肾血管平滑肌脂肪瘤的诊断与治疗

目的 探讨肾血管平滑肌脂肪瘤（错构瘤）的诊断与治疗方法。方法 回顾分析２２例肾错构瘤的临床资料。结果 诊断符合率Ｂ超为５４．５％,ＣＴ为８１．８％,术前诊断错构瘤１８例,误诊为肾癌４例,其中３例术中冻冻切片示错构瘤。行肿瘤剜除１１例,肾部分切除７例,肾切除３例,肾根治性切除１例。结论 ＣＴ是诊断错构瘤的首选检查手段,肿瘤体积小、脂仿含量少、瘤内出血是影像学改变不典型导致误诊的原因,仔细分析病史、影像学资料及术中冰冻切片可避免误诊,手术治疗应尽量采用保留肾脏的手术。     

双侧肾血管平滑肌脂肪瘤的诊断和治疗

目的：探讨双侧肾血管平滑肌脂肪瘤的诊治方法。方法：回顾性分析13例经手术治疗的双侧肾血管平滑肌脂肪瘤患者的诊治资料。男2例，女11例；平均年龄42岁。术前均经B超和CT检查，11例诊断肾血管平滑肌脂肪瘤，2例不排除有癌性结节。行单侧保肾手术5例，一期双侧保肾手术8例。结果：13例术中快速冰冻和术后病理检查结果均为肾血管平滑肌脂肪瘤。术后1例血肌酐短暂升高(350μmol／L)，2周后降至正常；12例肾功能未受影响。13例随访1～4年，肾功能正常，肿瘤无复发。结论：B超和CT是诊断双侧肾血管平滑肌脂肪瘤的重要手段，具有独特的影像学特点；术中快速冰冻切片检查有助于正确诊断和手术方法的选择；手术时应注意保留正常肾组织，保护肾功能。     

Pathologic vascularity of renal angiomyolipoma: cast corrosion and angiographic study.

Angiomyolipomas of the kidney at times have been preoperatively identified by the interlobar or interlobular artery eneurysms in the early vascular phase of renal arteriograms. A case report is presented in which a plastic cast corrosion technique was used postnephrectomy to demonstrate the presence of aneurysms identified angiographically prior to surgery. Portions of a cast corrosion model of a hypernephroma are also exhibited to compare and contrast pathologic vascular features of both types of tumors.     

Diagnostic and therapeutic problems in multicentric renal angiomyolipoma.

Multicentric renal angiomyolipoma is a rare form of benign tumor. However, its effective incidence as evaluated in autopsy studies may be as high as 8%. There are 2 main types of renal angiomyolipoma, that is isolated forms and those associated with other diseases, such as phakomatosis, polycystic kidneys and fibromuscular dysplasia. The tumor may also display malignant behavior with local invasiveness and regional lymph node involvement. However, the clinical course is benign and multicentricity is important for prognosis. Histopathological diagnosis often is difficult. Immunohistochemical analysis of surgical specimens using a panel of monoclonal antibodies, including HMB-45 and actin, enabled us to make a definitive diagnosis in 3 cases of multicentric renal angiomyolipoma.     

Massive renal angiomyolipoma: preoperative infarction by balloon catheter.

A case of a renal angiomyolipoma supplied by 2 renal arteries is reported. Preoperative, percutaneous transluminal infarction by a balloon catheter facilitated surgical removal of this massive neoplasm.     

Intra-sinusal renal angiomyolipoma. Differential diagnosis from renal artery aneurysm

We report a case of atypical renal angiomyolipoma (AML) because of its clinic presentation like colic renal pain, its uncommon intrasinusal location, its low fatty content, and especially because of the diagnostic doubt with a renal artery aneurysm that could not be resolved before the surgery in spite of making Doppler ultrasound, renal one sided arteriography, computed tomography (CT) and magnetic resonance imaging (MRI). We gave up doing a percutaneous needle biopsy because of the location, the association with hematoma and the possibility of a vascular origin. It has been said that diagnostic images, mainly ultrasound and CT, have a high capacity to get a renal AML diagnosis before the surgery. However when there is an atypical characteristics association like we have talked about, dismissing other pathologies is necessary. We believe AML must be considered one of these because of its difficult differential diagnosis.     

