Strajina Veljko, Dy Benzon M, Farley David R, Richards Melanie L, McKenzie Travis J, Bible Keith C, Que Florencia G, Nagorney David M, Young William F, Thompson Geoffrey B
Department of Surgery, Mayo Clinic, Rochester, MN, USA.
Division of Medical Oncology, Mayo Clinic, Rochester, MN, USA.
Ann Surg Oncol. 2017 Jun;24(6):1546-1550. doi: 10.1245/s10434-016-5739-5. Epub 2017 Jan 5.
Pheochromocytoma and paraganglioma (PPGL) are rare neoplasms; about 10% are malignant. Literature regarding possible benefit from resection is extremely limited.
A 20 year review of all patients undergoing surgery for malignant PPGL at the Mayo Clinic Rochester Campus between 1994 and June 2014 was performed.
We identified 34 patients undergoing surgery for malignant PPGL. Median follow up was 6 and 5 years survival was 90% (median 11 years). Complete resection (R0) was achieved in 14 patients (41%). Median disease-free survival was 4.6 years for patients with R0 resection (up to 12 years). Only eight patients (23%) were disease-free on last follow up. Elevated preoperative fractionated metanephrines or catecholamines were documented in 23 patients (68%); these normalized in 13 of 23 patients (56%) postoperatively-with symptom relief in 15 of 18 preoperatively symptomatic patients (79%). Among 23 patients with hormone-producing tumors, significant reduction in number of antihypertensive medications was also noted postoperatively; 11 patients have remained off all antihypertensives, 6 required 1 medication, 1 required 2, while 5 required full blockade with phenoxybenzamine and a beta-adrenergic blocker.
Surgery plays a significant role in the management of selected malignant PPGL. Resection can be effective in normalizing or significantly reducing levels of catecholamines and metanephrines, and can improve hormone-related symptoms and hypertension. Surgical resection, either complete or incomplete, is associated with durable survival despite a high rate of tumor recurrence.
嗜铬细胞瘤和副神经节瘤（PPGL）是罕见肿瘤；约10%为恶性。关于手术切除可能带来的益处的文献极为有限。
对1994年至2014年6月在梅奥诊所罗切斯特院区接受恶性PPGL手术的所有患者进行了为期20年的回顾。
我们确定了34例接受恶性PPGL手术的患者。中位随访时间为6年，5年生存率为90%（中位11年）。14例患者（41%）实现了完整切除（R0）。R0切除患者的中位无病生存期为4.6年（最长12年）。末次随访时仅有8例患者（23%）无疾病。23例患者（68%）术前记录有分馏后肾上腺素或儿茶酚胺升高；其中23例患者中的13例（56%）术后恢复正常，18例术前有症状的患者中有15例（79%）症状缓解。在23例产生激素的肿瘤患者中，术后还注意到抗高血压药物数量显著减少；11例患者停用了所有抗高血压药物，6例需要1种药物，1例需要2种，而5例需要用苯氧苄胺和β-肾上腺素能阻滞剂进行全面阻断。
手术在部分恶性PPGL的治疗中发挥着重要作用。切除可有效使儿茶酚胺和肾上腺素水平恢复正常或显著降低，并可改善激素相关症状和高血压。尽管肿瘤复发率高，但完整或不完整的手术切除均与持久生存相关。
