肾错构瘤合并结节性硬化的诊断与治疗(4例报告与文献复习)

目的　进一步提高对肾错构瘤合并结节性硬化的诊断和治疗水平。方法　对 4例肾错构瘤合并结节性硬化患者的临床资料和随访情况进行回顾性分析。结果　4例肾错构瘤合并结节性硬化患者均行手术治疗,术后病理结果明确诊断,术后定期复查B超,平均随访 30个月,均无复发。结论　肾错构瘤合并结节性硬化的患者多为双侧多发性病变,如一定要行手术治疗应慎重,必须要考虑肾功能的保留问题,肾部分切除和或选择性肾动脉栓塞术为首选。     

肾错构瘤自发性破裂出血的外科诊治（附14例报告）

目的：探讨肾错构瘤自发性破裂出血的诊断与治疗。方法分析手术治疗14例肾错构瘤自发破裂出血患者的临床资料,所有患者均有腰腹部疼痛病史就诊。术前检查均行彩超和CT检查,2例因有疑问行MRI检查。其中10例患者行患肾切除术,2例行肾部分切除术,2例行肿瘤剜除术。结果所有患者术后恢复良好,2例术后出现尿漏,延期痊愈后出院。随访8～48个月,无死亡病例,未发现肿瘤复发。结论彩超、CT及MRI是确诊肾错构瘤破裂出血简单而有效的方法。对于直径＞4 cm的肾错构瘤破裂出血患者,应积极行手术治疗,并尽可能保留有功能的肾组织。     

急诊介入治疗肾错构瘤破裂出血

目的:评价在急诊下栓塞治疗肾错构瘤破裂出血的安全性和有效性。方法:3例经B超、CT确诊的肾错构瘤患者,左肾1例,右肾1例,双肾1例,突然出现腰腹痛、神志淡漠,血压下降等出血性休克症状,急诊行选择性肾动脉造影和栓塞治疗,栓塞剂用无水酒精和弹簧圈。结果:3例患者造影发现:肿瘤区血管紊乱并有动脉瘤形成,栓塞后造影显示:肿瘤血管血供减少,肿瘤内载有动脉瘤血供闭塞。无严重并发症出现。术后随访患者血压上升,临床症状改善。结论:选择性动脉栓塞治疗肾错构瘤破裂出血,能有效地控制出血并能最大限度保护正常肾组织,应作为急诊出血首选治疗方法。     

巨大肾血管平滑肌脂肪瘤剜除术1例报告并文献复习

目的探讨巨大肾血管平滑肌脂肪瘤剜除术可行性及安全性。方法我科于2014年4月收住1例巨大肾血管平滑肌脂肪瘤患者,术前纠正贫血,采用上腹部反L形切口,良好显露肿瘤,常温条件下阻断肾蒂,剜除该肾巨大错构瘤。同时,结合文献分析讨论。结果成功剜除肿瘤保留肾脏,手术时间120 min,肾动脉阻断时间15 min,术中失血量150 ml,手术顺利,术后恢复1周出院。术后测肿瘤最大径22 cm,术后无漏尿,术后5个月复查CT患肾未见异常。检索CNKI及Pub Med相关文献至2014年10月,检出中文文献35篇,英文文献40篇。结论对于肾巨大错构瘤争取保留肾脏是可行的,选择合理的手术切口,充分显露手术视野是手术成功的关键。     

结节性硬化症合并双肾错构瘤恶变1例

结节性硬化症（tuberous sclerosis complex，TSC）是多种器官错构瘤为特征的系统性疾病，主要临床特征为面部皮脂腺瘤、癫痫及智力减退，80％的TSC患者合并肾脏的错构瘤，很少恶变，现将1例结节性硬化症合并双肾错构瘤恶变病例报告如下。     

结节性硬化的影像诊断

目的:探讨分析结节性硬化的临床表现及影像学特点。方法:回顾分析7例结节性硬化病例的临床、影像学表现并作文献复习。7例中,男性4例,女性3例,平均年龄38岁;其中3例有轻度的智力障碍,1例有阵发性抽搐;3例有典型的面部皮脂腺瘤及/或腰骶部颗粒斑。结果:7例病人头颅CT扫描都有不同程度的脑室室管膜下及脑皮层、脑白质的钙化结节;头颅MRI表现为室管膜下结节,室壁呈波浪状改变,其中1例可见巨细胞星形细胞瘤;6例可见双肾多发错构瘤,1例可见肝脏错构瘤。结论:结节性硬化的临床及影像学表现有一定特征性,CT及MRI在结节性硬化中的诊断具有十分重要的价值。     