肾错构瘤的诊断与治疗(附13例报告)

目的探讨肾错构瘤的诊断和治疗。方法本组13例患者手术治疗,4例行肿瘤剜除术,6例行患肾部分切除术,1例患者因急腹症就诊探查确诊切除患肾,1例恶变者行肾癌根治术,1例保守治疗。结果所有手术患者术后恢复顺利,术后随访6个月~2年,未见肿瘤复发。结论通过B超、CT等检查,大部分肾错构瘤的患者都可明确诊断,少数瘤体破裂大出血而诊断未明者,宜剖腹探查。手术是治疗肾错构瘤的主要方法,特别是对于肾错构瘤体积大于4cm者应尽早手术治疗。对少数非手术治疗者,应严密观察。手术宜尽量保留有功能的肾组织,恶变者行肾癌根治术。     

The diagnostic dilemma of renal angiomyolipoma

The diagnosis of renal angiomyolipoma has always been difficult to make preoperatively. When the clinical and radiologic features are highly suggestive of an angiomyolipoma, the surgeon may resort to open renal frozen-section biopsy in an attempt to avoid unnecessary radical nephrectomy. The authors report two cases of renal angiomyolipoma that highlight the diagnostic problems of the tumour and the difficulty in differentiating it from renal adenocarcinoma. This is a major problem for urologists in centres where facilities for computerized axial tomography are unavailable.     

乏脂性肾血管平滑肌脂肪瘤患者9例临床分析

目的提高对乏脂性肾血管平滑肌脂肪瘤的认识,探讨其诊治方法。方法报告9例经本院收治的乏脂性肾血管平滑肌脂肪瘤的临床资料,回顾性分析其临床特征及影像学表现。9例患者均无明显临床症状。术前超声检查均提示低回声占位,占位可见血流信号。CT检查8例平扫期未见脂肪样低密度。肿瘤动脉期呈现明显强化,CT值最高可达100Hu,实质期肿瘤持续均匀强化。术前诊断为肾脏恶性肿瘤。另1例患者平扫可见局部点状低密度灶,增强扫描点状低密度灶无强化。分析所有患者临床资料并检索Pubmed和CBM数据库,对该疾病相关文献进行复习。结果所有患者均接受手术治疗。1例因患者原因施行根治性肾切除术,余8例患者接受保留肾单位的肾部分切除术。其中6例行腹腔镜肾部分切除术,2例行开放肾部分切除术。术后病理提示肾血管平滑肌脂肪瘤。所有患者均获随访9~36个月,未见肿瘤复发。结论乏脂性肾血管平滑肌脂肪瘤临床少见,对病灶CT的细微分析能够给诊断带来帮助,对于乏脂性肾血管平滑肌脂肪瘤特别是体积较小的肿瘤应该力争行保留肾单位的手术,以减少盲目肾切除造成的过度治疗。     

52例肾血管平滑肌脂肪瘤并自发破裂出血的诊治

目的探讨肾血管平滑肌脂肪瘤并自发破裂出血的诊治方法。方法回顾性分析52例手术治疗的肾血管平滑肌脂肪瘤并发自发破裂出血的诊治资料。术前均行B超和CT检查,46例诊断为肾血管平滑肌脂肪瘤并发自发破裂出血,6例未能排除肾细胞癌出血。6例保守治疗,11例行肾动脉栓塞术;35例急诊开放手术。结果6例经保守治疗成功无需手术,11例行肾动脉栓塞术中6例需开放手术;9例行保留肾单位肾切除手术,32例行全肾切除。全肾切除的原因:26例肿瘤广泛侵入肾脏,4例出血,2例未能排除肾细胞癌。术后病理诊断为血管平滑肌脂肪瘤并出血。结论B超和CT是诊断肾血管平滑肌脂肪瘤并出血的重要手段,治疗可以选择保守治疗、肾动脉栓塞或者急诊开放手术。     

Fine needle aspiration biopsy in the diagnosis of renal angiomyolipoma

Exclusive reliance on radiographic techniques for the diagnosis of renal angiomyolipoma can lead to misdiagnosis when the histological status is atypical, computerized tomographic findings are equivocal or renal cell carcinoma coexists. We report our experience and those of others in combining fine needle aspiration biopsy and radiological imaging to identify renal angiomyolipoma. Fine needle aspiration biopsy is safe and provides accurate histological diagnosis of renal angiomyolipoma.     

