1、前 言肾血管平滑肌脂肪瘤是临床常见的肾良性肿瘤之一,以往我们只是把它当作一种局限于肾脏的肿瘤来看待,但实际上肾血管平滑肌脂肪瘤可以是一些全身疾病在肾脏的表现。在这里我们将肾血管平滑肌脂肪瘤分成三类:一类一类:散发的偶发的局限于肾脏的血管平滑肌脂肪瘤散发的偶发的局限于肾脏的血管平滑肌脂肪瘤二类二类:淋巴管肌瘤病累及肾脏的血管平滑肌脂肪瘤淋巴管肌瘤病累及肾脏的血管平滑肌脂肪瘤三类三类:伴有结节硬化症的肾脏的血管平滑肌脂肪瘤伴有结节硬化症的肾脏的血管平滑肌脂肪瘤一类一类:散发的偶发的局限于肾脏的血管平滑肌脂肪瘤散发的偶发的局限于肾脏的血管平滑肌脂肪瘤二类二类:淋巴管肌瘤病累及肾脏的血管平滑肌脂肪瘤淋
2、巴管肌瘤病累及肾脏的血管平滑肌脂肪瘤一般介绍:淋巴管平滑肌瘤(LAM)属全身性疾病，因淋巴管内的平 滑肌细胞异常增殖导致支气管、淋巴管和小血管阻塞。发病人群:育龄期妇女累及器官:病变可累及全身多个器官,以肺部最易受累。国外有报道 LAM可同时累及肺和腹膜后淋巴结、肾脏、子宫等器官。发病机理:不明,可能与激素分泌有关病理特征:胸部的基本病理过程是淋巴管、小血管、小气道及其周 围平滑肌细胞呈进行性增生,造成管腔结构狭窄或阻塞而 致空气潴留,远端肺泡扩大融合,呈囊状腔。纵隔和腹膜后 淋巴管平滑肌增生,造成淋巴回流受阻,淋巴管扩张形成淋 巴管肌 瘤。发病率：15淋巴管肌瘤病伴有肾脏血管肌脂瘤淋巴管肌瘤
3、病LAM的胸部CT表现:主要为要为双肺从肺尖至肺底呈弥漫分布的囊状改变,分布均匀或不均匀,薄壁,大部分囊的直径 5 cm,且常并发气胸和乳糜胸.LAM的腹部CT表现:肾血管平滑肌脂肪瘤淋巴腺病淋巴管肌瘤乳糜腹2、都可伴发伴发肾脏的血管平滑肌脂肪瘤On the right is a simple renal cyst(arrow),and a semi-solid cyst瘤有残余.显微所见：肾血管平滑肌脂肪瘤，肾门淋巴结内同样可见错构瘤，滑肌细胞异常增殖导致支气管、淋巴管和小血管阻塞。On the left is a solid renal mass(arrowhead)which had n
4、ot increased in size and might represent Campbell-Walsh Urology,9th edition.滑肌而极少侵犯淋巴管和淋滑肌而极少侵犯淋巴管和淋Although a benign status is well accepted for AML,extrarenal occurrences have been reported in the hilar lymph nodes,retro-peritoneum,and liver,and direct extension into the venous sys-2、都可伴发伴发肾脏的血管平滑
5、肌脂肪瘤临床分析：肾血管平滑肌脂肪瘤，多中心发生，不为转移。累及器官:最常累及的是皮肤、神经系统和肾脏。1、多发生于育龄期妇女Lymph nodal involvement by renal angiomyolipoma1、多发生于育龄期妇女2、都可伴发伴发肾脏的血管平滑肌脂肪瘤On the right is a simple renal cyst(arrow),and a semi-solid cystOn the left is a solid renal mass(arrowhead)which had not increased in size and might represent手
6、术方式：根治性右肾切除，肾门淋巴结切除三类三类:伴有结节硬化症肾脏的血管平滑肌脂肪瘤伴有结节硬化症肾脏的血管平滑肌脂肪瘤一般介绍一般介绍:结节性硬化症(tuberous sclerosis complex,TSC)又称 Bournerille 病是以错构瘤为主要表现的累及多个系统和器 官的常染色体显性遗传性神经皮肤综合征。临床表现临床表现:色素减退斑、面部血管纤维瘤、鲨革样斑、癫痫、智力障 碍、肾血管肌脂肪瘤、肾囊肿，肾细胞癌，嗜酸细胞瘤。累及器官累及器官:最常累及的是皮肤、神经系统和肾脏。发病人群发病人群:青少年，无性别差异。发病特点发病特点:双肾多发发发 病病 率：率：4580%的TSC伴
7、有血管肌脂瘤结节性硬化症Figure 1 Axial CT(a)before and(b)after contrast enhancement.On the right is a simple renal cyst(arrow),and a semi-solid cyst(large arrow)thought to be an atypical angiomyolipoma as there was no growth over 2 years.On the left is a solid renal mass(arrowhead)which had not increased in siz
