Diagnosis and treatment of renal angiomyolipoma

A series of nine patients surgically treated for histologically verified renal angiomyolipoma is presented. Only one patient had extrarenal stigmata of tuberous sclerosis. No evidence of malignancy was found. In three cases a preoperative diagnosis was achieved with computed tomography (CT), use of which is recommended in evaluation of renal tumours. Six angiomyolipomas were solitary and three bilateral. Abdominal or flank pain was present in eight cases and perirenal haemorrhage in four. Solitary tumours were treated with nephrectomy. For bilateral tumour, nephrectomy and conservative renal surgery were used in two cases and bilateral nephrectomy and kidney transplantation were performed in the third case. When adipose tissue containing renal tumour is demonstrated at CT, conservative surgery should be considered.     

肾错构瘤破裂出血的急诊处理

目的：探讨肾错构瘤(RAML)破裂出血的诊治方法。方法：回顾性分析11例RAML破裂出血的急诊处理过程。结果：11例患者均经病理明确诊断，4例行患肾切除术，2例行肾部分切除术，5例行肿瘤切除术。11例患者均恢复良好。结论：B超和CT检查为术前确诊主要依据；对RAML破裂出血应采取积极的治疗态度；手术治疗仍为主要治疗方法。     

肾血管平滑肌脂肪瘤21例诊治分析

目的 提高肾血管平滑肌脂肪瘤的诊断与治疗水平。方法 回顾性分析21例肾血管平滑肌脂肪瘤患的临床资料并结合献讨论其诊断和治疗。结果 18例行手术治疗，术后病理证实为肾血管平滑肌脂肪瘤，定期观察3例，随访1～2年无复发、恶变。结论 B超、CT是诊断本病的主要检查方法，手术治疗的原则是尽可能的保留正常肾脏组织。     

Diagnosis and management of renal angiomyolipoma

Renal angiomyolipoma (hamartoma) is an uncommon benign tumor of the kidney. Although about 400 cases of renal angiomyolipoma have been reported, it still causes difficulties in diagnosis and treatment. Until a few years ago it was almost impossible to arrive at a correct diagnosis preoperatively, and most of the cases underwent nephrectomy with the wrong diagnosis of renal carcinoma. More recently, abdominal computerized tomography and renal sonography have made it possible to reach the correct preoperative diagnosis in many cases, and thus to avoid unnecessary nephrectomies. We report on our experience with 13 cases of renal angiomyolipoma, and our conservative approach in a number of cases, which has enabled us to preserve renal tissue and function.     

肾血管平滑肌脂肪瘤18例报告

目的 探讨肾血管平滑肌脂肪瘤的诊断及治疗。方法 回顾性分析近10年来收治的肾血管平滑肌脂肪瘤18例的临床资料。结果 18例术前行B超检查．16例行CT检查。手术治疗16例．其中行肾切除术2例．肿瘤剜除术14例，术后均病理检查证实；定期随访2例．随访2～5年，无肿瘤恶变及复发。结论 对肾血管平滑肌脂肪瘤直径大于4cm．可手术治疗；对肿瘤直径小于4cm，可观察。     

Diagnosis and treatment of renal angiomyolipoma (based on 15 cases)

Fifteen cases of renal angiomyolipoma not associated with tuberous sclerosis are presented. The clinical and radiological features, the pathology and the management of these rare but well-documented tumors are discussed. They pose problems in diagnosis, and controversy still exists regarding their management. Additional techniques such as ultrasonography and computerized tomography may help in the diagnosis. When conservative surgery is not possible, or when a solid tumor cannot be confidently excluded, nephrectomy should be performed.     

肾血管平滑肌脂肪瘤的诊断与治疗（附32例报告）

目的：提高肾血管平滑肌脂肪瘤的诊断与治疗水平。方法：总结肾血管平滑肌脂肪瘤32例的诊断及治疗经验,根据肿瘤直径分为：小（＜4cm)、中（4－8cm)、大（＞8cm）三类,同时根据病变大小及症状分别采取观察、栓塞、肿瘤切除、肾部分切除及紧切除术。结果：B超诊断准确率为78．1％（25／32）,CT诊断准确率为87．1％（28／32）。对病变小、无症状者（5／32）采用定期观察,中等大小者（24／32）采用肿瘤切除（13／32）、肾部分切除（9／32）和肾切除（2／32）;病变大者（3／32）则采用肾切除术。32例患者全部获随访,均生存。结论：结合B超、CT以及HMB－45、HMB－50、CD63和细针穿刺细胞学检查明确诊断,根据病变大小和症状分别选择观察、栓塞、肿瘤切除、肾部分切除及肾切除术,治疗肾血管平滑肌脂肪瘤效果良好。     

