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[塞尔维亚贝尔格莱德临床中心内分泌研究所嗜铬细胞瘤的外科治疗结果]

[Results of surgical treatment of pheochromocytoma at the Institute of Endocrinology of the Clinical Center of Serbia in Belgrade].

作者信息

Janković Radovan, Diklić Aleksandar, Paunović Ivan, Krgović Ksenija, Hivaljević Vladan, Todorović-Kazić Milena, Havelka Marija, Tatić Svetislav, Dimitrijević Nevena

机构信息

Centre of Endocrine Surgery, Institute of Endocrinolgy, Clinical Centre of Serbia, Belgrade.

出版信息

Srp Arh Celok Lek. 2002 Jul;130 Suppl 2:38-42.

Abstract

Pheochromocytoma is a rare tumour that synthesis extensive amounts of cathecholamines. It is usually called a "ten percent" tumour, as it is inherited in nearly 10% of cases, extra-adrenal in 10%, bilateral, multicentric and malignant in also nearly 10% of cases. Nowadays, surgery of pheochromocytoma is very successful as a result of well equipped laboratory techniques, radiological diagnosis and pharmacological drugs in preoperative therapy and control examinations during surgery. Although the operative technique and anaesthesia are delicate, operative risk is reduced to minimum, and definite recovery can be achieved in almost all benign diagnosed pheochromocytomas. Unrecognized pheochromocytomas remain the main problem, often ending with sudden death because of cerebrovascular complications or myocardial infarction, and are diagnosed during autopsy. The aim of the study is to underline the principal characteristics of pheochromocytomas and evaluate the facts that influence the diagnosis and outcome of the treatment. Medical records of the patients operated on for pheochromocytoma in our Institution are analysed. From 1981 to 2001, 102 patients underwent surgery for pheochromocytoma, 71 females and 31 males, average age 42 years (range 9 to 71). Laboratory confirmation was based on 24-hour urine cathecholamines, and in 12 patients only plasma cathecholamines were determined. In imaging studies, we used ultrasound (US), computerised tomography (CT) and nuclear magnetic resonance (NMR). If there was a suspicion of inherited or extra adrenal form of a tumour, we also used Metaiodbenzilguanidine scan marked with radioactive I131 (I131-MIBG). Diagnostic confirmation of tumours localisation was completely reliable. In 89 patients adrenal pheochromocytoma was found (12 bilateral, 2 with paraganglioma). In 13 patients, pheochromocytoma was extra-adrenal. Inheritated character was registered in 18 patients (MEN2 in 16, NF-1 in 2). Malignant pheohromocytoma was found in 11 patients, in 4 of 13 with extra-adrenal tumours, and in 7 of 89 with adrenal tumours. We prepared all patients with diagnosed pheochromocytoma for operation with phenoxybenzamine in daily doses of 20 to 90 mg, no matter whether they were normotensive or with hypertension. Two days before operation, we included propranolol in the therapy in doses of 20 mg twice or three times a day. Surgery was done in neuroleptic anaesthesia. We performed 100 primary operations and 9 reoperations (in 2 patients, the first operation was carried out in another institution), and extracted 123 tumours. In 2 patients, during reoperation we extracted the opposite adrenal tumour, in one recurrent benign tumour, and in the others, reoperation was done because of recurrent malignant pheochromocytoma. Hypertension was completely corrected in 79% of operated patients, reduced in 13%, and in 7% there was no effect on hypertension We observed the following operative complications: peritonitis due to a small bowel lesion (1), infection and wound dehiscence (1), pleural effusion (1), pneumothorax (1), gastrointestinal haemorrhage (1), haemathoma (1), temporary hemiparesis (1) and seroma of the wound (1). There was no operative mortality.

摘要

嗜铬细胞瘤是一种罕见的肿瘤,可合成大量儿茶酚胺。它通常被称为“百分之十”肿瘤,因为在近10%的病例中为遗传性,10%为肾上腺外肿瘤,近10%的病例为双侧、多中心和恶性肿瘤。如今,由于实验室技术完善、放射诊断以及术前治疗和手术中控制检查所使用的药物,嗜铬细胞瘤手术非常成功。尽管手术技术和麻醉较为精细,但手术风险已降至最低,几乎所有诊断为良性的嗜铬细胞瘤都能实现确切康复。未被识别的嗜铬细胞瘤仍然是主要问题,常常因脑血管并发症或心肌梗死导致猝死,并在尸检时被诊断出来。本研究的目的是强调嗜铬细胞瘤的主要特征,并评估影响诊断和治疗结果的因素。我们分析了在我们机构接受嗜铬细胞瘤手术患者的病历。1981年至2001年,102例患者接受了嗜铬细胞瘤手术,其中女性71例,男性31例,平均年龄42岁(9至71岁)。实验室确诊基于24小时尿儿茶酚胺,12例患者仅测定了血浆儿茶酚胺。在影像学检查中,我们使用了超声(US)、计算机断层扫描(CT)和核磁共振(NMR)。如果怀疑肿瘤为遗传性或肾上腺外形式,我们还使用了用放射性I131标记的间碘苄胍扫描(I131-MIBG)。肿瘤定位的诊断确认完全可靠。89例患者发现肾上腺嗜铬细胞瘤(12例双侧,2例伴有副神经节瘤)。13例患者为肾上腺外嗜铬细胞瘤。18例患者有遗传特征(16例为MEN2,2例为NF-1)。11例患者发现恶性嗜铬细胞瘤,13例肾上腺外肿瘤中有4例,89例肾上腺肿瘤中有7例。无论患者血压正常还是患有高血压,我们都用苯氧苄胺为所有诊断为嗜铬细胞瘤的患者准备手术,每日剂量为20至90毫克。手术前两天,我们将普萘洛尔纳入治疗,剂量为每日20毫克,每日两次或三次。手术在神经安定麻醉下进行。我们进行了100例初次手术和9例再次手术(2例患者的首次手术在其他机构进行),共摘除123个肿瘤。2例患者在再次手术时摘除了对侧肾上腺肿瘤,1例为复发性良性肿瘤,其他患者因复发性恶性嗜铬细胞瘤进行了再次手术。79%的手术患者高血压得到完全纠正,13%有所减轻,7%对高血压无影响。我们观察到以下手术并发症:小肠损伤导致的腹膜炎(1例)、感染和伤口裂开(1例)、胸腔积液(1例)、气胸(1例)、胃肠道出血(1例)、血肿(1例)、暂时性偏瘫(1例)和伤口血清肿(1例)。无手术死亡病例。

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