腹膜后肾外血管平滑肌脂肪瘤的诊断与治疗

目的 探讨腹膜后肾外血管平滑肌脂肪瘤(AML)的诊断与治疗特点.方法 回顾性分析4例腹膜后肾外AML患者临床资料,结合相关文献,对本病的临床、病理特点、影像学诊断和治疗作初步探讨.男3例,女1例,年龄38～60岁.肿瘤位于左肾盂旁1例、右肾盂后下方1例、左肾上极内后侧与腹膜后间隙内2例.肿瘤直径2～20 cm.类圆形1例、不规则形状3例.临床表现为肉眼血尿2例、腰部酸胀不适1例.4例行IVU、CT检查,3例行超声检查.IVU提示占位性病变2例.超声误诊为肾盂癌2例,误诊为脂肪瘤1例.CT确诊1例,3例分别误诊为肾盂旁囊肿合并出血、肾脏AML、腹膜后畸胎瘤.结果 4例均在气管插管麻醉下行手术探查,术中冰冻活检均示AML或良性肿瘤.行肿瘤切除术2例,肾和肿瘤切除术2例.术后病理诊断均为腹膜后肾外AML.术后CT随访6个月～8年,未见局部复发和转移.结论 腹膜后肾外AML术前定性困难,需综合分析临床表现、查体及IVU、B超、CT等影像学表现诊断.肿瘤完整切除是治疗本病的首选方法.预后好,但应注意密切随访.     

肾上皮样血管平滑肌脂肪瘤临床病理分析

目的 探讨肾上皮样血管平滑肌脂肪瘤的临床病理学特点,提高对该病的诊断及治疗水平.方法 回顾性分析一例病理诊断为肾上皮样血管平滑肌脂肪瘤患者的主要症状、诊治情况,结合文献对该病的临床表现、诊断、病理学特征、治疗及预后进行讨论.结果 患者男性,47岁,入院前1年出现劳累后右腰部酸痛症状,于2013年1月28日行彩超检查发现“右肾占位”.入院后诊断为“右肾占位病变”.患者拒绝行右肾部分切除术,要求行根治性右肾切除术.术后恢复良好.病理:右肾上皮样血管平滑肌脂肪瘤.组织学特点:瘤细胞胞浆丰富,粉染,形状多样,排列成片.免疫组化:CD117(-),CD10(-),CK7(-),CK8/18(-),CK20(-),RCC(-),Vimentin(+),HMB45(+),Melan-A(+),CD68(+),SMA(+),Ki67(＜3％+).随访15个月未见复发及转移.结论 肾上皮样血管平滑肌脂肪瘤是一种罕见的血管平滑肌脂肪瘤,易与肾细胞癌及其他恶性肾肿瘤相混淆,免疫组化有助于鉴别.由于它具有潜在恶性的生物学行为,故有必要进行长期随访复查.     

肾上皮样血管平滑肌脂肪瘤临床特点分析

目的 探讨肾上皮样血管平滑肌脂肪瘤的临床及病理特点.方法 肾上皮样血管平滑肌脂肪瘤患者6例.单发4例,多发2例.肿瘤直径平均9(5～14)cm.有结节硬化家族史1例.CT检查发现肿瘤内含脂肪组织(CT值-30～-120 HU)3例,诊断为肾血管平滑肌脂肪瘤;3例瘤组织CT值30～55 HU,增强扫描CT值70～110 HU.行肿瘤剜除术3例,行根治性肾切除术3例.结果 3例行肿瘤剜除术者肿瘤完整剜除,2例出现肾盂破损,行肾盂修补及留置双J管.其中1例双肾肿瘤者,2个月后手术剜除对侧肿瘤.3例根治性肾切除者完整切除肾脏、肾周脂肪组织及肿大淋巴结.病理检查:镜下肿瘤以上皮样细胞为主,并出现多形、异形核,有明显的上皮样分化,浸润破坏性生长,瘤组织广泛坏死.免疫组化染色HMB45(+++),EMA、CK(-).病理诊断均为肾上皮样血管平滑肌脂肪瘤.平均随访26(10～44)个月,肿瘤无复发及转移5例;1例根治术后18个月出现双肺转移,10个月后死亡.结论 肾上皮样血管平滑肌脂肪瘤多为良性,部分缺乏脂肪组织,主要由单核或多核的上皮样细胞构成,免疫组化染色HMB45强阳性为特征表现,具有潜在恶性倾向,可发生转移;CT检查易误诊为肾癌;手术为主要治疗方法.     