超选择性动脉栓塞治疗孤立肾巨大错构瘤1例北大核心CSCD

1临床资料患者女,42岁,于2020年8月26日入院,入院时无明显症状。患者有结节性硬化症病史,20年前检查发现双侧肾错构瘤,因右侧较大行右侧部分肿瘤切除术,10年前又因左肾错构瘤破裂大出血行左肾全切术,2013年前右肾肿瘤破裂出血多次,均予保守治疗后改善,2013年6月18日右肾肿瘤再次破裂出血,保守治疗无效后急诊行右肾动脉栓塞术,术后至今患者未再次出血。     

肾移植术后尿路上皮肿瘤的临床特点及诊疗经验

 目的 总结肾移植术后尿路上皮肿瘤的临床特点及诊治体会。方法 回顾性分析清华大学第一附属医院和解放军总医院第三医学中心2004-07至2017-12收治的31例肾移植术后尿路上皮肿瘤患者的临床资料。结果 31例肾移植术后尿路上皮肿瘤患者中,男10例,女21例,15例为输尿管肿瘤,7例为膀胱肿瘤,3例膀胱肿瘤同时合并输尿管肿瘤,5例肾盂肿瘤,1例移植肾肾盂癌。患者均接受手术治疗,其中17例患者术后肿瘤复发而再次接受手术治疗。随访期内6例因肿瘤复发死亡,1例因肠梗阻伴脓毒症死亡。结论 肾移植受者更易发生肿瘤,应早期诊断并根据肿瘤特点尽早采取积极的手术治疗措施。     

手术治疗多发性结节性甲状腺肿60例临床体会

目的：探讨手术治疗多发性结节性甲状腺肿60例临床疗效。方法：采用回顾性分析的方法,分析我院收治的60例多发性结节性甲状腺肿患者的临床资料。结果：所有患者手术均顺利完成,均未发生伤口感染、出血,术后无声嘶及呼吸困难等,未出现喉返神经受损及喉头水肿等并发症发生,所有患者伤口一期愈合后出院。随访6—24个月,随访率100％,术后复发7例,其中患侧甲状腺腺叶切除术5例,腺体次全切除术2例,经二次手术切除甲状腺结节及颈部淋巴结,术后恢复良好。结论：采取不同术式对多发性结节性甲状腺肿治疗疗效明显。     

选择性动脉栓塞治疗孤立肾错构瘤6例

目的 评估平阳霉素碘油混合液联合无水乙醇超选择动脉栓塞治疗孤立肾错构瘤的有效性及安全性.方法 回顾性分析2009年5月至2015年10月收治的6例孤立肾错构瘤患者.所有患者接受多次超选择动脉栓塞术,根据每次随访结果以不同栓塞材料再次作栓塞治疗.采用CT增强或MRI平扫检查随访患者病灶清除率及复发情况.结果 6例患者每次栓塞术均成功,技术成功率100％.每例患者平均手术3次,平均手术间隔时间约1年,平均随访时间2.5年.术后3、6、12个月随访提示平阳霉素碘油混合液联合PVA颗粒栓塞可缩小病灶,但不能清除病灶;平阳霉素碘油混合液联合无水乙醇栓塞可清除病灶,远期随访未见原病灶复发.结论 平阳霉素碘油混合液联合无水乙醇可安全有效地栓塞肾错构瘤,可能是孤立肾错构瘤患者栓塞治疗的较好材料.     

Frequency and imaging appearance of hepatic angiomyolipomas in pediatric and adult patients with tuberous sclerosis

OBJECTIVE: This study evaluates the frequency and imaging appearance of hepatic angiomyolipomas in pediatric and adult patients with tuberous sclerosis. CONCLUSION: Multiple hepatic angiomyolipomas are often found in patients with tuberous sclerosis, particularly in those with bilateral diffuse renal angiomyolipomas.     

Management of symptomatic renal angiomyolipomas by embolization

Renal angiomyolipomas are benign tumors which occur commonly in association with tuberous sclerosis or as isolated lesions. Symptomatic angiomyolipomas, particularly those in patients with tuberous sclerosis, should be approached conservatively with the goal of preserving as much normal renal tissue as possible. We report 2 patients in whom selective embolization was used to achieve this goal.     

Management of symptomatic renal angiomyolipomas by embolization

Renal angiomyolipomas are benign tumors which occur commonly in association with tuberous sclerosis or as isolated lesions. Symptomatic angiomyolipomas, particularly those in patients with tuberous sclerosis, should be approached conservatively with the goal of preserving as much normal renal tissue as possible. We report 2 patients in whom selective embolization was used to achieve this goal.     

Cystic renal disease in tuberous sclerosis

The clinical and radiologic findings of tuberous sclerosis in three family members, which manifested as renal insufficiency due to extensive renal cystic disease, are described. The family was thought to have polycystic kidney disease until coexisting tiny angiomyolipomas were found among the cysts (by computed tomography in two patients and at autopsy in one). The radiologic and pathologic factors that establish the diagnosis of renal cystic disease associated with the tuberous sclerosis complex are reviewed.     

