肝脏血管平滑肌脂肪瘤的临床病理分析

目的 探讨肝脏血管平滑肌脂肪瘤(HAML)的临床病理特点及诊治方法.方法 对1998年10月至2007年10月复旦大学附属中山医院肝脏外科收治的51例HAML患者的临床表现、病理特点、诊治及预后进行回顾性分析.结果 本组男性10例,女性41例,平均年龄44岁,症状以腹部肿块(33例)和腹部疼痛不适(15例)为主,少数表现为发热(2例).肿瘤由血管、平滑肌和脂肪细胞组成,免疫组织化学染色显示HMB-45(50/51)、SMA(47/49)和S-100(39/42)的阳性率较高.51例HAML均手术切除,平均住院时间13.8 d,术中平均出血263 ml,平均随访时间为55.4个月,无一例出现复发或转移.结论 HAML为肝脏少见良性间质瘤,确诊依赖于病理学及组织化学检查,HBM-45阳性肌细胞是重要的诊断标志.手术切除是最佳治疗手段,预后好.     

中国肝血管平滑肌脂肪瘤近23年的流行病学特征及诊治经验

目的探讨中国近23年肝脏血管平滑肌脂肪瘤(HAML)的流行病学特征和诊治经验。方法联合检索中国知网和维普网有关HAML的文献,获取相关信息,对患者的临床资料进行回顾性分析。结果中国自1987年7月至2010年12月共报道523例HAML,男女比例为1:2.63,平均年龄41.7岁,平均年龄在35~55岁之间者占总病例数的81.84%。76.42%的病例分布在华东、华北地区;66.92%无症状;明确诊断需依靠病理及免疫组织化学染色,肿瘤表达HMB-45、SMA、Vimentin、CD117阳性率分别为98.85%、96.10%、65.85%、40.71%。治疗方法以手术切除为主。结论 HAML多发于中青年女性;华东、华北地区病例最多;由于HAML临床表现无特异性、影像学表现多样,术前正确诊断率较低;大部分患者需依靠术后病理及免疫组化明确诊断,肿瘤细胞表达HMB-45、SMA阳性,是诊断HAML的可靠依据。手术切除为主要治疗手段,预后良好。     

肝血管平滑肌脂肪瘤的诊治分析

肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)为一少见的肝脏间质性肿瘤.1976年Ishak[1]首次报道该病.迄今国外文献共报道不超过200例[2].随着病理诊断水平的提高,近年来对其报道逐渐增多,但临床、病理医师对该肿瘤的认识还不够,致使误诊率可达50%.本文根据我院2007年诊断的3例HAML的临床病理特点与免疫组织化学特征,结合文献复习,进一步探讨HAML的诊治.     

肝血管平滑肌脂肪瘤的诊断和治疗

目的 探讨肝血管平滑肌脂肪瘤的临床表现、诊治特点及预后.方法 回顾性分析1989年至2007年我院收治的14例肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)患者的临床资料.结果 本组患者中男4例,女10例,中位年龄41岁.肿瘤位于肝左叶者6例,右叶8例.术前行B超检查者12例,CT枪查8例,MRI检查7例,血管造影2例,5例影像学检查后考虑HAML.1例术前行细针穿刺活检,未能确诊.14例均行手术切除,术后病理诊断明确,HMB-45检测14例,S-100检测7例,SMA检测6例,检测结果 均为阳性.术后随访6个月至18年(中位随访时间3年),除1例术后因DIC及心功能衰竭死亡外,其余13例无病生存.结论 肝血管平滑肌脂肪瘤发病率女性高于男性.影像学检奁有助于建立术前诊断,但确诊率不高.免疫组化HMB-45阳性是重要的病理诊断依据.手术切除是治疗肝血管平滑肌脂肪瘤的有效手段.     

肝血管平滑肌脂肪瘤5例临床病理观察

目的:研究肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)的形态学特点,探讨其诊断和鉴别诊断要点. 方法采用HE切片、光镜观察和免疫组织化学SP法染色对5例HAML进行分析. 结果5例HAML镜下见由厚壁的畸形血管、肌样细胞和脂肪组织混合组成,免疫组化瘤细胞表达HMB 45 、S-100和Desmin. 结论HAML 3种成分分布、比例及病理形态变化多样,HMB45阳性是诊断HAML 的重要指标之一.     