原发性附睾肿瘤的诊断与治疗(附35例报告)

目的:探讨原发性附睾肿瘤的诊断及治疗方法。方法:回顾性分析本院35例原发性附睾肿瘤患者的临床资料。行肿瘤切除术10例,患侧附睾切除23例,患侧睾丸附睾切除加腹股沟淋巴结清扫1例,患侧根治性睾丸切除加二期腹膜后淋巴结清扫术1例。附睾恶性纤维组织细胞瘤1例术后辅以放疗及化疗。结果:术后病理诊断良性肿瘤33例,其中腺瘤样瘤21例,平滑肌瘤7例,纤维瘤4例,乳头状囊腺瘤1例;恶性肿瘤2例,附睾恶性纤维组织细胞瘤1例,附睾腺癌1例。35例术后随访10个月至6年,未见复发、转移及死亡病例。结论:原发性附睾肿瘤术前明确诊断困难,对于高度怀疑附睾肿瘤的患者应首选手术探查,良性者可行肿瘤或附睾切除,恶性者行根治性睾丸切除加腹膜后淋巴结清扫术。     

泌尿系统肉瘤样癌和癌肉瘤八例报告并文献复习

目的 探讨泌尿系统肉瘤样癌和癌肉瘤的组织学特点、临床表现、治疗和预后.方法 泌尿系统肉瘤样癌和癌肉瘤8例.其中膀胱肉瘤样癌4例,男3例,女1例,年龄58、63、78、79岁,均因无痛性肉眼血尿就诊,肿瘤直径平均4.5(1.5～6.7)cm,为不规则、广基的浸润性肿物.行膀胱部分切除术2例,TURBt 1例,膀胱癌根治术1例.肾肉瘤样癌1例,男,64岁,因肉眼血尿就诊,伴同侧肾上腺和胰腺转移,行肾癌根治术和胰腺体尾切除术.转移性右肾上腺肉瘤样癌1例,男,47岁,原发灶为左肺肉瘤样癌,行左肺下叶和右肾上腺切除术.肾盂癌肉瘤1例,女,64岁,表现为无痛性肉眼血尿,行左肾、输尿管及部分膀胱切除术.膀胱癌肉瘤1例,男,77岁,无痛性肉眼血尿,肿瘤直径2.5～3.0 cm,行TURBt术.8例均经病理检查确诊.8例均获随访,随访时间36～96个月.结果 膀胱肉瘤样癌4例中:1例G_3、T_(2a)者行TURBt,术后行丝裂霉素膀胱灌注化疗,随访96个月无瘤生存;1例63岁女性先行TURBt,术后病理为肉瘤样癌、T_1伴有原位癌(T_m),1个月后行膀胱癌根治切除术.随访36个月无瘤生存;1例T3a者行膀胱部分切除术,术后辅以放疗,随访36个月无瘤生存;1例T4a者行姑息性膀胱部分切除术,术后病理切缘阳性,术后2个月死于肿瘤多脏器转移.肾肉瘤样癌患者术后2个月开始化疗(盐酸吉西他滨加卡铂),又出现肝、肺转移,术后5个月死亡.转移性右肾上腺肉瘤样癌患者术后在外院行3个疗程化疗(顺铂加依托泊苷),5个月后出现左肾上腺转移,术后7个月死亡.肾盂癌肉瘤患者的肿瘤以软骨肉瘤为主,伴少许鳞状细胞癌及未分化癌,随访60个月无瘤生存.膀胱癌肉瘤患者肿瘤病理分期为T2a,主要是平滑肌肉瘤,伴有鳞状细胞痛和腺癌,术后予以全身化疗(盐酸吉西他滨加卡铂),随访12个月死于慢性阻塞性肺气肿、肺部感染.结论 泌尿系统肉瘤样癌和癌肉瘤少见,多见于老年男性,恶性程度高,浸润性强,预后差.肿瘤的病理分期和治疗是影响预后的因素,早期诊断和积极的综合治疗可望获得较好的治疗结果、延长患者生存期.     