8、e and might represent a minimal fat or atypical angiomyolipoma.TSC（结节硬化症）（结节硬化症）LAM（淋巴管肌瘤病）（淋巴管肌瘤病）相同点相同点1、都可累及多个器官2、都可伴发伴发肾脏的血管平滑肌脂肪瘤不同点不同点1、多发于青少年,双侧多发2、常染色体显性遗传3、最常累及皮肤及神经系统4、除了伴发肾脏的血管平滑肌 脂肪瘤外，还可伴发肾脏的 嗜酸细胞瘤,肾囊肿，甚至是 肾癌.5、极少侵犯肺主要累及血管平 滑肌而极少侵犯淋巴管和淋 巴结,同时乳糜胸罕见。1、多发生于育龄期妇女2、最常累及肺，其次是 肾，预后不好 总结总结1、同一疾病
9、过程的不同阶段（a spectrum of a similar disease process.）2、LAM may occur sporadically or as part of TSC 病例一基本资料：40岁，女性，右上腹痛为主诉入院术前诊断：右肾癌，肾门淋巴结转移手术方式：根治性右肾切除，肾门淋巴结切除显微所见：肾血管平滑肌脂肪瘤，肾门淋巴结内同样可见错构瘤，包括平滑肌及脂肪细胞临床分析：肾血管平滑肌脂肪瘤，多中心发生，不为转移。预 后：随访8年未见复发及转移Lymph nodal involvement by renal angiomyolipomaInternational Jou
10、rnal of Urology(2000)7,7,386389病例二基本资料：女性，44岁，右侧腰背部间歇性酸痛1个月为主诉入院术前诊断：右肾癌，伴腹膜后淋巴结转移手术方式：根治性右肾切除，腹膜后淋巴结清扫术显微所见：右肾上极肿块为血管平滑肌脂肪瘤，淋巴结被未成熟的 平滑肌细胞侵润而引起淋巴结肿大，造成淋巴回流受阻临床诊断：淋巴管肌瘤病预 后：术后随访25个月，未有复发及转移肾淋巴肌瘤病一例报告中华泌尿外科杂志2007年7月7期基本资料:女性,30-40岁之间,左肾占位术前诊断:肾错构瘤手术方式:左肾切除术,术中因肿瘤巨大跨过中线且包绕腹主动脉,肿 瘤有残余.目前诊断:肺淋巴管肌瘤病预 后:术
11、后三个月复查肾CT左肾仍存在巨大肿瘤,并出现乳糜胸.病例三From CMU 2nd affiliated hospital基本资料:女,49岁,体检左肾占位 手术方式:根治性左肾切除术肾门淋巴结切除术病 理:左肾血管平滑肌脂肪瘤,淋巴结内可见血管平滑肌成分.目前诊断:淋巴管肌瘤病,尚未累及肺脏？病例四From home hospitaltem has been reported.Lymph nodal involvement by renal angiomyolipomaLymph nodal involvement by renal angiomyolipomaLymph nodal inv
12、olvement by renal angiomyolipoma1、多发生于育龄期妇女On the right is a simple renal cyst(arrow),and a semi-solid cyst滑肌细胞异常增殖导致支气管、淋巴管和小血管阻塞。Bournerille 病是以错构瘤为主要表现的累及多个系统和器LAM可同时累及肺和腹膜后淋巴结、肾脏、子宫等器官。4、除了伴发肾脏的血管平滑肌Lymph nodal involvement by renal angiomyolipoma1、多发生于育龄期妇女术前诊断：右肾癌，肾门淋巴结转移(large arrow)thought to
13、 be an atypical angiomyolipoma as there显微所见：肾血管平滑肌脂肪瘤，肾门淋巴结内同样可见错构瘤，On the right is a simple renal cyst(arrow),and a semi-solid cyst基本资料：女性，44岁，右侧腰背部间歇性酸痛1个月为主诉入院滑肌细胞异常增殖导致支气管、淋巴管和小血管阻塞。临床表现:色素减退斑、面部血管纤维瘤、鲨革样斑、癫痫、智力障On the right is a simple renal cyst(arrow),and a semi-solid cystAlthough a benign st