CT differentiation of large exophytic renal angiomyolipomas and perirenal liposarcomas

OBJECTIVE: The purpose of our study was to describe the imaging findings and CT characteristics that lead to accurate distinction of large exophytic renal angiomyolipomas from retroperitoneal perirenal liposarcomas, which at times can be confused on imaging studies and even at pathologic examination. MATERIALS AND METHODS: We retrospectively analyzed CT images of 15 large exophytic renal angiomyolipomas and 12 well-differentiated perirenal liposarcomas. Pathologic correlation was available for six of 15 angiomyolipomas and all of the liposarcomas. All examinations were evaluated for lesion size, renal parenchymal defect, enlarged vessels, kidney displacement, lesion encapsulation or margination, associated hemorrhage, and additional angiomyolipomas. The records of patients with tuberous sclerosis or the forme fruste of that condition were excluded from the study. RESULTS: The average size of the angiomyolipomas was 14 x 10 cm. They showed a renal parenchymal defect (n = 15), enlarged vessels (n = 12), renal displacement (n = 14), good margination without a distinct capsule (n = 14), hemorrhage (n = 1), and additional (one or two) angiomyolipomas (n = 4). The average size of the liposarcomas was 18 x 11.6 cm. They showed enlarged vessels (n = 3), renal displacement (n = 11), and encapsulation (n = 4); none showed a renal parenchymal defect, hemorrhage, or associated angiomyolipomas. CONCLUSION: Although large exophytic angiomyolipomas and well-differentiated retroperitoneal liposarcomas may have similar appearances on imaging, careful evaluation for a defect in the renal parenchyma combined with the presence of enlarged vessels in angiomyolipomas should enable accurate differentiation in almost all cases. Achieving an accurate diagnosis can have a significant impact on patient treatment.     

结节性硬化的CT征象分析

目的分析结节性硬化的CT征象以提高对本病的认识。方法14例临床证实的结节性硬化患者均经脑部与腹部CT平扫,其中,6例患者又经对比增强CT扫描。对所有患者的CT表现与临床资料进行了回顾性分析。结果在14例结节性硬化患者中,室管膜下结节见于14例,室管膜下巨细胞星形细胞瘤4例,皮质和皮质下结节6例,脑白质病变2例,肾脏血管平滑肌脂肪瘤4例,肝脏肿块1例。结论脑部与肾脏结节性硬化具有利于确诊本病的特征性CT征象。     

Various radiological appearances of angiomyolipoinas in the same kidney

A 21-year-old woman with tuberous sclerosis presented with abdominal distension and flank pain. Imaging studies, including CT and MR imaging, revealed bilateral renal mass lesions, containing fat and suggesting the diagnosis of tuberous sclerosis. However the imaging characteristics of one of these lesions differed from the others with no radiologically detectable fat tissue in this solid lesion suggesting renal cell carcinoma. Histopathological examination of this lesion in the left kidney revealed an angiomyolipoma with minimal fat tissue. The radiological diagnosis of angiomyolipomas with minimal fat tissue remains difficult and the differential diagnosis is discussed. Received: 5 October 1998; Revised: 22 December 1998; Accepted: 7 June 1999     

Intratumoral pseudoaneurysm: atypical radiologic manifestation of bleeding from an angiomyolipoma

We present the case of a patient with tuberous sclerosis and multiple bilateral renal angiomyolipomas (AML) who developed an intralesional pseudoaneurysm in the context of bleeding from an AML. The diagnosis was reached by ultrasonography and computed tomography, and the histological diagnosis of a contained hematoma confirmed the bleeding.     

Hemorrhagic renal angiomyolipoma: Superselective renal arterial embolization for preservation of renal function

A 35-year-old woman with tuberous sclerosis and known bilateral renal angiomyolipomas presented with shock due to massive hematuria arising from the left kidney. The cause of bleeding was diagnosed angiographically as arising from a left upper pole renal artery aneurysm within the tumor. Cessation of bleeding and clinical stabilization occurred without deterioration of renal function after superselective embolization of the dorsal segmental renal artery with a single 5-mm Gianturco coil.     

Lipomatous tumors of the liver: evaluation with CT and US

Seven cases of lipomatous masses within the liver parenchyma were demonstrated with computed tomography (CT). Five of these cases were obtained from a retrospective review of 50 cases of renal angiomyolipoma in which the liver was adequately demonstrated. The other two cases were from the files of the Armed Forces Institute of Pathology and had no associated renal lesions. Three of the five cases were associated with tuberous sclerosis. In all seven cases, the fatty tumors appeared on CT scans as a well-defined, 0.8-13-cm mass, with attenuation coefficients of less than -30 HU. On ultrasound studies, the lesions were well circumscribed, highly echogenic, and similar to hemangiomas. While distinctly rare lesions, these lipomatous masses are not as unusual as the literature would indicate. One may anticipate such masses in patients with renal angiomyolipomas and in a relatively high percentage of those with tuberous sclerosis.