肝血管平滑肌脂肪瘤77例诊治分析

目的探讨肝血管平滑肌脂肪瘤(HAML)的临床、病理学特点及治疗方法和预后。方法回顾性分析上海东方肝胆外科医院2010年1月至2013年9月77例经手术后病理证实为HAML的病例的临床、影像学、病理和免疫组化结果。结果本组患者多为女性,术前肿瘤标记物AFP、CEA、CA19-9均阴性,术前影像学表现多样性,仅15例患者术前诊断为HAML,明确诊断依赖于术后病理及免疫组化结果。术后随访6~45个月,术后复发1例,复发患者再次手术治疗。结论 HAML术前易误诊,手术切除是有效的治疗方法,HAML有恶变可能,术后应密切随访。     

肝脏血管平滑肌脂肪瘤影像学及临床病理特征分析

目的：分析肝脏血管平滑肌脂肪瘤（hepatic angiomyolipoma,HAML）的影像学、病理学特征以及治疗和预后特点。
方法：回顾性分析2007—2009年5例HAML患者的临床病理资料。患者均行B超,CT或MRI检查,以肝脏实质性病变入院行肿块及部分肝脏切除术。
结果：影像学显示肿瘤形态规则、边界清楚。病理结果显示肿瘤均由不同比例的血管、平滑肌、脂肪组成,免疫组化结果HMB45和SMA均阳性表达,经常规病理及免疫组化确诊为HAML。术后6个月至1年随访未见复发及死亡。
结论：HAML为少见的肝脏肿瘤,肿瘤生物学行为以良性为主,由于其形态学表现多样,容易误诊为恶性,病理医师在诊断时应有HAML的概念,避免误诊。

     

肝脏血管平滑肌脂肪瘤的诊断与治疗

目的总结肝脏血管平滑肌脂肪瘤(HAML)的临床、影像学和病理学特点以及治疗方法。方法对1999年12月至2007年12月间我院治疗的110例HAML患者的临床资料进行回顾性分析。所有患者均行B超检查,多数患者同时行CT或MRI检查。所有患者均行肝切除术,经病理及免疫组化明确诊断并随访至今。结果 110例患者中女80例,男30例,年龄25～68岁,多数(86/110)无明显临床症状。肿瘤长径平均5.9 cm。B超多表现为不均质高回声光团;部分患者CT、MRI见动脉期病灶明显强化,门脉期仍有不同程度强化。病理学检查证实肿瘤由脂肪组织、厚壁血管及平滑肌细胞以不同比例构成。免疫组化结果HMB45(107/110)、SMA(109/110)、CD117(43/60)、CD34(40/60)表达阳性。所有病例随访无复发。结论由于HAML影像学表现多样,术前往往不易明确诊断,大部分患者通过术后病理及免疫组化明确诊断,HMB-45和SMA阳性是HAML的病理学特征。治疗上目前仍以手术切除肿瘤为首选,疗效满意。     

肝脏血管平滑肌脂肪瘤的诊断与治疗(26例经验总结)

目的总结肝脏血管平滑肌脂肪瘤(HAML)的临床、影像学及病理学特点以及治疗方法。方法回顾性分析了1998年10月至2003年1月作者单位手术治疗的26例HAML病人的临床资料。所有病人均行B超、彩色多普勒及CT检查，部分病例同时行MRI检查。所有病人均行肝切除术，诊断经病理及免疫组化证实并随访至今。结果26例病人中男性5例，女性21例，多无明显临床症状。B超多表现为不均质高回声光团，彩色多普勒显示点状、短线状彩色血流，注射造影剂后显示早期及延迟增强。CT显示动脉期增强，门脉期延迟强化。病灶由不同比例的厚壁血管、平滑肌细胞及脂肪混合组成。免疫组化结果HMB45(26／26)、A103(24／26)和SMA(24／26)表达阳性。所有病例手术切除随访无复发。结论多数HAML可经术前影像学检查获得诊断，HMB45、A103和SMA检测有助于确诊。肝切除术治疗HAML可获得满意效果。     

肝脏血管平滑肌脂肪瘤的诊断与治疗

目的 总结肝脏血管平滑肌脂肪瘤的临床、影像及病理特征,探讨提高术前确诊率的途径.方法 回顾性分析2000年至2007年第二军医大学东方肝胆外科医院收治并经病理检查证实的73例肝脏血管平滑肌脂肪瘤患者的影像学特点及诊治经验.根据影像学特点对患者进行分型并进行相应治疗.结果 术前确诊7例.B超检查诊断率为0,CT检查诊断率为13%(7/56),MRI检查诊断率为6%(2/33).根据影像学检查结果分型:血管瘤型6例,脂肪瘤型17例,平滑肌瘤型4例,混合型46例.手术切除72例,RFA治疗1例.24例患者出现肺部感染、胸腔和腹腔积液、轻度肝功能损害.术后免疫组织化学检测阳性率依次为:HMB45＞平滑肌肌动蛋白＞波形蛋白＞增殖细胞核抗原＞CD34＞癌胚抗原蛋白＞CD18＞CD19＞p53.1例术后因复发死亡.结论 肝且庄血管平滑肌脂肪瘤易误诊,影像学分型有助于术前正确诊断,治疗上应积极手术切除.     