肝血管平滑肌脂肪瘤的诊断与治疗

目的总结肝血管平滑肌脂肪瘤（HAML）的诊断与治疗。方法回顾性分析1990年1月至2010年12月安徽医科大学第一附属医院收治的15例HAML患者的临床资料。15例患者进行血清AFP、CEA、CAl9．9等常见消化系统肿瘤标志物指标检查,根据临床特征、影像学检查（超声、CT、MRI及血管造影检查）结果选择手术方式。术后进行病理学检查。采用门诊及电话随访,随访时间截至2011年6月。结果15例患者中5例患者无临床症状,所有患者无肝炎病史。术前超声、CT、MRI及血管造影检查诊断准确率分别为3／15、3／13、2／6和1／3。肿瘤位于肝左叶者4例、肝右叶者11例,肿瘤均为单发。15例患者均行手术治疗,其中13例行肝肿瘤剜除及周围部分正常肝组织切除术,2例因肿瘤直径较大行左半肝切除术。术后免疫组织化学染色检查结果示HMB45、CD34和阳一67表达均为阳性。15例患者术后痊愈出院,13例患者获得随访,随访时间6个月至9．8年,中位随访时间3．2年。11例获得完整随访,另2例患者术后分别随访9．8年和6年后失访。所有患者随访期间均生存,无肿瘤复发及转移。结论HAML无特异性临床症状,影像学检查确诊率不高,病理标本免疫组织化学染色检查HMB45阳性是特征性诊断依据,手术切除是目前治疗HAML的首选方法,患者预后较好。     

肝血管平滑肌脂肪瘤的诊断及治疗

目的探讨肝血管平滑肌脂肪瘤的临床、影像及病理特征,以提高诊断率。方法对1994-2009年间我院及东方肝胆外科医院手术后病理学检查证实的8例HAML患者的临床资料进行回顾性分析。结果临床无明显症状者5例,腹部不适者2例,腹部包块1例;肿瘤最大直径8.5 cm,位于肝左叶3例,右叶5例。B超检查8例,显示肿块边界清楚,强回声或混合回声光团5例,中等回声1例,低回声2例。肝CT平扫8例,占位呈低密度6例,混杂密度2例;4例增强扫描动脉期肿块明显强化,门脉期及延迟期持续强化。4例行MRI检查T1加权高信号3例、低信号1例;T2加权高信号3例,提示有脂肪成分者1例。8例均手术切除,术后送病理镜下示:主要由畸形血管、平滑肌样细胞和成熟的脂肪细胞3种成分以不同比例组成。免疫组织化学染色,HMB-45均呈阳性表达。结论肝血管平滑肌脂肪瘤临床缺少特异表现,容易误诊,综合分析超声、CT、MRI等影像特征有助于提高术前诊断率,免疫组化HMB-45阳性表达有助于病理确定诊断。     

肝脏血管平滑肌脂肪瘤的诊断与治疗

目的 总结肝脏血管平滑肌脂肪瘤的临床、影像及病理特征,探讨提高术前确诊率的途径.方法 回顾性分析2000年至2007年第二军医大学东方肝胆外科医院收治并经病理检查证实的73例肝脏血管平滑肌脂肪瘤患者的影像学特点及诊治经验.根据影像学特点对患者进行分型并进行相应治疗.结果 术前确诊7例.B超检查诊断率为0,CT检查诊断率为13%(7/56),MRI检查诊断率为6%(2/33).根据影像学检查结果分型:血管瘤型6例,脂肪瘤型17例,平滑肌瘤型4例,混合型46例.手术切除72例,RFA治疗1例.24例患者出现肺部感染、胸腔和腹腔积液、轻度肝功能损害.术后免疫组织化学检测阳性率依次为:HMB45＞平滑肌肌动蛋白＞波形蛋白＞增殖细胞核抗原＞CD34＞癌胚抗原蛋白＞CD18＞CD19＞p53.1例术后因复发死亡.结论 肝且庄血管平滑肌脂肪瘤易误诊,影像学分型有助于术前正确诊断,治疗上应积极手术切除.     

Diagnosis and treatment of hepatic angiomyolipoma

Background

Hepatic angiomyolipoma (HAML) is a rare liver tumor. This paper summarized the clinical, radiological and pathological features of HAML.

Methods

Seventeen cases of HAML were analyzed retrospectively. All patients were subjected to surgical resection of tumor, one of which was performed emergency surgery because of hemorrhage of tumor.

Results

There are 13 females and 4 males, most of whom were asymptotic except 4 had minimal abdominal discomfort. US, CT and/or MRI were taken and corresponding data was comprehensively analyzed with other clinical signs and symptoms. Correct preoperative diagnosis was able to be achieved in 9 patients. Pathological analysis and immunohistochemistry of HMB-45 was used as final diagnosis. All patients were followed up and survived without recurrence.

