肾脏少脂肪血管平滑肌脂肪瘤患者21例临床分析并文献复习

目的提高对少脂性肾血管平滑肌脂肪瘤的认识,探讨其诊治方法。方法回顾性分析经术后病理证实的21例少脂性肾血管平滑肌脂肪瘤的临床特征及影像学表现。结果少脂性肾血管平滑肌脂肪瘤多见于年轻女性,超声显示肿瘤为低回声或等回声,彩色多普勒可见肿瘤内有血流信号。CT检查21例平扫期未见脂肪样低密度。其平扫CT值36~59 Hu。增强后CT值63~89 Hu。结论少脂性肾血管平滑肌脂肪瘤缺乏特异性的临床症状和体征,对病灶CT的细微分析能够给诊断带来帮助。应谨慎处理肾脏高密度病变以避免不必要的肾脏切除术。     

乏脂肪肾血管平滑肌脂肪瘤的CT特征分析（附40例报告）

目的通过分析40例术前诊断为肾癌的乏脂肪肾血管平滑肌脂肪瘤的CT影像学特征,以期提高肾脏肿瘤的诊断准确率,减少过度治疗。方法回顾性分析40例术前影像学诊断为肾癌的乏脂肪血管平滑肌脂肪瘤,评价CT平扫及增强后三期扫描中肿瘤的大小、位置、强化模式、强化程度、是否伴有钙化等。结果肿瘤最大径1.6～7.9（3.48±1.68）cm;38例表现为实性占位,2例表现为囊实性占位（BosniakⅣ型）;1例肿瘤伴有钙化;就强化程度而言,13例表现为轻度强化,18例表现为中度强化,9例表现为明显强化;就强化模式而言,28例表现为延迟强化,12例表现为非延迟强化;10例表现为不均匀强化,30例表现为均匀强化。结论 CT强化程度,强化模式及特殊征象在乏脂肪肾血管平滑肌脂肪瘤的诊断中具有一定价值。     

乏脂肪肾血管平滑肌脂肪瘤与肾透明细胞癌的CT鉴别诊断

目的探讨CT平扫及动态增强扫描在肾血管平滑肌脂肪瘤与肾透明细胞癌鉴别诊断中的临床应用价值。方法回顾性搜集CT误诊为肾癌的肾血管平滑肌脂肪瘤(AML)16例及肾透明细胞癌(ccRCC)30例患者的CT平扫及动态增强扫描资料,由2名影像医师分别独立评价肿瘤形态特征如有无钙化、假包膜、劈裂征、喙征;测量乏脂肪肾血管平滑肌脂肪瘤(fpAML)和(ccRCC)CT平扫及增强扫描各期的CT值、计算强化指数,进行独立样本t检验。结果平扫fpAML肿瘤CT值为(44±9)Hu,ccRCC平扫CT值为(31±4)Hu,差异有统计学意义(P0.05),增强扫描各期CT值两者间差异无统计学意义(P0.05)。fpAML与ccRCC皮质期、实质期、排泄期强化指数比较,差异有统计学意义(P0.05)。实质期fpAML75%强化较为均匀一致,而ccRCC组93.3%表现为强化不均匀。结论 fpAML平扫CT值明显高于ccRCC,增强扫描fpAML在皮质期、实质期和排泄期强化程度均低于ccRCC,实质期fpAML强化较为均匀一致,ccRCC强化不均匀。这三点是fpAML与ccRCC重要的CT鉴别诊断要点。     