14、atus is well accepted for AML,extrarenal occurrences have been reported in the hilar lymph nodes,retro-peritoneum,and liver,and direct extension into the venous sys-Although a benign status is well accepted for AML,extrarenal occurrences have been reported in the hilar lymph nodes,retro-peritoneum,a
15、nd liver,and direct extension into the venous sys-tem has been reported.A uniformly benign clinical course in such cases has argued in favor of multicentric origin rather than metastasis.Campbell-Walsh Urology,9th edition.三类三类:伴有结节硬化症肾脏的血管平滑肌脂肪瘤伴有结节硬化症肾脏的血管平滑肌脂肪瘤Figure 1 Axial CT(a)before and(b)afte
16、r contrast enhancement.On the right is a simple renal cyst(arrow),and a semi-solid cyst(large arrow)thought to be an atypical angiomyolipoma as there was no growth over 2 years.On the left is a solid renal mass(arrowhead)which had not increased in size and might represent a minimal fat or atypical a
17、ngiomyolipoma.手术方式:根治性左肾切除术肾门淋巴结切除术基本资料:女,49岁,体检左肾占位(large arrow)thought to be an atypical angiomyolipoma as thereBournerille 病是以错构瘤为主要表现的累及多个系统和器LAM可同时累及肺和腹膜后淋巴结、肾脏、子宫等器官。平滑肌细胞侵润而引起淋巴结肿大，造成淋巴回流受阻Although a benign status is well accepted for AML,extrarenal occurrences have been reported in the hilar
18、 lymph nodes,retro-peritoneum,and liver,and direct extension into the venous sys-在这里我们将肾血管平滑肌脂肪瘤分成三类:On the right is a simple renal cyst(arrow),and a semi-solid cystLymph nodal involvement by renal angiomyolipomaLAM可同时累及肺和腹膜后淋巴结、肾脏、子宫等器官。手术方式:左肾切除术,术中因肿瘤巨大跨过中线且包绕腹主动脉,肿4、除了伴发肾脏的血管平滑肌4、除了伴发肾脏的血管平滑肌滑肌而
19、极少侵犯淋巴管和淋On the left is a solid renal mass(arrowhead)which had not increased in size and might representOn the right is a simple renal cyst(arrow),and a semi-solid cyst目前诊断:肺淋巴管肌瘤病瘤有残余.2、最常累及肺，其次是病例一基本资料：40岁，女性，右上腹痛为主诉入院术前诊断：右肾癌，肾门淋巴结转移手术方式：根治性右肾切除，肾门淋巴结切除显微所见：肾血管平滑肌脂肪瘤，肾门淋巴结内同样可见错构瘤，包括平滑肌及脂肪细胞临床分析：
20、肾血管平滑肌脂肪瘤，多中心发生，不为转移。预 后：随访8年未见复发及转移Lymph nodal involvement by renal angiomyolipomaInternational Journal of Urology(2000)7,7,386389目前诊断:淋巴管肌瘤病,尚未累及肺脏？1、多发生于育龄期妇女在这里我们将肾血管平滑肌脂肪瘤分成三类:手术方式：根治性右肾切除，肾门淋巴结切除(large arrow)thought to be an atypical angiomyolipoma as there临床分析：肾血管平滑肌脂肪瘤，多中心发生，不为转移。Lymph nodal
