Pheochromocytoma: A three-decade clinical experience in a multicenter study.

Area of Science:

• Endocrinology
• Surgical Oncology
• Oncologic Imaging

Background:

• Pheochromocytoma (PCC) is a rare neuroendocrine tumor arising from chromaffin cells.
• Understanding the clinical spectrum and management of PCC is crucial for patient outcomes.

Purpose of the Study:

• To comprehensively analyze the clinical and analytical features of pheochromocytoma (PCC).
• To evaluate diagnostic modalities, therapeutic interventions, and patient outcomes in surgically treated PCC.
• To identify factors associated with tumor characteristics and disease recurrence.

Main Methods:

• A multicenter retrospective study involving 106 surgically treated patients with PCC across 3 Spanish tertiary referral hospitals.
• Data collection included clinical presentation, diagnostic test results (e.g., 24-h urinary fractionated metanephrines, ¹²³I-metaiodobenzylguanidine scintigraphy), surgical techniques, and follow-up outcomes.
• Statistical analysis compared features between sporadic and familial PCC, and symptomatic and incidental tumors.

Main Results:

• The study evaluated 106 patients (57.5% women, mean age 52.3 years); 83% had sporadic PCC, and 62% were symptomatic.
• Familial PCC patients were younger and often associated with MEN2A. Tumor size correlated with 24-h urinary fractionated metanephrines.
• ¹²³I-metaiodobenzylguanidine scintigraphy showed 81.9% sensitivity. Laparoscopic surgery was performed in 2/3 of patients with low complication rates (20%) and recurrence (10%). Malignant PCC was uncommon (6.3%).

Conclusions:

• Surgically treated pheochromocytomas in Spain are generally large, symptomatic, and sporadic, diagnosed in the sixth decade.
• Hereditary PCC is frequently linked to MEN2A.
• Laparoscopic surgery is the preferred approach, with a low incidence of metastatic disease.