Scholarlink

Purpose <p>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.</p> Methods <p>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<sup>®</sup>.</p> Results <p>Among 410 patients, 6.3% (<i>n</i> = 26) had bilateral pheochromocytomas. Most patients were male (57.7%, <i>n</i> = 15), with a mean age of 43.3 ± 18.7 years. Tumors were synchronous in 80.8% (<i>n</i> = 21). All genetically tested bilateral cases harbored germline mutations, mainly MEN2A (62.5%, <i>n</i> = 15), and VHL (17%, <i>n</i> = 4). Compared with unilateral disease, bilateral pheochromocytomas occurred in younger patients (<i>p</i> = 0.008), were smaller (<i>p</i> = 0.021), and were more strongly associated with germline mutations (<i>p</i> &lt; 0.001). Biochemical normalization after surgery was more frequent in unilateral cases (<i>p</i> &lt; 0.001). Surgical (<i>p</i> = 0.816) and postoperative complications rates (<i>p</i> = 0.874), as well as length of hospital stay, were similar (<i>p</i> = 0.119). Rates of recurrence (<i>p</i> = 0.529), metastasis (<i>p</i> = <i>0.709</i>) and mortality (<i>p</i> = <i>0.477</i>) did not differ significantly.</p> Conclusions <p>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.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Bilateral pheochromocytoma: differences in clinical and surgical outcomes with unilateral pheochromocytomas

  • 期刊论文

  • 发表日期：

    2026年4月4日

查看全文
  • Sara Donato,
  • Aura D. Herrera-Martínez,
  • Iñigo García Sanz,
  • César Mínguez Ojeda,
  • Felicia Alexandra Hanzu,
  • Mireia Mora,
  • Rocío Revuelta Sánchez-Vera,
  • Margarita González Boillos,
  • Pablo Abellán-Galiana,
  • Cristina Lamas,
  • María del Carmen López García,
  • María del Castillo Tous,
  • María Ruíz Rodríguez,
  • Rebeca Barahona San Millán,
  • Mónica Recasens,
  • Paola Gracia Gimeno,
  • Cristina Robles Lazaro,
  • Ana Herrero-Ruiz,
  • Theodora Michalopoulou,
  • Edelmiro Menéndez,
  • Rosario Fernández García-Salazar,
  • Patricia Pérez Castro,
  • Cristina Álvarez-Escolá,
  • Patricia Martín Rojas-Marcos,
  • Rogelio García-Centeno,
  • Laura González,
  • Paz de Miguel Novoa,
  • Concepción Blanco,
  • Nuria Valdés,
  • María Calatayud,
  • Marta Araujo-Castro

摘要

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.