Sporadic metastatic pheochromocytoma: A case report highlighting diagnostic and clinical considerations and brief literature review

Sporadic metastatic pheochromocytoma is a rare, typically localized tumor, associated with catecholamine excess and have potential for metastatic spread, posing significant diagnostic and treatment challenges. We report the case of a 41-year-old woman who presented with a six-month history of episodic headaches, palpitations, diaphoresis, severe hypertension, weight loss, and worsening fatigue. She was initially treated with beta-blockade for presumed anxiety and hypertension, which led to worsening symptoms. Laboratory evaluation revealed markedly elevated plasma and urinary metanephrines. Imaging demonstrated an 11 cm heterogeneous left adrenal mass with areas of necrosis, retroperitoneal lymphadenopathy, and bilateral pulmonary nodules. Functional imaging with I-123 metaiodobenzylguanidine scintigraphy confirmed metastatic involvement of the lymph nodes and lungs.
After multidisciplinary discussion, the patient underwent preoperative alpha-adrenergic blockade with phenoxybenzamine and volume expansion, followed by open left adrenalectomy with regional lymph node dissection. Histopathology showed classic pheochromocytoma with Zellballen architecture, marked pleomorphism, lymphovascular invasion, and a Ki-67 index of 12%. Immunohistochemical (IHC) analysis was positive for chromogranin A, synaptophysin, GATA3, and S100, indicating the presence of S100-positive sustentacular cells. 
Following surgery, the patient’s blood pressure and biochemical markers normalized. At 28-month follow-up, she remained clinically stable with decreased pulmonary nodules and no evidence of disease progression. This case highlights the importance of early recognition, avoiding isolated beta-blockade, coordinated multidisciplinary care, and long-term surveillance in metastatic pheochromocytoma.