MSCT对肾血管平滑肌脂肪瘤的诊断价值

目的：探讨多层螺旋CT（MSCT）在肾血管平滑肌脂肪瘤诊断中的价值。方法：对手术证实的38例肾血管平滑肌脂肪瘤的术前多层螺旋CT资料进行回顾性分析,并在工作站行MPR、MIP图像重建,分析肾血管平滑肌脂肪瘤的MSCT表现,并与手术病理结果对照。结果：38例肾血管平滑肌脂肪瘤中,CT诊断血管平滑肌脂肪瘤的病灶被手术病理证实正确诊断的有32例,另外6例被病理证实为上皮样血管平滑肌脂肪瘤的病灶,其中3例CT误诊为肾癌,后期的3例被正确诊断。总诊断准确率达92.1%（35/38）。32例的血管平滑肌脂肪瘤CT平扫呈等、低、高低混杂密度的,其中31例显示有脂肪密度,多期增强扫描该31例皮质期肿块呈均匀或不均匀强化,但低于肾皮髓质强化,脂肪、坏死囊变、出血区无强化,分泌期肿块持续强化;1例整个病灶显示为高密度出血灶,多期增强扫描无明显异常强化;6例上皮样血管平滑肌脂肪瘤CT平扫呈均匀等密度,多期增强扫描显示皮质期明显强化,分泌期强化减弱。MIP显示其中31例血管平滑肌脂肪瘤的肿内迂曲、增粗的血管,其中1例无明显增粗迂曲血管影;6例上皮样血管平滑肌脂肪瘤内显示细小血管。结论：多层螺旋CT及多期增强扫描能对术前正确诊断肾血管平滑肌脂肪瘤有重要价值,但对上皮样血管平滑肌脂肪瘤的诊断尚需结合临床和实验室检查,甚至需要穿刺活检。     

肾血管平滑肌脂肪瘤自发破裂出血的急诊处理(附24例报告)

目的 探讨肾血管平滑肌脂肪瘤自发破裂出血的急诊处理.方法 对24例肾血管平滑肌脂肪瘤自发破裂出血患者的临床资料及随访结果进行回顾性分析.术前均行B超和CT检查,22例诊断为肾血管平滑肌脂肪瘤自发破裂出血,2例未能排除肾癌出血.3例保守治疗,4例行选择性肾动脉栓塞术,17例急诊手术.结果 3例保守治疗患者中1例病情稳定,2例改行手术治疗.4例行肾动脉栓塞术患者栓塞成功,出血停止.4例行肾切除术,13例行保留肾单位手术,其中5例行后腹腔镜手术.术后病理诊断为肾血管平滑肌脂肪瘤并出血.随访3个月～6年,肿瘤无复发或转移.结论 B超和CT是诊断肾血管平滑肌脂肪瘤出血的重要手段.治疗可以选择保守治疗、肾动脉栓塞或者急诊手术.     

肾血管平滑肌脂肪瘤32例诊治分析

目的 提高肾血管平滑肌脂肪瘤的诊断符合率和治疗效果.方法 回顾性分析32例患者临床资料.结果 根据肿瘤的大小和病变程度,24例行肾错构瘤剜除术,5例行肾部分切除术,3例行肾切除术.结论 B超和CT为诊断肾血管平滑肌脂肪瘤的首选方法,治疗应以外科手术为主.肿瘤直径〈4 cm的患者多无临床症状,可临床随访 肿瘤直径〉4 cm的患者应积极行手术治疗,以保留肾单位的手术为首选.     

肾血管平滑肌脂肪瘤的诊治(附19例报道)

本文报道19例肾血管平滑肌脂肪瘤,13例有腰痛,2例有肉眼血尿症状,4例体检发现。19例均行B超检查,16例行CT检查。15例行手术治疗,其中13例行肾脏切除术,2例行肾部分切除手术;4例随访观察。认为B超、肾动脉造影、CT是肾血管平滑肌脂肪瘤的主要诊断方法,14例术前明确诊断。根据病人的全身情况、临床症状及肿瘤的大小、部位,决定手术方式及处理,多数采用肾切除术,少数采用肾部分切除术及随访观察治疗。     