21、 involvement by renal angiomyolipomaBournerille 病是以错构瘤为主要表现的累及多个系统和器Lymph nodal involvement by renal angiomyolipoma一类:散发的偶发的局限于肾脏的血管平滑肌脂肪瘤Lymph nodal involvement by renal angiomyolipomaOn the right is a simple renal cyst(arrow),and a semi-solid cyst临床分析：肾血管平滑肌脂肪瘤，多中心发生，不为转移。Lymph nodal involvement b
22、y renal angiomyolipoma一般介绍:淋巴管平滑肌瘤(LAM)属全身性疾病，因淋巴管内的平一般介绍:结节性硬化症(tuberous sclerosis complex,TSC)又称LAM可同时累及肺和腹膜后淋巴结、肾脏、子宫等器官。一类:散发的偶发的局限于肾脏的血管平滑肌脂肪瘤tem has been reported.2、都可伴发伴发肾脏的血管平滑肌脂肪瘤4、除了伴发肾脏的血管平滑肌1、多发生于育龄期妇女(large arrow)thought to be an atypical angiomyolipoma as there滑肌而极少侵犯淋巴管和淋滑肌细胞异常增殖导致支气管
23、、淋巴管和小血管阻塞。基本资料：女性，44岁，右侧腰背部间歇性酸痛1个月为主诉入院Bournerille 病是以错构瘤为主要表现的累及多个系统和器术前诊断：右肾癌，肾门淋巴结转移A uniformly benign clinical course in such cases has argued in favor of multicentric origin rather than metastasis.From CMU 2nd affiliated hospital主要为要为双肺从肺尖至肺底呈弥漫分布的囊状改变,分布均匀或不均匀,薄壁,大部分囊的直径 5 cm,且常并发气胸和乳糜胸.Lymp
24、h nodal involvement by renal angiomyolipoma预 后：随访8年未见复发及转移滑肌而极少侵犯淋巴管和淋1、多发生于育龄期妇女was no growth over 2 years.2、最常累及肺，其次是was no growth over 2 years.滑肌而极少侵犯淋巴管和淋在这里我们将肾血管平滑肌脂肪瘤分成三类:在这里我们将肾血管平滑肌脂肪瘤分成三类:Lymph nodal involvement by renal angiomyolipoma手术方式：根治性右肾切除，腹膜后淋巴结清扫术手术方式：根治性右肾切除，肾门淋巴结切除On the right
25、is a simple renal cyst(arrow),and a semi-solid cyst手术方式：根治性右肾切除，腹膜后淋巴结清扫术三类:伴有结节硬化症肾脏的血管平滑肌脂肪瘤On the right is a simple renal cyst(arrow),and a semi-solid cyst手术方式：根治性右肾切除，腹膜后淋巴结清扫术a minimal fat or atypical angiomyolipoma.病 理:左肾血管平滑肌脂肪瘤,淋巴结内可见血管平滑肌成分.基本资料:女性,30-40岁之间,左肾占位Although a benign status is w
26、ell accepted for AML,extrarenal occurrences have been reported in the hilar lymph nodes,retro-peritoneum,and liver,and direct extension into the venous sys-瘤有残余.累及器官:病变可累及全身多个器官,以肺部最易受累。预 后：随访8年未见复发及转移On the right is a simple renal cyst(arrow),and a semi-solid cyst2、都可伴发伴发肾脏的血管平滑肌脂肪瘤滑肌细胞异常增殖导致支气管、淋巴管和小血管阻塞。平滑肌细胞侵润而引起淋巴结肿大，造成淋巴回流受阻A uniformly benign clinical course in such cases has argued in favor of multicentric origin rather than metastasis.