肾脏血管平滑肌脂肪瘤的CT诊断(附24例分析)

目的探讨肾脏血管平滑肌脂肪瘤（RAML）的CT征象及CT检查在其诊断中的价值。方法回顾性分析总结了24例经手术病理及随访证实的RAML的临床及CT资料，其中男8例，女16例，年龄23—73岁，病程3个月-2年。结果24例RAML中，双侧发病6例，单侧发病18例，病灶大小1—15cm不等。CT表现为肾脏内单发或多发圆形混杂密度肿块影，其内并见脂肪征、蜂窝征和线样征。2例合并病灶周围环形钙化，3例病灶合并出血。增强检查表现为病灶内软组织成分强化。结论典型的RAML有其特征性的CT征象，确诊的依据足病灶内是否含有脂肪组织。CT检查在RAML的诊断中具有重要价值。     

肾血管平滑肌脂肪瘤的影像学诊断（附35例报告）

目的提高肾血管平滑肌脂肪瘤的诊治水平。方法对35例肾血管平滑肌脂肪瘤患影像学检查及手术情况进行回顾性研究。结果影像学检查对RAML具有特异性,RAML的组成成分也影响影像学诊断的准确性。结论脂肪含量低或混有血凝块是肾血管平滑肌脂肪瘤误诊的主要原因,可通过加密扫描或螺旋CT加以解决。影像学检查对肿瘤定位及成分的分析有助于指导治疗方案的选择。     

The natural history of renal angiomyolipoma]

PURPOSE: To make the policy of treatment with angiomyolipomas (AML) more clear, we discussed the natural history of angiomyolipomas retrospectively. PATIENTS AND METHODS: Between May 1982 and December 1997, 14 patients with AML in 18 kidneys were followed, who were 2 men in 2 kidneys and 12 women in 16 kidneys, 27 to 80 years old. No patients suffered from tuberous sclerosis. Symptoms, initial sizes and changes of the size were evaluated for these patients. RESULTS: Ten patients with AML in 14 kidneys were asymptomatic and four patients were symptomatic. But one of the 4 patients had symptoms of abdominal pain and palpable mass which were due to contralateral AML that were treated with nephrectomy, so symptoms due to small AML were seen in 3 cases (2.0 cm, 3.5 cm, 3.8 cm). Among 11 patients in 15 kidneys followed radiologically for more than 6 months, the tumors were unchanged in size in 7 kidneys, which were in all of 6 cases with unilateral solitary tumor and in 1 with bilateral multiple tumors. In other 8 kidneys the sizes of the tumors were increased, which were in the cases with multiple tumors in one kidney or in bilateral cases. Compared to the cases of unilateral solitary AML, the size of AML with multiple tumors in one kidney or in bilateral kidneys significantly increased (p < 0.01). Embolization were performed for 4 kidneys, which were in 2 cases with increased tumor in size to more than 4 cm in following period, in 1 with dull flank pain, and in 1 with the tumor more than 4 cm at diagnosis that grew to more than 5 cm. CONCLUSIONS: Unilateral solitary AML was appeared to be hard to increase in size and to have a different natural history from bilateral or multiple tumors.     

Rupture of renal angiomyolipoma

Spontaneous rupture of renal angiomyolipoma is a rare, but life threatening condition. A case is reported which illustrates the role of computerized tomography in the diagnosis and management of this condition. Rupture of these benign lesions should be managed initially with blood transfusion and angiographic embolization if haemorrhage continues. In cases where surgery is necessary, a kidney-sparing technique should be performed when possible, such as enucleation of the lesion or partial nephrectomy.     

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.     

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.