State University of New York Upstate Medical Center, Syracuse, NY; Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Urol Oncol. 2021 Feb;39(2):134.e1-134.e8. doi: 10.1016/j.urolonc.2020.11.019. Epub 2020 Dec 8.
Patients with a confirmed germline mutation in the von Hippel-Lindau (VHL) tumor suppressor gene have been followed at the National Cancer Institute since the 1980s. In this study, we identify VHL patients with pheochromocytoma and long-term follow-up to determine the best candidates for active surveillance and surgical resection.
A prospectively collected database of patients with a confirmed germline VHL mutation was reviewed to identify patients with a history of pheochromocytoma and at least 10 years of follow up. The presence of symptoms was assessed at the time of resection. Imaging data obtained at each clinic visit was reviewed to evaluate mass size and annual growth rate. Catecholamine data were reviewed to evaluate for data above the upper limit of the reference range. Masses that underwent imaging at least 3 months apart were considered in our surveillance cohort.
Median follow up was 16.7 years. There was a size-dependent increase in catecholamine production (P<0.05). For 36 masses on active surveillance, growth rate increased exponentially from 0.03 cm/y when masses were <1 cm to 0.32 cm/y when masses were greater than 2 cm. Approximately 1/3 of patients developed another pheochromocytoma after initial resection with a median time of 7.9 years. Partial adrenalectomy was associated with no metastatic events and a steroid-free rate of 97%.
Active surveillance is a safe strategy for management of VHL associated pheochromocytoma in masses less than 2 cm.
自 20 世纪 80 年代以来，患有 von Hippel-Lindau（VHL）肿瘤抑制基因种系突变的患者一直在国家癌症研究所接受随访。在这项研究中，我们确定了患有嗜铬细胞瘤和长期随访的 VHL 患者，以确定最适合主动监测和手术切除的患者。
回顾性收集了一组经证实存在 VHL 种系突变的患者的前瞻性数据库，以确定有嗜铬细胞瘤病史且随访时间至少 10 年的患者。在切除时评估症状的存在。回顾每次就诊获得的影像学数据，以评估肿块大小和年增长率。评估儿茶酚胺数据是否超过参考范围的上限。将至少相隔 3 个月进行影像学检查的肿块纳入我们的监测队列。
中位随访时间为 16.7 年。儿茶酚胺的产生呈与肿块大小相关的增加（P<0.05）。在主动监测的 36 个肿块中，当肿块<1cm 时，生长速度呈指数增长，从 0.03cm/y 增加到肿块>2cm 时的 0.32cm/y。大约 1/3 的患者在初次切除后又发生了另一个嗜铬细胞瘤，中位时间为 7.9 年。部分肾上腺切除术与无转移事件相关，且类固醇使用率为 97%。
对于体积小于 2cm 的 VHL 相关嗜铬细胞瘤，主动监测是一种安全的管理策略。
