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

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.