成人睾丸良性占位病变的诊断与治疗选择(附16例报告)

目的 探讨成人睾丸良性占位病变的诊断与治疗选择.方法 对2003～2010年诊治的16例睾丸良性肿瘤的临床资料及随访情况进行回顾性分析.结果 16例睾丸肿瘤病例术前经超声、CT或MRI、AFP和β- HCG检查,诊断13例考虑为良性病变,3例可疑恶性.术中行冰冻切片检查10例,其中7例行睾丸部分切除术,3例性质不定行睾丸根治性切除术.6例未行术中病理检查直接行根治性睾丸切除.术后经病理诊断睾丸间质细胞瘤2例,成熟畸胎瘤4例,腺瘤样瘤3例,平滑肌瘤3例,皮样囊肿1例,混合肿瘤3例.术后随访2个月～12年均未出现复发及转移.结论 睾丸良性占位的术前诊断对手术处理方式选择非常重要,B超、CT或MRI、肿瘤标记物以及术中冰冻切片活检均具有临床诊断价值.睾丸良性肿瘤的治疗应首选保留睾丸的肿瘤切除.     

肾肿瘤自发性破裂出血的诊断与治疗(附3例报告及文献复习)

目的:探讨肾肿瘤自发性破裂出血的影像学特点和诊治方法.方法:对3例肾肿瘤自发性破裂出血患者的临床诊治资料进行回顾性分析,3例均无外伤史及抗凝治疗史,均行B超,CT检查,1例行静脉肾盂造影检查.均经手术后病理证实.结果:3例患者均行开放手术,1例行根治性肾癌切除术,1例行肾部分切除术,另1例行肾切除术.其中1例为肾透明细胞癌,另2例为肾血管平滑肌瘤.结论:肾肿瘤自发性破裂为泌尿外科急症,肿瘤破裂以肾多见,CT可以准确显示出血程度和部位,肾癌破裂出血应行根治性肾切除,肾血管平滑肌瘤应行保留肾单位手术.     

恶性倾向肾巨大血管平滑肌脂肪瘤伴下腔静脉瘤栓1例报告并文献复习

目的：报告1例巨大肾血管平滑肌脂肪瘤合并腔静脉瘤栓病例。通过文献复习,探讨恶性倾向肾血管平滑肌脂肪瘤（renalangiomyolipoma,AML）的诊断与治疗。方法：报道和分析1例巨大AML伴下腔静脉瘤栓的病例资料,并通过查阅文献,分析和探讨具有恶性倾向的AML的临床表现、临床与病理学特性、诊断与鉴别诊断及外科治疗。结果：患者以双下肢肿胀就诊并行CT及MRI检查,提示AML伴肝后下腔静脉瘤栓（MayoIII）形成。行根治性肾切除和腔静脉瘤栓取出术,术后恢复良好。组织病理学证实为AML及静脉内肿瘤栓子。结论：散发的或结节性硬化症（tuberous sclerosis complex,TSC）相关的AML通常为间叶组织来源的良性肿瘤。罕见的,当瘤体侵犯肾静脉或下腔静脉并形成瘤栓时具有侵袭性。这种恶性潜能被认为是上皮样血管平滑肌脂肪瘤（EAMLs）的生物学特性,手术切除肿瘤并取出瘤栓是可能治疗这类AML的惟一有效方法。 我们的研究强调了严密的术前评估、精细的术中操作、审慎的组织病理学检查及密切的术后随访,以求获得更好的预后。     

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.     