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。     

《肾脏平滑肌脂肪瘤合并出血如何治疗?》诊疗建议

患者,男性,34岁,体力劳动后突发左腰痛.原文见<现代泌尿生殖肿瘤杂志>2011年第2期128页.从CT看,左肾占位(6 cm ×5 cm)伴患肾周围高密度影.建议测CT値.如为-20或以下,表明有脂肪成分.虽然极少数肾癌也含有脂肪成分,但同时伴有钙化灶,而肾血管平滑肌脂肪瘤绝大多数都含有脂肪成分,但不会同时合并钙化.本患者CT平扫显示左肾区无钙化灶,如CT値为-20或以下,则"左肾血管平滑肌脂肪瘤合并出血"诊断成立.虽然肾血管平滑肌脂肪瘤大多为良性肿瘤,但患者年轻,瘤体较大(6 cm ×5 cm)且合并出血,故应积极治疗.具体治疗方法可因治疗者经验和患者具体情况而定.如治疗者手术操作经验丰富,患者全身情况许可,应首先考虑患肾部分切除术.由于患者一月前有过肾周出血,术中估计粘连明显,应仔细分离粘连部分,尽可能完整地切除肿瘤,以减少复发,并最大限度保留正常肾单位.     

肝血管平滑肌脂肪瘤的外科治疗

目的 探讨肝血管平滑肌脂肪瘤的临床表现、病理学特征、诊断和外科治疗.方法 回顾性分析我院1990年1月至2006年12月诊治的22例肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)患者的临床资料.结果 22例患者中男6例,女16例,平均年龄48.2岁.17例患者无临床症状,所有患者均无肝炎病史,均无肾血管平滑肌脂肪瘤病史.女性患者无口服避孕药史.血AFP、CEA、CA19-9、CA242均正常.术前超声、CT、MRI诊断准确率分别为21%(4/19)、23.5%(4/17)、25%(2/8).肿瘤均为单发,直径4～17 cm,均无完整包膜.22例患者均行手术切除,17例行肝肿瘤不规则切除术,5例行左半肝切除术.1例患者术后第2天死于并发症,1例患者出现轻微胆漏,术后病理免疫组化HMB45均表达阳性,4例见髓外造血明显.随访率91%(20/22),随访时间6个月至17年,术后无肿瘤复发.结论 肝血管平滑肌脂肪瘤无特异性临床症状,术前影像学检查难以确诊,应积极的手术治疗.     

原发性肾窦肿瘤三例报告

目的 总结原发性肾窦肿瘤的临床特点及治疗方法.方法 原发性肾窦肿瘤患者3例.女2例,男1例.平均年龄40(33～55)岁.临床表现为腰痛伴血尿1例,单纯腰部疼痛2例.肿瘤位于右侧2例,左侧1例.直径平均5.5(3.5～8.5)cm.3例均经行手术治疗. 结果行右肾窦肿瘤探查术1例,术中病理提示良性肿瘤,单纯切除肿瘤,病理报告为血管平滑肌瘤,随访3年未见复发.1例术中因肿瘤出血及肾盂破坏严重,行右肾切除,病理报告为血管平滑肌脂肪瘤,随访10个月未见复发.1例因肿瘤包绕肾动静脉,局部严重黏连,行左肾切除术,病理报告为脂肪瘤,随访4年未见肿瘤复发.结论 原发性肾窦肿瘤临床罕见,多为良性,易误诊为肾盂肿瘤,CT、MRI、IVU检查有助于鉴别;良性肿瘤直径<4 cm且无症状者可密切随访,有症状或直径>4 cm者可行肿瘤切除;肿瘤累及肾蒂并严重破坏肾盂者可行单纯肾切除;恶性肿瘤应行根治性肾切除.     

Diagnosis and treatment of renal angiomyolipoma

We have hitherto reported 6 cases of renal angiomyolipoma. Recently, we encountered two more such cases. Case 1 is a 34-year-old woman with fever as the chief complaint. DIP revealed a tumor mass in the right upper pelvic pole. This mass showed a strong echo level on ECHO and adipose tissue of low density on CT scan. Therefore, the patient was diagnosed as having renal angiomyolipoma. Since liposarcoma was not ruled out by the examination of frozen sections during operation, nephrectomy was performed. Case 2 is a 40-year-old woman. Diagnosed as having bilateral renal angiomyolipoma, she underwent right nephrectomy 14 years ago. Two years ago, she had heavy hematuria, and had embolization of the left renal artery. She has had no bleeding since the embolization. We are of the view that ECHO and CT can are very useful for diagnosis of renal angiomyolipoma, and embolization for heavy hematuria, a complication, should be performed first of all.     

