中华医学杂志
中華醫學雜誌
중화의학잡지
National Medical Journal of China
2014年
12期
935-937
,共3页
黄钟明%李汉忠%纪志刚%肖河
黃鐘明%李漢忠%紀誌剛%肖河
황종명%리한충%기지강%초하
结节性硬化症%脂肪瘤%栓塞治疗
結節性硬化癥%脂肪瘤%栓塞治療
결절성경화증%지방류%전새치료
Tuberous sclerosis%Lipoma%Therapeutic embolization
目的 探讨双肾血管平滑肌脂肪瘤(RAML)伴结节性硬化症(TSC)的临床诊治特点.方法 分析10例RAML伴TSC患者的临床诊治资料,观察临床症状与病变的大小、部位,比较不同方法治疗后肿瘤的进展情况.患者男4例,女6例,年龄9～44岁,平均23.5岁.5例患者因腰痛、血尿等症状就诊;5例因肾外表现诊断为TSC,行影像学检查发现RAML.结果 10例患者为双侧多发RAML,并符合TSC临床诊断标准,RAML最大直径2～15 cm,平均(7.3±3.5) cm.6例患者存在单侧或双侧RAML破裂出血,肿瘤最大直径平均9.2 cm,其中4例行选择性动脉栓塞(SAE),2例行止血、抗炎、制动治疗;剩余4例肿瘤无局部症状者密切随访观察.随访9～116个月,4例行SAE治疗者中,1例1年后因再次出血复行SAE,剩余3例无症状复发,肿瘤最大直径平均每年增加1.5 cm,血肌酐平均每年增加3.1μmol/L.行保守治疗或观察的6例患者中,1例因并发肺淋巴管平滑肌瘤,3个月后死于呼吸功能衰竭,剩余5例肿瘤最大直径平均每年增加0.7 cm,血肌酐平均每年增加2.2 μmol/L.2例分别在随访18个月及40个月时出现腰痛等症状,1例经CT证实肿瘤出血行SAE,1例改予雷帕霉素治疗1年后肿瘤明显缩小.结论 合并有TSC的RAML多为双侧多发,在基本除外恶性可能后,SAE因其微创、效果良好且可重复进行,已逐渐成为治疗此类疾病的重要手段;在合并TSC的RAML的药物治疗方面,雷帕霉素显示出一定的疗效.
目的 探討雙腎血管平滑肌脂肪瘤(RAML)伴結節性硬化癥(TSC)的臨床診治特點.方法 分析10例RAML伴TSC患者的臨床診治資料,觀察臨床癥狀與病變的大小、部位,比較不同方法治療後腫瘤的進展情況.患者男4例,女6例,年齡9～44歲,平均23.5歲.5例患者因腰痛、血尿等癥狀就診;5例因腎外錶現診斷為TSC,行影像學檢查髮現RAML.結果 10例患者為雙側多髮RAML,併符閤TSC臨床診斷標準,RAML最大直徑2～15 cm,平均(7.3±3.5) cm.6例患者存在單側或雙側RAML破裂齣血,腫瘤最大直徑平均9.2 cm,其中4例行選擇性動脈栓塞(SAE),2例行止血、抗炎、製動治療;剩餘4例腫瘤無跼部癥狀者密切隨訪觀察.隨訪9～116箇月,4例行SAE治療者中,1例1年後因再次齣血複行SAE,剩餘3例無癥狀複髮,腫瘤最大直徑平均每年增加1.5 cm,血肌酐平均每年增加3.1μmol/L.行保守治療或觀察的6例患者中,1例因併髮肺淋巴管平滑肌瘤,3箇月後死于呼吸功能衰竭,剩餘5例腫瘤最大直徑平均每年增加0.7 cm,血肌酐平均每年增加2.2 μmol/L.2例分彆在隨訪18箇月及40箇月時齣現腰痛等癥狀,1例經CT證實腫瘤齣血行SAE,1例改予雷帕黴素治療1年後腫瘤明顯縮小.結論 閤併有TSC的RAML多為雙側多髮,在基本除外噁性可能後,SAE因其微創、效果良好且可重複進行,已逐漸成為治療此類疾病的重要手段;在閤併TSC的RAML的藥物治療方麵,雷帕黴素顯示齣一定的療效.
