巨型囊性嗜铬细胞瘤1例报道及文献复习

A case report of giant cystic pheochromocytoma and literature review

嗜铬细胞瘤和副神经节瘤是罕见的神经内分泌肿瘤,特征是儿茶酚胺过量释放,表现为头痛、心悸、大量出汗以及各种其他体征和症状的经典三联征。嗜铬细胞瘤的诊断需要依据儿茶酚胺过度释放,以及儿茶酚胺分泌肿瘤的解剖学定位。除非有手术禁忌证,否则手术是所有嗜铬细胞瘤患者的主要治疗方式。旨在消除儿茶酚胺分泌过多和肿瘤生长的风险。儿茶酚胺分泌过多应在手术前和手术期间通过药物治疗控制。术后主要并发症为低血压和反弹性低血糖,应严密随访24~48 h。而巨型囊性嗜铬细胞瘤是一种更为罕见的肿瘤,通常无症状,大多在影像学检查或术中偶然发现。本文介绍了1例59岁的男性巨型囊性嗜铬细胞瘤病例,该患者因反复胸痛至心内科行冠脉造影术术前检查中偶然发现右侧肾上腺巨大囊性占位,而后顺利进行腹膜后巨大肿物切除,病理提示嗜铬细胞瘤,预后良好。此外,本文还进行了文献复习,以了解囊性嗜铬细胞瘤的流行病学和表现,以便及时诊断和管理嗜铬细胞瘤。

Pheochromocytoma and paraganglioma are rare neuroendocrine tumors,which are characterized by excessive release of catecholamine and featured by a classic triad of headache,palpitation,profuse sweating and various other signs and symptoms.The diagnosis of pheochromocytoma requires both the excessive release of catecholamine and the anatomical localization of catecholamine-secreting tumor.Unless there are surgical contraindications,surgery is the main treatment for all patients with pheochromocytoma.The operation aims to eliminate the risk of excessive catecholamine secretion and tumor growth.Excessive catecholamine secretion shall be carefully controlled by medication before and during surgery.The main postoperative complications are hypotension and rebound hypoglycemia,which shall be closely followed up for 24-48 hours.Being a rarer tumor and usually asymptomatic,a giant cystic pheochromocytoma is often found incidentally during imaging examination or surgery.A 59-year-old male case with giant cystic pheochromocytoma is introduced in this paper.Because of repeated chest pain,the patient underwent the preoperative examination of coronary angiography in the Department of Cardiology,with a giant cystic tumor accidentally found in the right adrenal gland.Then the resection of the giant retroperitoneal tumor was successfully performed.Pathology indicated that pheochromocytoma had a good prognosis.In addition,the literature was also reviewed in this paper,so as to understand the epidemiology and manifestations of the cystic pheochromocytoma.So as to diagnose and manage pheochromocytoma in time.