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嗜铬细胞瘤——当前治疗观点

Phaeochromocytoma--views on current management.

作者信息

Williams D T, Dann S, Wheeler M H

机构信息

Department of Endocrine Surgery, University Hospital of Wales, Heath Park, CF14 4XW, Cardiff, UK.

出版信息

Eur J Surg Oncol. 2003 Aug;29(6):483-90. doi: 10.1016/s0748-7983(03)00071-4.

Abstract

AIMS

To evaluate the current investigation and management of phaeochromocytoma.

METHODS

Retrospective analysis of patients who underwent surgical excision of phaeochromocytoma in the Department of Endocrine Surgery at the University Hospital of Wales, Cardiff. Forty-seven patients (24 female and 23 males) were studied. Preoperative diagnosis was established by measurement of urinary catecholamines (HMMA, metadrenalines, and fractionated catecholamines). Tumour localisation was achieved by using ultrasound, CT, MRI and MIBG scintigraphy. Preoperative medical preparation and control of hypertension was achieved in the majority of cases by alpha adrenergic blockade with phenoxybenzamine and the beta blocker propranol. Surgery was performed by a variety of approaches which included laparotomy, posterior extraperitoneal and laparoscopic methods. All patients were followed up post-operatively in a surgical endocrine clinic.

RESULTS

Seventy percent of patients presented with hypertension but only 21.3% gave a history of paroxsmal hypertension. CT scanning and MRI proved to be the most sensitive localisation investigations. Excellent preoperative control of hypertension was achieved with alpha adrenergic blockade but induction of anaesthesia, rather than tumour handling was noted to be associated with most hypertensive surges of blood pressure. There was a zero 30 day post-operative mortality but 10 complications of surgery occurred in 8 patients (21.3%). Cure of hypertension was achieved in 80% of patients. Attempts to perform cortex sparing procedures in patients with familial disease and multiple tumours was not successful in the long term.

CONCLUSIONS

Surgical excision of phaeochromocytoma is a procedure, which can be performed with zero mortality and a low morbidity resulting in a high cure rate for hypertension. Adequate preoperative pharmacological control of hypertension is mandatory. Localisation techniques permit a focussed approach with increasing use of laparoscopy. Those patients with familial disease and those with multiple tumours pose particular management challenges. For an optimum and satisfactory outcome a planned multidisciplinary approach is required.

摘要

目的

评估嗜铬细胞瘤目前的检查与治疗情况。

方法

对在威尔士大学医院加的夫分校内分泌外科接受嗜铬细胞瘤手术切除的患者进行回顾性分析。共研究了47例患者(24例女性和23例男性)。通过测定尿儿茶酚胺(高香草酸、变肾上腺素和去甲肾上腺素)进行术前诊断。通过超声、CT、MRI和间碘苄胍闪烁扫描实现肿瘤定位。大多数病例通过使用苯苄胺进行α肾上腺素能阻滞和β受体阻滞剂普萘洛尔实现术前药物准备和高血压控制。手术采用多种方法,包括剖腹手术、后腹膜外手术和腹腔镜手术。所有患者术后均在外科内分泌门诊进行随访。

结果

70%的患者表现为高血压,但只有21.3%有阵发性高血压病史。CT扫描和MRI被证明是最敏感的定位检查方法。通过α肾上腺素能阻滞实现了对高血压的良好术前控制,但注意到麻醉诱导而非肿瘤操作与大多数高血压血压波动相关。术后30天死亡率为零,但8例患者(21.3%)出现了10例手术并发症。80%的患者实现了高血压的治愈。对患有家族性疾病和多发肿瘤的患者尝试进行保留肾上腺皮质手术从长期来看并不成功。

结论

嗜铬细胞瘤的手术切除是一种死亡率为零、发病率低且高血压治愈率高的手术。术前对高血压进行充分的药物控制是必不可少的。定位技术允许采用更有针对性的方法,腹腔镜手术的使用越来越多。患有家族性疾病和多发肿瘤的患者带来了特殊的管理挑战。为了获得最佳和满意的结果,需要采用有计划的多学科方法。

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