Conclusions

Preoperative diagnosis of HAML can be benefited from comprehensive analysis of clinical manifestations. The malignant potential and fast growth of tumor suggested surgical removal of tumor while it was diagnosed.Key Words: Hepatic tumor, angiomyolipoma, diagnosis, surgery     

肝脏血管平滑肌脂肪瘤的术前诊断与外科治疗

目的 探讨肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)的临床特点、诊断与外科治疗.方法 回顾性分析中国医学科学院肿瘤医院病理证实的14例肝血管平滑肌脂肪瘤的临床病理资料并进行随访.结果 14例肝血管平滑肌脂肪瘤病人无特异性临床表现,肿瘤均为单发.血清学AFP检查正常,影像学检查无特征性表现.14例均获手术切除,手术疗效好,术后随访无复发.结论 肝血管平滑肌脂肪瘤的部分病例诊断依赖实验室和影像学检查,术前可以作出较准确的诊断,手术治疗既可取得满意疗效,还可验证术前诊断是否正确.     

肾错构瘤破裂出血的诊断及外科治疗(附9例报告)

目的提高肾错构瘤破裂出血的诊断和治疗水平。方法回顾性分析9例肾错构瘤破裂出血的诊治过程。结果9例患者均行外科手术治疗,其中3例行患肾切除术,2例行肾部分切除术,4例行肿瘤切除术。术后病理诊断均为肾错构瘤,无恶变。9例患者均恢复顺利,5例患者获随访,时间2个月~14年,平均91.6个月,无肿瘤复发。结论肾错构瘤破裂出血在临床上无典型临床表现,B超和CT检查为术前确诊主要依据,尽早手术探查是首选治疗手段,术中尽可能选择保肾手术。     

肾错构瘤自发性破裂出血的外科诊治(附5例报告)

目的:探讨肾错构瘤破裂出血的诊断和治疗方法。方法:回顾性分析2003年7月～2007年3月收治的5例肾错构瘤破裂出血患者的临床资料,4例急诊行手术治疗,1例行保守治疗后1周行手术治疗。手术方式均选择患肾切除术。结果:5例术后恢复良好,术后病理检查报告为肾错构瘤,无恶变。结论:B超和CT检查对诊断肾错构瘤破裂出血有帮助。肾错构瘤的治疗应全面考虑,手术仍为主要的治疗方法。     

肾错构瘤117例的诊断与治疗

目的探讨肾错构瘤（RAML）准确的诊断方法及选择最佳治疗方法。方法回顾性分析手术治疗RAML患者117例,其中男性24例（24/117,20.51%）,女性93例（93/117,79.49%）,术前应用彩超、CT、IVU或CTU、MRI等检查明确诊断,根据病情采用保留肾单位肾肿瘤剜除术（NSS）或肾切除术,术后定期随访,复查彩超、CT、IVU。结果术前确诊为RAML的病例64例（64/117,54.7%）,术前误诊为肾癌患者21例（21/117,17.94%）。根据病情98例（98/117,83.76%）采用NSS,19例（19/117,16.24%）行肾切除术。手术时间（92.5±3.1）min（40～180min）,NSS肾蒂阻断时间（15.5±0.7）min（3～35min）,失血量（129±4.2）mL（40～800mL）,肿瘤直径（4.8±0.9）cm（0.7～25cm）。117例病例中肿瘤直径≥4cm的64例,其中行NSS手术的45例,手术时间（99.4±2.8）min（65～180min）,出血（142.3±4.7）mL（50～800mL）,肾蒂阻断时间（17.4±1.2）min（9～35min）。肿瘤直径〈4cm的53例,均行NSS治疗,手术时间（86.8±2.8）min（40～150min）,出血（117.7±3.9）mL（40～300mL）,肾蒂阻断时间（13.9±1.4）min（3～20min）。肿瘤直径〈4cm的较肿瘤直径≥4cm的患者手术时间短,肾蒂阻断时间短,出血量少,患者术后恢复快,最大限度保留了肾脏的功能。无死亡病例,NSS术后1例（1/117,1.27%）漏尿并继发感染,二次手术行肾切除,另1例随访8年后复发,二次行NSS治疗。所有患者均术后3～6个月复诊,行CT及IVU检查,肾脏排泄功能基本正常。结论彩超、CT在RAML诊断及鉴别诊断、术后随访中起到了重要的作用,NSS是治疗RAML安全而有效的方法,对于肿瘤直径〉2cm的RAML我们建议积极行手术治疗。     

24例肾血管平滑肌脂肪瘤诊治分析

目的提高肾血管平滑肌脂肪瘤的诊治效果.方法回顾性总结分析24例肾血管平滑肌脂肪瘤患者的临床资料.结果3例(12.5%)术前误诊.诊断正确率B超为52.6%(10/19),CT为75%(9/12),MRI为80%(12/15),B超联合CT为90%(9/10).治疗以保留肾单位的肿瘤局部剜除术为主.结论应提高对肾血管平滑肌脂肪瘤的术前诊断水平,治疗中尽量保留肾单位.     