成人肾恶性横纹肌样瘤6例分析

目的:探讨成人恶性肾横纹肌样瘤的临床表现、病理特点和诊治方法。方法:对6例成人恶性肾横纹肌样瘤进行临床分析及文献复习,从临床表现、病理特点和诊治方法等方面进行总结。临床表现为腰痛2例,无痛全程肉眼血尿2例,查体发现2例;B超、CT及IVU等影像学检查均提示肾脏占位性病变,直径2.8～20.4cm。CT发现肿瘤内有坏死液化及不均匀强化影像。4例经腰行根治性肾切除术,1例经腹行左肾根治性切除术,1例经胸腹联合行左肾根治性切除术。结果:6例术后病理报告均为肾恶性横纹肌样瘤,其中1例并发透明细胞癌。术后均给予生物免疫治疗。1例术后2个月发现肺转移,1年后死于肿瘤肺转移;1例术后出现肺部感染,1年后死于心肺功能衰竭;3例术后半年无瘤生存,现在随访中;1例失访。结论:成人肾恶性横纹肌样瘤罕见,临床表现无特征性,CT表现为肿瘤内部坏死液化,轻度强化而且强化不均匀,最终确诊依赖于病理组织学、免疫组织化学和电镜检查结果。治疗方法包括开放手术或腹腔镜手术,并且辅助术后化疗或生物免疫治疗。     

Renal angiomyolipoma: report of 2 cases of tuberous sclerosis in female siblings

Two cases of renal angiomyolipomas in female siblings associated with tuberous sclerosis were reported. The first case was a 47-year-old housewife who visited the hospital because of severe right flank pain. Bilateral renal angiomyolipomas and hemorrhage in the right angiomyolipoma were recognized by computerized tomography, ultrasonography and renal angiography. After 2 months she had another episode of left flank pain and was diagnosed to have hemorrhage in the left renal tumor. The pain remissed by conservative treatment. Bilateral nephrectomy and hemodialysis would be necessary if she were to have a third attack. The second case was her 36-year-old sister with the chief complaint of left flank pain and genital bleeding. CT and renal angiography suggested a large angiomyolipoma in her left kidney and a small one in her right kidney. Left nephrectomy, right partial nephrectomy and hysterectomy were performed to prevent intratumoral hemorrhage. The histopathological diagnosis was angiomyolipoma of kidneys, uterus and lymphnodes of right renal hilus. Although pre-operative differentiation of angiomyolipoma from renal cell carcinoma has been difficult, recently diagnosis has become possible by CT, ultra-sonography and angiography.     

Malignant epithelioid angiomyolipoma ('sarcoma ex angiomyolipoma') of the kidney: a case report and review of the literature

Malignant epithelioid angiomyolipoma is a recently described rare tumor of the kidney. Its existence has been questioned, however, on the basis of incomplete evidence of malignant behavior, the absence of an associated classic angiomyolipoma component, or the absence of immunoreactivity for HMB-45 in some cases. We describe a case that was HMB-45-positive and arose in association with a classic angiomyolipoma. The patient was treated with a partial nephrectomy. Three years later, she developed rapidly enlarging liver nodules. A fine-needle aspiration of the liver confirmed the presence of pleomorphic epithelioid cells morphologically and immunohistochemically identical to those comprising the primary renal tumor. After two cycles of treatment with doxorubicin, there was a 50% reduction in the size of the tumors with marked improvement in performance status. We believe this case confirms the existence of a malignant epithelioid angiomyolipoma.     

原发性肾脏类癌2例报告并文献复习

目的：探讨原发性肾脏类癌的临床特征及诊治方法。方法：总结2例原发性肾脏类癌患者的临床资料结合文献复习讨论。例1,女,48岁。因右腰酸痛1个月入院。B超示右肾中上极中等回声占位,边界清楚,内有血流信号。CT示右肾中上极10．0cm×7．5cm实性占位,增强后病灶均匀轻度强化。例2,男,44岁。体检发现左肾占岱40天入院。B超示左肾门处中等回声占位,边界清楚,内有血流信号。CT示左肾中极肾门处3．7cm×3．5cm实性占位,增强后病灶均匀轻度强化;其内伴-钙化点。结果：2例均行根治性肾切除术。病理检查镜下肿瘤细胞形态为多边形,嗜酸性颗粒状细胞质,边界不清,呈柱状或缎带状排列混合有实性巢状排列;细胞核呈园形大小一致,核分裂像难见。免疫组化：突触素、波形蛋白均为阳性。病理诊断为肾脏类癌伴淋巴结转移。例1术后30个月死于肝骨转移;例2术后随访26个月,未见肿瘤复发转移。结论：原发性肾脏类癌临床罕见,易发生淋巴结转移,确诊依靠病理学检查,根治性肾切除应为治疗原发性肾类癌的首选方法。     

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.     