不典型肾错构瘤的诊断与治疗（附18例报告）

目的：探讨不典型肾错构瘤的误诊原因,提高其诊治水平。方法：回顾性分析18例不典型肾错构瘤的临床资料。18例均行B超检查,15例行CT扫描,8例行MRI扫描,6例行IVU检查。3例接受保守治疗;15例接受手术治疗,其中3例行肿瘤剜除术,5例行肾部分切除术,7例行肾癌根治术。结果：B超诊断肾错构瘤10例（10／18）,CT诊断肾错构瘤7例（7／15）。3例保守治疗者获得随访,肿瘤大小无明显变化,无转移。15例手术治疗者术后病理检查均为肾错构瘤,随访未见肿瘤转移或复发。结论：大部分肾错构瘤可通过B超、CT等影像学检查明确诊断;对于不典型肾错构瘤,术前应仔细分析影像学检查,结合病史尽量明确诊断,减少不必要的肾切除。     

Renal angiomyolipoma with marked extrarenal development: a case report

A 28-year-old female complained of minimal fever elevation. Computed tomography (CT) revealed a left renal tumor of 10 cm in diameter. Ultrasonogram and CT, magnetic resonance imaging and angiography suggested a renal angiomyolipoma (AML) with marked extrarenal development. Partial nephrectomy was performed using a microwave tissue coagulater without clamping of the renal artery. The tumor weight was 800 g and the pathological diagnosis was AML. The management of large AML is reviewed in the literature. Nephron sparing surgery should be performed even in patients who have a larger tumor with extrarenal development.     

后肾始基腺瘤的CT与病理学特点分析

目的:探讨后肾始基腺瘤的CT特征及病理特点。方法:回顾性分析经手术病理证实的18例后肾始基腺瘤患者的临床资料,其中男10例,女8例,年龄33~75岁(平均50岁),均为单侧发病,左肾8例,右肾10例。患者均行CT平扫及增强扫描。分析18例患者CT和病理特点以及两者之间的关系。结果:18例患者CT平扫肿物呈等密度或稍低密度软组织肿块,最大径1.5~7.0cm,平均3.6cm;呈类圆形12例,不规则形6例;边界清晰6例,欠清晰7例,边界不清晰5例;密度均匀及不均匀各4例,密度欠均匀10例;CT值27~45HU,平均39HU。CT增强扫描示肿物未强化2例,轻到中度强化16例,呈软组织密度肿物影,低于肾实质强化水平,CT值50~77HU,平均63HU。11例延迟期扫描示肾盂肾盏有造影剂充盈并显示受压移位。患者术后病理学均证实为后肾始基腺瘤,光镜下肿瘤细胞体积较小,均匀分布,紧密排列,大小一致,形成长管样结构,部分可见乳头状或肾小球样结构。结论:后肾腺瘤的CT检查特征与病理特点有一定的相关性;正确认识其CT及病理学特点,有助于指导手术方案的制定,避免不必要的全肾切除术。     

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.     

巨大肾血管平滑肌脂肪瘤临床分析(附27例报告)

目的 探讨巨大肾血管平滑肌脂肪瘤(renal angiomyolipoma,RAML)的临床特点,总结诊治经验.方法 回顾性分析2008年1月至2017年1月我院收治的27例巨大RAML患者(肿瘤直径8 cm及以上)的临床资料.其中男4例,女23例,年龄16~66岁,平均38岁.肿瘤位于左侧14例,右侧13例.16例有临床症状,其中7例为RAML破裂出血,11例为无症状体检发现.27例术前均行CT检查,3例行MRI检查.结果 本组27例均行手术治疗,其中11例行肾切除术,9例行保留肾单位手术,4例行腹腔镜保留肾单位手术,3例行肾癌根治术.7例RAML破裂出血患者中,1例行急诊肾切除术,1例行急诊保留肾单位手术,其余5例待症状稳定,完善相关检查后予手术治疗.根据术后肿瘤大体标本,肿瘤直径为8~27 cm,平均13.7 cm.术后21例患者获得随访,随访时间1~108个月,中位随访时间42个月,无复发或因肿瘤导致死亡者.结论 巨大RAML临床少见,多数具有腰部酸胀不适等临床症状,CT检查为主要的诊断方式,B超可以作为患者随访的首选检查.8 cm及以上巨大RAML肿瘤破裂出血风险明显增加,建议积极行手术治疗.巨大RAML以良性为主,预后较好.     