5例肝血管平滑肌脂肪瘤的诊断与治疗分析

目的 探讨肝血管平滑肌脂肪瘤（HAML）的临床、影像学、病理学特点及治疗方法。方法 回顾性分析我院2016年1月至2020年10月间收治的5例肝血管平滑肌脂肪瘤病例,并结合相关文献对本病进行探讨。结果 5例肝血管平滑肌脂肪瘤病人均经外科手术治疗,预后良好,所有病人术后随访期间,未见肿瘤复发及转移,无死亡病例。结论 肝血管平滑肌脂肪瘤临床罕见,常缺乏特征性临床表现,早期诊断困难。明确诊断有赖于术后病理及免疫组化结果。治疗方案首选手术治疗,其疗效确切,预后良好。     

Simple enucleation for the treatment of renal angiomyolipoma

OBJECTIVES: To report on the role of simple enucleation for treating renal angiomyolipoma (AML) in a series of patients treated in our department. PATIENTS AND METHODS: We retrospectively reviewed the data of all 37 patients with a histopathological diagnosis of renal AML who had either radical nephrectomy (three) or nephron-sparing surgery by simple enucleation (34) between January 1986 and December 2005. Indications for intervention included either symptomatic AML or a tumour of >4 cm, regardless to the presence of symptoms or renal masses suspicious of malignancy. The patients' status was evaluated last in October 2006. RESULTS: The mean (sd, median, range) pathological tumour size was 5.2 (3.4, 4.8, 1.5-15) cm; five patients (15%) were affected by tuberous sclerosis. Simple enucleation was successful in all patients but in three (9%) a sharp dissection a few millimetres from the tumour was used during critical steps of the procedure where it seemed difficult to define the right plane of enucleation. Warm ischaemia was used in 79% of patients, with a mean ischaemic time of 11.2 min. Two patients (6%) required renal hypothermia. A simple parenchymal compression was used in five cases (15%). The mean (range) intraoperative blood loss was 170 (70-650) mL. None of the patients had postoperative bleeding requiring re-intervention but one (3%) required two units of blood after surgery. There were no major complications, e.g. prolonged acute tubular necrosis/chronic renal insufficiency and urinary leakage/urinoma, but two patients had urosepsis not associated with perirenal fluid collection and that required targeted antibiotic therapy. At a mean (median, range) follow-up of 56 (50.5, 10-120) months none of the patients had local tumour recurrence. Two patients had a small AML elsewhere in the operated kidney, detected 18 and 36 months after surgery, with a kidney recurrence rate of 6%. CONCLUSIONS: Our data confirm the optimum results of simple enucleation for renal AMLs; this technique provides excellent long-term local control and no patient had urinary leakage/fistula afterward.     

肾肿瘤剜除术治疗肾细胞癌、肾血管平滑肌脂肪瘤

目的探讨肾肿瘤剜除术治疗肾细胞癌及肾血管平滑肌脂肪瘤的疗效。方法回顾分析15例在我院进行肾肿瘤剜除术的肾细胞癌及肾血管平滑肌脂肪瘤患者的临床及病理资料。结果全部肾肿瘤均成功剜除,平均热缺血时间为15min,术中肿瘤剜除面平均出血25ml,术后无继发出血,无急性肾小管坏死、慢性肾功能不全及尿瘘等并发症发生。术后平均随访时间为2．5年,均未见肿瘤复发或转移。依据2003AJCC肾癌分期方法,所有肾癌患者均为Tla期,组织学形态为透明细胞癌。病理分级按Fuhrman标准为G1。结论肾肿瘤剜除术对有假性包膜的Tla肾细胞癌和肾血管平滑肌脂肪瘤是有效和安全的,术后并发症少,可以最大程度地保留肾脏功能。     

肾血管平滑肌脂肪瘤的诊断与治疗(附72例报告)

目的　探讨肾血管平滑肌脂肪瘤的诊治方法。　方法　总结分析 72例肾血管平滑肌脂肪瘤患者的临床资料。统计学方法采用 χ2 检验。　结果　 13例 (18.1% )术前误诊。诊断正确率B超为 73.6 % (5 3/ 72 ) ,CT为 81.9% (5 9/ 72 ) ,两者同时误诊者 10例 (13.9% )。直径 <4cm的肿瘤误诊率高于≥ 4cm者 ,P <0 .0 5。治疗以保留肾单位的手术为主 ,术前选择性动脉栓塞及术中暂时阻断肾动脉可显著减少出血。　结论　应提高对肾血管平滑肌脂肪瘤的术前诊断水平 ,采取各种措施保留肾单位。