Rapidly growing renal angiomyolipoma during pregnancy with tumor thrombus into the inferior vena cava: a case report

A case of renal angiomyolipoma rapidly growing during pregnancy with tumor thrombus into the inferior vena cava is reported. Angiomyolipoma in a 31-year-old woman was incidentally found by ultrasonography. CT scan revealed a fat-containing tumor in the right kidney with 4 cm in diameter. The patient was followed at outpatient clinic without any treatment. Fifteen months later, the post-delivery follow-up CT scan showed that tumor size had grown up to 11 cm in diameter. Although laparoscopic right nephrectomy was tried, open transperitoneal right nephrectomy was performed because the tumor thrombus extending into the inferior vena cava was found during the laparoscopic procedure. Pathological diagnosis was angiomyolipoma of the kidney. There are several reports that indicate angiomyolipoma grows rapidly during pregnancy. Our case demonstrates that careful follow-up is required for angiomyolipoma in women with possible conception and delivery.     

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.     

Role of mammalian target of rapamycin inhibitor in the treatment of metastatic epithelioid angiomyolipoma: A case report

Epithelioid angiomyolipoma has malignant potential; however, no effective therapy has been established for advanced cases. A 50‐year‐old woman with a history of right nephrectomy for epithelioid angiomyolipoma was referred to our institution. Computed tomography and magnetic resonance imaging showed multiple tumors in her lung, liver and pelvic cavity. The liver and pelvic tumor specimens obtained by needle biopsy confirmed the diagnosis of epithelioid angiomyolipoma recurrence. The patient was treated with everolimus (10 mg/day). Three months later, pulmonary lesions disappeared; liver and pelvic tumors significantly shrank in size, but the pelvic tumor gradually enlarged again. We carried out surgical resection of the residual liver and pelvic cavity tumors. Although the mammalian target of rapamycin inhibitor seems to be effective for treating epithelioid angiomyolipoma, its long‐term effects remain unknown. Thus, aggressive administration of a multidisciplinary treatment including molecular target therapy and surgical resection is required to improve the prognosis of epithelioid angiomyolipoma.     

A case of postpartum spontaneous rupture of an angiomyolipoma

A case of postpartum spontaneous rupture of angiomyolipoma in a 27-year-old woman with the chief complaints of right flank pain and fever was reported. Physical examinations revealed an infant-head sized, hard elastic mass with a smooth surface in the right flank. Laboratory studies showed a decrease of Hb to 9.0 g/dl and Ht to 26.5%, and elevated LDH (2914 IU/l). Angiomyolipoma was suspected with ultrasonography, CT scan and angiography. Transperitoneal right nephrectomy was performed. The right kidney and tumor weighed 1,870 g. Pathological diagnosis also confirmed angiomyolipoma of the kidney. Discussion of the case and a brief review of the literature on spontaneous rupture of angiomyolipoma in the intra- and postpartum are made.     

Two cases of leiomyoma of the kidney]

Renal leiomyomas large enough to be clinically diagnosed are extremely rare. We review 30 cases of clinically diagnosed renal leiomyoma from the literature in Japan, including our 2 new cases. Case 1: In a 52-year-old man with no symptoms a renal mass was found accidentally on an ultrasonogram. CT scan showed a mass with a cystic area at the upper pole of the right kidney. Angiogram showed a hypovascular mass. Case 2: CT scan revealed a cystic mass and angiogram showed an avascular mass at the upper pole of the left kidney in a 19-year-old man having gross hematuria and left flank pain. Transperitoneal nephrectomy was done in both cases. Histologically each tumor was composed of monotonous proliferation of spindle shaped cells without atypia, which showed intense immunoreactivity for alpha-smooth muscle actin. The diagnosis of benign leiomyoma was made in each case. In a review of 30 cases, we found that renal leiomyomas occur most often in female (77%), between decades 2 and 5 of life (median: 46 years). On the angiogram it appears most often as an avascular or hypovascular mass. CT scan shows cystic or mixed solid/cystic or solid lesion, occasionally with calcification. Preoperative diagnosis is extremely difficult to be made. Histologically, fibroma, angiomyolipoma, congenital mesoblastic nephroma and leiomyosarcoma should be differentiated.