A case of angiomyolipoma in a horseshoe kidney

The patient was 52 years old. She had undergone a breast cancer operation 4 years before this visit. On computed tomography (CT), a left renal tumor in a horseshoe kidney was incidentally pointed out. CT scan showed a 1.8-cm enhanced tumor in the upper pole of the left kidney. It was hyperechoic on ultrasonography. Since renal cell carcinoma could not be excluded preoperatively, left partial nephrectomy was performed. Pathological diagnosis was a renal angiomyolipoma. The incidence of horseshoe kidney is 1 in 400. The occurrence of hydronephrosis, infection and calculous disease is not uncommon. However, a case of angiomyolipoma simultaneously with a horseshoe kidney is very rare, this being the 7th case in the literature.     

肾上皮样血管平滑肌脂肪瘤诊治探讨

目的 提高肾上皮样血管平滑肌脂肪瘤(EAML)的诊治水平.方法 肾EAML患者5例.男3例,女2例.平均年龄46(26～64)岁.患侧腰部胀痛3例,体检偶然发现2例.肿瘤直径2.9～10.1 cm.B超和CT检查诊断为血管平滑肌脂肪瘤2例,肾癌、肾上腺肿瘤、肝癌各1例.行肾肿瘤剜除术3例,肾楔形切除术1例,肾切除术1例.结果 病理报告:肿瘤由不同比例的异常厚壁血管、平滑肌和脂肪组织构成.上皮样细胞胞质浓密嗜酸性或透亮,有嗜酸性颗粒,并有丰富的血管,上皮样细胞簇常围绕血管.组织学形态表现为圆形、多边形和梭形上皮样细胞灶性分布于脂肪瘤中,上皮样瘤细胞免疫组化标记SMA、Vimentin、HMB45表达阳性.5例病理诊断均为肾EAML.随访2～12个月,未见局部复发及远处转移.结果 肾EAML由厚壁血管、脂肪和平滑肌细胞混合组成,上皮样细胞簇常围绕血管.确诊依赖于临床、影像学及病理学检查相结合,免疫组织化学染色有助于诊断及鉴别诊断.早期诊断及完整切除肿瘤是治疗关键.     

Retro-peritoneoscopic assisted cryoablation for small renal tumors: the first cases treated in Romania

Nephron-sparing surgery (NSS), has been demonstrated to be a safe and effective alternative to radical nephrectomy for selected cases. Retro-peritoneoscopic cryoablation (RCA), combine the benefits of minimal invasiveness of the laparoscopy with the advantage of preserving renal function of the nephron sparing surgery. The aim of our study was to assess the initial results with RCA of small renal tumors. Since Jan 2007, twelve consecutive patients, with small renal tumors (mean tumor size 3.89 cm) underwent RCA at our institution. The patients were assessed using: clinical exam, lab exam, ultrasound, contrast enhanced CT scan. For cryoablation, we used the Galil Medical SeedNet with 17 Gauge cryoprobes, under combined retro-peritoneoscopic and ultrasound guidance. Protocol follow-up design includes clinical exam, lab exam and contrast enhanced CT scan at 3,6 and 12 months and annually thereafter. Mean surgical time was 145.42 min. and mean blood loss was 179.17 ml. Two patients presented: bleeding at the extraction of the cryoprobes and urinary fistula which healed with surgical treatment. Histological examination of the core biopsy revealed clear cell carcinoma in 8 patients, papillary carcinoma in 3 patients and angiomyolipoma in 1 patient. Cryosurgical ablation of small renal tumors using multiple ultrathin 17 Gauge cryoprobes is a feasible treatment option. Retro-peritoneoscopic approach allows optimal access to the kidney and endoscopic real-time ultrasound control of the freezing process.