목적 탐토쌍신혈관평활기지방류(RAML)반결절성경화증(TSC)적림상진치특점.방법 분석10례RAML반TSC환자적림상진치자료,관찰림상증상여병변적대소、부위,비교불동방법치료후종류적진전정황.환자남4례,녀6례,년령9～44세,평균23.5세.5례환자인요통、혈뇨등증상취진;5례인신외표현진단위TSC,행영상학검사발현RAML.결과 10례환자위쌍측다발RAML,병부합TSC림상진단표준,RAML최대직경2～15 cm,평균(7.3±3.5) cm.6례환자존재단측혹쌍측RAML파렬출혈,종류최대직경평균9.2 cm,기중4례행선택성동맥전새(SAE),2례행지혈、항염、제동치료;잉여4례종류무국부증상자밀절수방관찰.수방9～116개월,4례행SAE치료자중,1례1년후인재차출혈복행SAE,잉여3례무증상복발,종류최대직경평균매년증가1.5 cm,혈기항평균매년증가3.1μmol/L.행보수치료혹관찰적6례환자중,1례인병발폐림파관평활기류,3개월후사우호흡공능쇠갈,잉여5례종류최대직경평균매년증가0.7 cm,혈기항평균매년증가2.2 μmol/L.2례분별재수방18개월급40개월시출현요통등증상,1례경CT증실종류출혈행SAE,1례개여뢰파매소치료1년후종류명현축소.결론 합병유TSC적RAML다위쌍측다발,재기본제외악성가능후,SAE인기미창、효과량호차가중복진행,이축점성위치료차류질병적중요수단;재합병TSC적RAML적약물치료방면,뢰파매소현시출일정적료효.
Objective To explore the clinical characteristics,diagnosis,treatment and outcomes of renal angiomyolipoma(RAML) associated with tuberous sclerosis complex (TSC).Methods The clinical data were analyzed for 10 cases of renal angiomyolipomas associated with tuberous sclerosis.There were 4 males and 6 females with a mean age of 23.5 (9-44) years.Five patients were admitted for flank pain or hematuria.RAML was found by TSC associated imaging examinations for those with extra renal organ involvement.Results Bilateral multiple renal angiomyolipomas were all confirmed on imaging tests and consistent with clinical TSC.The maximal diameter of RAMLs was from 2 to 15 (7.3 ± 3.5) cm.Six patients of tumor hemorrhage had an average maximal diameter of 9.2 cm.Selective arterial embolization (SAE) (n =4) and homeostasis,antiinflamation and immobilization therapy (n =2) were performed.Four asymptomatic cases accepted close observation.During a follow-up period of 9-116 months,one of 4 SAE cases underwent secondary SAE due to rebleeding 12 months later while there was no recurrence of flank pain in other 3 cases.The mean enlargement of maximal diameter was 1.5 cm/year and serum creatinine elevated by 3.1 μmol/L/year.Six cases on conservative medical therapy or under observation had a mean enlargement of maximal diameter of 0.7 cm/year and serum creatinine elevated by 2.2 μmol/L/year.Three cases remained asymptomatic,one died of respiratory failure from TSC-associated pulmonary lymphangioleiomyomatosis,two had flank pain at 16 and 40 months respectively,one underwent secondary SAE and another received rapamysin with marked tumor regression.Conclusions Bilateral multiple RAMLs occur in most TSC patients.After excluding malignancy,SAE becomes an important choice for its mini-invasiveness,efficacy and chances of re-embolization.And rapamysin provides ameliorative treatment for TSC patients.
