Rodríguez García J, Lancina Martín A, Gómez Veiga F, Chantada Abal V, Picallo Sánchez J, González Martín M
Servicio de Urología, Hospital Juan Canalejo, La Coruña.
Actas Urol Esp. 1992 Jan;16(1):5-16.
Presentation of a series of 14 cases of neural crest derived tumours located in the retroperitoneal space in adult patients (five pheochromocytoma, six paraganglioma, two ganglioneuroma, and one neuroblastoma), and review and update of the diagnostic and therapeutic aspects. All pheochromocytoma cases presented high BP and the classic triad of sudation, tachycardia and headaches, as well as high levels of blood and urine catecholamines and/or their metabolites. CAT, ultrasound scanning and 123MIBG were the main diagnostic techniques used. All four paraganglioma were functioning and generally located surrounding both kidneys (one case was paired). No malignancy was found in any of the 11 tumours while controls remain with normal BP and normal levels of urine catecholamine metabolites. None of the two ganglioneuromas showed specific signs and symptoms but were diagnosed accidentally. The one neuroblastoma was juxtavesical showing a highly unfavourable evolution in spite of radical surgery, radiotherapy and multiple chemotherapy and the patient died within 16 months with local recurrence and haematogenous dissemination to bones and lungs.
介绍14例成年患者腹膜后间隙神经嵴源性肿瘤（5例嗜铬细胞瘤、6例副神经节瘤、2例神经节神经瘤和1例神经母细胞瘤），并对诊断和治疗方面进行回顾与更新。所有嗜铬细胞瘤病例均表现为高血压以及出汗、心动过速和头痛的典型三联征，同时血、尿儿茶酚胺和/或其代谢产物水平升高。CT、超声扫描和123MIBG是主要的诊断技术。所有4例副神经节瘤均有功能，通常位于双肾周围（1例为双侧）。11例肿瘤中均未发现恶性肿瘤，而对照组血压和尿儿茶酚胺代谢产物水平均正常。2例神经节神经瘤均未表现出特定的体征和症状，而是偶然被诊断出来。1例神经母细胞瘤位于膀胱旁，尽管接受了根治性手术、放疗和多次化疗，但病情进展极为不利，患者在16个月内死于局部复发以及血行播散至骨骼和肺部。
