC) are rare catecholamine-secreting tumors that originate from adrenal medulla chromaffin cells; 10 to 15% of PCC cases are metastatic and present similarly with identical histology. Treatment depends on whether the metastasis is diffuse or limited. This case demonstrates metastatic PCC in an elderly male with significant social barriers.
A 76-year-old male with history of hypertension presented with altered mentation and hypertensive urgency after 8 years without care, reporting a 15-lb weight loss in 3 weeks. Imaging showed a 4.9 x 5.3 cm right adrenal mass with mild thickening of the left adrenal gland. The patient stabilized with alpha and beta blockade and was discharged. Plasma metanephrines (MN) were 4.89 nmol/L (0-0.49 nmol/L) and normetanephrines (NM) 27.80 nmol/L (0-0.89 nmol/L). After weekly BP checks and medication titration, he underwent right adrenalectomy. Repeat MN and NM decreased to 0.16 and 2.68 nmol/L, respectively. Pathology was reported as benign with a PASS score of 2, indicating very little concern for malignant potential. He was lost to follow-up.
Two years later, now undomiciled, he returned with hypertensive urgency with elevated plasma MN and NM to 9.00 nmol/L and 46.41 nmol/L. Liver ultrasound revealed multiple hepatic lesions; CT showed bilobar hypoattenuating lesions concerning for metastasis and recurrent PCC. He was restarted on doxazosin with biopsy deferral until appropriate blockade. Ga-68 dotatate PET scan was advised on discharge but not completed.
Two months later he returned after a fall with hypertension. He frequently left AMA and was non-adherent to BP checks and medications. An I-123 MIBG/SPECT scan was done inpatient which found innumerable skeletal metastases in the spine, pelvis, femur, and ribs, and liver lesions. MN and NM remained elevated to 2.68 nmol/L and 46.10 nmol/L. Liver biopsy one month after alpha blockade confirmed metastatic PCC. Multidisciplinary discussions led to a plan of initiating outpatient belzutifan. He was discharged to a subacute rehabilitation facility (SAR) and was lost to follow-up.
This case highlights the discovery of recurrent metastatic pheochromocytoma along a multi-year course, complicated by significant social challenges. Between the initial PCC discovery and the next admissions, the patient became undomiciled and could not adequately adhere to diagnostics. He was deemed to have no capacity to refuse treatment and SAR placement. These factors together contributed to the delay in his eventual diagnosis. In such cases, engaging multidisciplinary teams and incorporating early screening for social determinants of health are essential to achieving timely diagnosis. Medical complexity combined with social barriers hinder the recognition of high-risk conditions, such as PCC.

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