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偶然发现的肾上腺髓脂肪瘤。3例手术患者报告及文献综述

Incidentally discovered adrenal myelolipoma. Report on 3 operated patients and update of the literature.

作者信息

Porcaro Antonio Benito, Novella Giovanni, Ficarra Vincenzo, Cavalleri Stefano, Antoniolli Stefano Zecchini, Curti Pierpaolo

机构信息

Department of Urology, University Hospital, Verona, Italy.

出版信息

Arch Ital Urol Androl. 2002 Sep;74(3):146-51.

PMID:12416010
Abstract

INTRODUCTION AND OBJECTIVES

Adrenal myelolipomas are rare benign non functioning tumors which are mostly detected incidentally by imaging (8-15%). The aim of this study was to find out the clinical outcome of 3 patients operated for incidentally discovered adrenal myelolipoma as well as to update the literature concerning this topic.

PATIENTS AND METHODS

During a 24 year period (1976-2000) we operated on 28 of 39 (72%) patients for incidentally discovered adrenal mass and pathology assessed 3 myelolipomas (11%). All patients were males and average age was 54.3 years (range 46-66). Myelolipoma was associated with arterial hypertension in 2 patients and BPH in 1. Patients underwent unilateral adrenalectomy. Results Myelolipomas, all unilateral, were right-sided in 2 cases (67%) and left sided in 1. Average tumor diameter, as detected by CT scan, resulted 10.3 cm range (3-17). Endocrine investigation was negative. Average tumor size, as detected by pathology, resulted 9.3 cm (range 3-14) and mean weight 653 gr (range 210-980). Histopathology assessed primary adrenal myelolipoma in all cases. Mean follow-up is 110.6 months (range 102-128) and all patients are alive and disease free.

CONCLUSIONS

Ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) are effective in diagnosing adrenal myelolipomas in about 90% of cases. Fine needle aspiration (FNA) cytology under CT or US control has an important role in investigating as well as diagnosing undefined masses suspected of adrenal or extraadrenal myelolipoma thus avoiding unnecessary surgery. The increasing number of incidentally discovered adrenal myelolipomas arise the question of appropriate treatment modalities which range from watchful waiting to surgical removal. Small asymptomatic tumors sized less than 4 cm can be monitored expectantly. Tumors when symptomatic or larger than 4 cm should be removed since the risk of spontaneous rupture with retroperitoneal hemorrhage and life threatening shock. Bilateral adrenalectomy for large and symptomatic tumors implies lifelong substitution with hydrocortisone. Actually, laparoscopic adrenalectomy is the new gold standard in treating myelolipomas since both showing more effective postoperative recovery than open surgery as well as reducing the number of patients who undergo long and close follow up.

摘要

引言与目的

肾上腺髓质脂肪瘤是罕见的良性无功能肿瘤,大多通过影像学偶然发现(8%-15%)。本研究的目的是了解3例因偶然发现的肾上腺髓质脂肪瘤而接受手术的患者的临床结局,并更新有关该主题的文献。

患者与方法

在24年期间(1976 - 2000年),我们对39例因偶然发现肾上腺肿块而接受手术的患者中的28例(72%)进行了手术,病理检查发现3例髓质脂肪瘤(11%)。所有患者均为男性,平均年龄54.3岁(范围46 - 66岁)。2例髓质脂肪瘤患者合并动脉高血压,1例合并良性前列腺增生。患者均接受了单侧肾上腺切除术。结果髓质脂肪瘤均为单侧,2例(67%)位于右侧,1例位于左侧。CT扫描检测到的平均肿瘤直径为10.3 cm(范围3 - 17 cm)。内分泌检查结果为阴性。病理检测到的平均肿瘤大小为9.3 cm(范围3 - 14 cm),平均重量为653 g(范围210 - 980 g)。所有病例经组织病理学评估均为原发性肾上腺髓质脂肪瘤。平均随访时间为110.6个月(范围102 - 128个月),所有患者均存活且无疾病。

结论

超声(US)、计算机断层扫描(CT)和磁共振成像(MRI)在约90%的病例中对诊断肾上腺髓质脂肪瘤有效。在CT或US引导下的细针穿刺(FNA)细胞学检查在疑似肾上腺或肾上腺外髓质脂肪瘤的不明肿块的检查和诊断中具有重要作用,从而避免不必要的手术。偶然发现的肾上腺髓质脂肪瘤数量的增加引发了关于适当治疗方式的问题,治疗方式从密切观察到手术切除不等。小于4 cm的无症状小肿瘤可进行观察。有症状或大于4 cm的肿瘤应予以切除,因为存在自发破裂伴腹膜后出血和危及生命的休克的风险。对于大的有症状肿瘤进行双侧肾上腺切除术意味着终身用氢化可的松替代治疗。实际上,腹腔镜肾上腺切除术是治疗髓质脂肪瘤的新金标准,因为它不仅术后恢复比开放手术更有效,还减少了需要长期密切随访的患者数量。

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