Militello Claudio, Jorge Cesar, Pisani Juan, Cenice Fernando, Chagra Carolina
Hospital San Bernardo. Universidad de Ciencias de la Salud (UNSA). Salta. Argentina.
Arch Esp Urol. 2020 Sep;73(7):611-623.
Perform a review on the diagnosis and treatment of pheochromocytomas and malignant paragangliomas. MATERIAL AND METHOD: A search was conducted in PubMed and Google Scholar of articles or clinical guides that referred to the diagnosis and treatment of these tumors. RESULTS: For the diagnosis of malignancy, a histological confirmation of a pheochromocytoma or paraganglioma should be provided, plus the presence of metastasis confirmed by images. Methanephrines are recommended over other biochemical determinations. For staging, PET-CT with 18F-FDG or 18F-DOPA is preferred because of its greater sensitivity than conventional images. The 123I-MIBG scan should be requested when radiotherapy with 131I-MIBG is planned.For treatment, control of adrenergic symptoms through the use of α-blockers is recommended. Active surveillance was an option in selected patients with slowly progressive tumors. Surgical treatment improved OS (148 months vs 36 months p=<0.01). Therapy with 131I-MIBG was indicated in patients with positive scintigraphy, reporting a global survival of 50% at 5 years with variable tumor responses. Chemotherapy was proposed in rapidly progressive disease, reporting a median overall survival of 6 years. Ablative therapies should be considered when there is a limited number of lesions, to achieve local tumor control and reduce the symptoms of excess catecholamines. External radiation therapy at high doses would be effective for patients with local symptoms due to their tumor burden. Prospective multi-institutional clinical trials are needed to determine the true benefits of molecular therapies in these patients.
We recommend a multidisciplinary approach in centers of high complexity to be able to offer the entire diagnostic - therapeutic arsenal available so far that they improve the survival and quality of life of these patients.
对嗜铬细胞瘤和恶性副神经节瘤的诊断与治疗进行综述。
在PubMed和谷歌学术上搜索提及这些肿瘤诊断与治疗的文章或临床指南。
对于恶性肿瘤的诊断，应提供嗜铬细胞瘤或副神经节瘤的组织学确诊，以及影像学证实的转移情况。相较于其他生化检测，推荐使用甲氧基肾上腺素。对于分期，由于18F-FDG或18F-DOPA的PET-CT比传统影像具有更高的敏感性，因此更受青睐。当计划使用131I-MIBG进行放射治疗时，应进行123I-MIBG扫描。对于治疗，建议通过使用α受体阻滞剂来控制肾上腺素能症状。对于部分肿瘤进展缓慢的患者，主动监测是一种选择。手术治疗可改善总生存期（148个月对36个月，p<0.01）。对于闪烁显像阳性的患者，建议使用131I-MIBG治疗，5年总生存率为50%，肿瘤反应各异。对于疾病进展迅速的患者，建议进行化疗，中位总生存期为6年。当病变数量有限时，应考虑消融治疗，以实现局部肿瘤控制并减轻儿茶酚胺过多的症状。高剂量外照射放疗对因肿瘤负荷导致局部症状的患者有效。需要进行前瞻性多机构临床试验来确定分子疗法在这些患者中的真正益处。
我们建议在高复杂性中心采用多学科方法，以便能够提供目前所有可用的诊断 - 治疗手段，从而提高这些患者的生存率和生活质量。
