Department of Nuclear Medicine and Endocrine Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland.
The Oncologic and Reconstructive Surgery Clinic, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland.
Endokrynol Pol. 2020;71(6):518-523. doi: 10.5603/EP.a2020.0075. Epub 2020 Oct 30.
Pheochromocytomas in hereditary syndromes tend to grow multifocal with adrenal involvement on both sides. Surgical treatment with bilateral adrenalectomy inevitably leads to life-long hormonal dependence, which significantly affects quality of life. The development of minimally invasive adrenal surgery has created a chance to preserve adrenal cortex function in these patients. The aim of the present study was to evaluate the safety of laparoscopic cortical-sparing adrenal surgeries and their efficacy in the prevention of postoperative adrenal insufficiency in patients with hereditary pheochromocytomas.
We retrospectively analysed the medical histories of 10 patients, who underwent 10 laparoscopic cortical sparing adrenal surgeries from January 2015 to January 2019 in our centre. The decision to perform sparing surgery was based on preoperative diagnosis of hereditary syndrome in line with the result of DNA analysis or its diagnosis based on the clinical appearance. All surgeries were performed laparoscopically from transperitoneal access in the lateral decubitus position, with preserving 1/3-1/4 adrenal tissue. The sufficiency of remnant adrenal tissue was assessed in all patients. The median time of follow-up was three years (ranged 0.5-4 years).
No intraoperative complications were observed. One case of acute heart failure was the only early postoperative adverse event. There were no late postoperative complications and no local recurrences observed. In one out of three patients undergoing sparing surgery as a second procedure after former total adrenalectomy, adrenal cortex failure occurred. In all patients after unilateral surgery or after bilateral surgery performed simultaneously (total adrenalectomy at one side and sparing surgery contralaterally), function of remnant adrenal tissue was preserved.
In hereditary pheochromocytomas, with minimal risk of malignant process, laparoscopic cortical sparing adrenal surgeries are the safe approach and provide the chance to preserve adrenal cortex function.
遗传性综合征相关的嗜铬细胞瘤往往为双侧肾上腺多发病灶，且常伴有肾上腺外侵犯。双侧肾上腺切除术不可避免地导致患者终生依赖激素，严重影响生活质量。微创肾上腺手术的发展为这些患者保留肾上腺皮质功能创造了机会。本研究旨在评估腹腔镜保留皮质肾上腺手术的安全性及其在预防遗传性嗜铬细胞瘤患者术后肾上腺功能不全方面的疗效。
我们回顾性分析了 2015 年 1 月至 2019 年 1 月在我中心接受 10 例腹腔镜保留皮质肾上腺手术的 10 例患者的病历。保留手术的决定是基于术前诊断为遗传性综合征，符合 DNA 分析结果，或基于临床表现进行的诊断。所有手术均采用经侧卧位经腹腔入路的腹腔镜进行，保留 1/3-1/4 的肾上腺组织。所有患者均评估残留肾上腺组织的功能。中位随访时间为 3 年（0.5-4 年）。
术中无并发症。仅 1 例发生急性心力衰竭，为唯一的早期术后不良事件。无晚期术后并发症和局部复发。在 3 例接受保留手术的患者中，有 1 例在先前的全肾上腺切除术之后再次发生肾上腺皮质功能衰竭。在单侧手术或同期双侧手术（一侧全肾上腺切除术和对侧保留手术）的所有患者中，残留肾上腺组织的功能均得以保留。
在遗传性嗜铬细胞瘤中，恶性过程的风险最小，腹腔镜保留皮质肾上腺手术是一种安全的方法，可以保留肾上腺皮质功能。
