Aygun Nurcihan, Uludag Mehmet
Department of General Surgery, University of Health Sciences Turkey, Sisli Hamidiye Etfal Teaching and Research Hospital, Istanbul, Turkey.
Sisli Etfal Hastan Tıp Bul. 2020 Dec 11;54(4):391-398. doi: 10.14744/SEMB.2020.58998. eCollection 2020.
Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. Postoperative major complications are hypotension and rebound hypoglycemia, and patients should be followed closely for 24-48 hours. The choice of surgical approach is determined based on multiple factors, including germline genetic test results, the size of the tumor, body mass index, surgeon's experience, and the likelihood of malignancy. Primary tumor resection does not completely eliminate the risk of tumor persistence and recurrence. Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location Combined and alfa;- and beta- adrenergic blockade is usually applied in patients with PPGL preoperatively. Some patients may present with pheochromocytoma multisystem crisis, which is a life-threatening condition that can involve cardiovascular, pulmonary, neurological, gastrointestinal, renal, hepatic and metabolic systems. Pheochromocytoma crisis may be spontaneous or may present with the tumor manipulation, trauma, corticosteroids, beta-blockers, anesthetic drugs, and the stimulation of non-adrenal surgical stress. These patients should be considered as medical emergencies rather than surgical emergencies. In this review, it was aimed to evaluate the pre-, per and post-operative management, curative and palliative surgical management, and postoperative outcomes and follow-up of the patients with PPGLs.
嗜铬细胞瘤（PCC）和副神经节瘤（PGL）是起源于肾上腺髓质、交感神经或副交感神经节嗜铬细胞的罕见神经内分泌肿瘤。目前，嗜铬细胞瘤/副神经节瘤（PPGL）唯一的治愈性治疗选择是手术切除。手术旨在消除分泌过多和肿瘤生长的风险。在手术前和手术期间，应通过药物治疗仔细控制分泌过多的后果。术后主要并发症是低血压和反弹性低血糖，患者应密切随访24至48小时。手术方式的选择取决于多种因素，包括种系基因检测结果、肿瘤大小、体重指数、外科医生的经验以及恶性可能性。原发性肿瘤切除并不能完全消除肿瘤持续存在和复发的风险。因此，所有接受手术治疗的PPGL患者都应随访至少10年，以监测疾病复发和新肿瘤形成。尽管手术切除是PPGL唯一的治愈性治疗方法，但除了转移性疾病中可切除的局部区域转移灶或孤立的远处转移灶外，手术治疗是姑息性的。姑息性治疗的目的是减少激素分泌，并预防关键解剖部位与转移相关的并发症。PPGL患者术前通常应用联合α和β肾上腺素能阻滞剂。一些患者可能出现嗜铬细胞瘤多系统危象，这是一种危及生命的情况，可累及心血管、肺、神经、胃肠、肾、肝和代谢系统。嗜铬细胞瘤危象可能是自发的，也可能在肿瘤操作、创伤、皮质类固醇、β受体阻滞剂、麻醉药物以及非肾上腺手术应激刺激时出现。这些患者应被视为内科急症而非外科急症。本综述旨在评估PPGL患者的术前、术中和术后管理、治愈性和姑息性手术管理以及术后结果和随访情况。
