Urologic Oncology Branch, National Cancer Institute, Bethesda, MD.
Urologic Oncology Branch, National Cancer Institute, Bethesda, MD; Department of Urology, SUNY Upstate Medical University, Syracuse, NY.
Urology. 2020 Jun;140:85-90. doi: 10.1016/j.urology.2020.02.015. Epub 2020 Feb 26.
To evaluate the recurrence and functional outcomes in a primarily hereditary cohort of patients undergoing partial adrenalectomy for pheochromocytoma.
A retrospective review from a prospectively managed database of patients undergoing partial adrenalectomy from 1995 to 2018 at the National Cancer Institute was performed. Local recurrence was defined as imaging evidence of a recurrent or de novo lesion on the operative side. Steroid dependency was defined as requiring daily steroid replacement at time of last follow-up.
One hundred and twenty-four partial adrenalectomies, removing 162 tumors, were performed in 107 patients. Most patients had a known hereditary predisposition to develop bilateral, multifocal, and recurrent pheochromocytoma. Median tumor size was 2 cm (interquartile range (IQR) 1.5-2.8). Median follow-up was 60 months (IQR 13-131). Local recurrence occurred in 17 patients (15.8%) and were managed with active surveillance or surgery. A single patient (1/106, 0.9%) developed metastatic spread of pheochromocytoma approximately 14 years after his first of 2 partial adrenalectomies and remains alive under active surveillance. Median time to recurrence was 71 months (IQR 26-127) with 10 patients (9.3%) requiring daily steroid replacement at time of last follow-up.
Partial adrenalectomy offers excellent oncologic and functional outcomes, sparing most patients from lifelong steroid replacement therapy. Recurrences can be easily managed with repeat surgery or active surveillance via functional work-up and imaging. Partial adrenalectomy remains the recommended surgical management for patients pre-disposed to development of bilateral, multifocal and recurrent pheochromocytoma.
评估原发性遗传性患者行部分肾上腺切除术治疗嗜铬细胞瘤的复发和功能结局。
回顾性分析了 1995 年至 2018 年在国家癌症研究所行部分肾上腺切除术的前瞻性管理数据库中的患者资料。局部复发定义为手术侧有复发性或新发性病变的影像学证据。激素依赖性定义为最后一次随访时需要每日激素替代治疗。
107 例患者共行 124 次部分肾上腺切除术，切除 162 个肿瘤。大多数患者有双侧、多灶性和复发性嗜铬细胞瘤的遗传性倾向。肿瘤大小中位数为 2cm（四分位距 1.5-2.8）。中位随访时间为 60 个月（四分位距 13-131）。17 例（15.8%）患者发生局部复发，采用积极监测或手术治疗。1 例（1/106，0.9%）患者在首次行 2 次部分肾上腺切除术约 14 年后发生嗜铬细胞瘤转移，并在积极监测下仍存活。复发中位时间为 71 个月（四分位距 26-127），10 例（9.3%）患者在最后一次随访时需要每日激素替代治疗。
部分肾上腺切除术可提供良好的肿瘤学和功能结局，使大多数患者免于终身激素替代治疗。通过功能检查和影像学检查，可通过再次手术或积极监测轻松处理复发。对于有双侧、多灶性和复发性嗜铬细胞瘤发生倾向的患者，部分肾上腺切除术仍然是推荐的手术治疗方法。
