Pitfalls in the diagnosis and follow-up of a giant pheochromocytoma

Abstract

Introduction: We report the case of a giant pheochromocytoma, which was a composite tumor, a very rare pathology accounting for only 3% of pheochromocytomas. Case presentation: A 61-year-old male patient presented with paroxysms: palpitation episodes, headache, and spells of elevated blood pressure (BP) for 4 years, as well as nocturnal diaphoresis, chronic diarrhea, and weight loss. The 24-h urine analysis showed a marked increase in normetanephrines and metanephrines. Computed tomography (CT) showed massive heterogeneous solid-cystic expansive formation with septations and areas showing soft tissue density. MIBG-iodine 131 had an extensive, rounded, heterogeneous hyper-uptake lesion in the left upper abdominal region, which measured 16.9 cm at its largest diameter. Finally, magnetic resonance imaging (MRI) revealed a massive expansive solid cystic lesion likely originating in the left adrenal gland. Discussion: Overall, 90% of patients with catecholamine-secreting tumors present with symptoms, and half have paroxysms. Fractionated metanephrines are one of the most sensitive and specific products released by tumors. Every patient with catecholamine-secreting tumors must undergo resection. Given the high surgical risk, the pre-operative period must be well managed by controlling the patient's blood pressure to avoid a hypertensive crisis during surgery. Conclusion: Proper pheochromocytoma diagnosis and treatment are important in endocrinology clinical practice because they help avoid sequelae and unexpected outcomes. Special care should be taken to perform a differential diagnosis in patients with elevated blood pressure, such as changing the workup applied to hypertensive patients, a common condition affecting the overall population.

Keywords

• Pheochromocytoma
• Secondary hypertension
• Adrenal gland
• Catecholamines