Bilateral pheochromocytoma: differences in clinical and surgical outcomes with unilateral pheochromocytomas.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Donato Sara, Herrera-Martínez Aura D, García Sanz Iñigo, Mínguez Ojeda César, Hanzu Felicia Alexandra, Mora Mireia, Revuelta Sánchez-Vera Rocío, González Boillos Margarita, Abellán-Galiana Pablo, Lamas Cristina, López García María Del Carmen, Del Castillo Tous María, Ruíz Rodríguez María, Barahona San Millán Rebeca, Recasens Mónica, Gracia Gimeno Paola, Robles Lazaro Cristina, Herrero-Ruiz Ana, Michalopoulou Theodora, Menéndez Edelmiro, Fernández García-Salazar Rosario, Pérez Castro Patricia, Álvarez-Escolá Cristina, Martín Rojas-Marcos Patricia, García-Centeno Rogelio, González Laura, de Miguel Novoa Paz, Blanco Concepción, Valdés Nuria, Calatayud María, Araujo-Castro Marta
Аннотация
PURPOSE: Pheochromocytomas are rare neuroendocrine tumors, and data on bilateral cases remain limited. This study aimed to characterize the clinical, genetic and outcome features of bilateral pheochromocytomas and to compare them with unilateral tumors. METHODS: A retrospective multicenter study was conducted across 18 tertiary hospitals, including patients who underwent surgical resection of pheochromocytoma between 1986 and 2025. Demographic, clinical, biochemical, hormonal, genetic, radiological, surgical, and long-term follow-up data were extracted from electronic medical records and recorded in an electronic database (RedCap). Statistical analyses were performed using Jamovi®. RESULTS: Among 410 patients, 6.3% (n = 26) had bilateral pheochromocytomas. Most patients were male (57.7%, n = 15), with a mean age of 43.3 ± 18.7 years. Tumors were synchronous in 80.8% (n = 21). All genetically tested bilateral cases harbored germline mutations, mainly MEN2A (62.5%, n = 15), and VHL (17%, n = 4). Compared with unilateral disease, bilateral pheochromocytomas occurred in younger patients (p = 0.008), were smaller (p = 0.021), and were more strongly associated with germline mutations (p < 0.001). Biochemical normalization after surgery was more frequent in unilateral cases (p < 0.001). Surgical (p = 0.816) and postoperative complications rates (p = 0.874), as well as length of hospital stay, were similar (p = 0.119). Rates of recurrence (p = 0.529), metastasis (p = 0.709) and mortality (p = 0.477) did not differ significantly. CONCLUSIONS: Bilateral pheochromocytomas are strongly associated with genetic syndromes. Individuals with bilateral disease are younger and have smaller tumors at diagnosis. Although surgical morbidity and long-term outcomes are comparable, biochemical normalization is less frequent in bilateral disease. This provides the first direct comparison between unilateral and bilateral pheochromocytomas.