Hepatic angiomyolipoma: A retrospective study of 25 cases

PURPOSE: We report our experience of diagnosing and treating hepatic angiomyolipoma (HAML), a rare benign mesenchymal tumor. METHODS: We analyzed retrospectively the clinicopathologic, radiological, and operative data of 25 patients who underwent surgery for HAML at our institute between November 2001 and May 2006. RESULTS: Most patients (20/25) were asymptomatic and had normal liver function. Ultrasonography (US) showed a heterogeneous hyperechoic mass in 13 of 23 patients, precontrast computed tomography (CT) showed that all of 12 lesions scanned were hypodense, and magnetic resonance imaging (MRI) showed hypointensity on T1-weighted images and hyperintensity on T2-weighted images in most (5/6) lesions. Marked enhancement in the arterial phase was seen in 10 of 12 lesions on CT scans and in 6 of 6 lesions on MRI scans. All tumors were composed of varying proportions of smooth muscle, adipose tissue, and blood vessels, and showed positive immunohistochemical staining for HMB-45. All patients underwent partial hepatectomy and there was no evidence of recurrence after a median follow-up of 43 months. CONCLUSION: The radiological features of HAML vary according to its histological components. The definitive diagnosis of HAML is challenging and depends on the presence of HMB-45-positive myoid cells. Hepatic angiomyolipoma is treated effectively with surgery and the prognosis is good.     

肝脏血管平滑肌脂肪瘤诊断与外科治疗：附19例报告

背景与目的 肝脏血管平滑肌脂肪瘤（HAML）是肝脏少见的一种间叶组织来源的良性肿瘤,影像学检查缺乏特异性,术前诊断率低,容易误诊为肝细胞癌或其他肝良性肿瘤,本文报告19例HAML患者的诊治过程,以期为临床提供参考和借鉴。方法 回顾性分析2011年1月—2019年12月收治的19例HAML患者临床资料,其中女12例,男7例;年龄28~61岁;体检发现无临床症状者14例,表现为上腹部隐痛者3例,间断腹胀者1例,腹痛伴腹泻者1例;1例合并慢性乙型病毒性肝炎及肺脓肿,1例合并乙型肝炎肝硬化代偿期。19例均不伴肾肺等其他脏器血管平滑肌脂肪瘤,均无结节性硬化症。肿瘤直径1.3~12 cm,平均直径（4.6±2.2）cm。AFP、CA19-9、CEA均正常。7例患者术前诊断肝细胞癌（36.8%）,12例术前诊断肝良性肿瘤（63.2%）,分别为7例诊断肝腺瘤,2例诊断炎性假瘤,1例诊断肝海绵状血管瘤,1例诊断肝局灶性结节性增生,1例术前行超声引导下肝脏穿刺活检病理证实HAML。结果 患者均行外科手术治疗,18例行肝切除治疗,1例行超声引导下肝肿瘤穿刺活检术、经皮穿刺肝肿瘤射频消融术。平均手术时间（172.7±80.4）min,术中平均出血量为（456.6±528.1）mL,平均术后住院时间（9.8±2.7）d。19例患者病理检查均证实HAML,其中7例为上皮样血管平滑肌脂肪瘤,免疫组化检查HMB-45、SMA均阳性表达。患者术后恢复良好,所有患者无肝衰竭、腹腔出血、胆汁漏等并发症发生,无死亡病例。全部病例均获随访,均未发现肿瘤复发及转移。术后患者有良好的生活质量。结论 HAML瘤属于良性肿瘤,术前诊断比较困难,一部分患者容易误诊为肝细胞癌,最终诊断依靠病理及免疫组织化学染色,外科手术切除是安全、有效的治疗选择,预后良好。     

Diagnosis and treatment of hepatic angiomyolipoma

Background

Hepatic angiomyolipoma (HAML) is a rare liver tumor. This paper summarized the clinical, radiological and pathological features of HAML.

Methods

Seventeen cases of HAML were analyzed retrospectively. All patients were subjected to surgical resection of tumor, one of which was performed emergency surgery because of hemorrhage of tumor.

Results

There are 13 females and 4 males, most of whom were asymptotic except 4 had minimal abdominal discomfort. US, CT and/or MRI were taken and corresponding data was comprehensively analyzed with other clinical signs and symptoms. Correct preoperative diagnosis was able to be achieved in 9 patients. Pathological analysis and immunohistochemistry of HMB-45 was used as final diagnosis. All patients were followed up and survived without recurrence.

Conclusions

Preoperative diagnosis of HAML can be benefited from comprehensive analysis of clinical manifestations. The malignant potential and fast growth of tumor suggested surgical removal of tumor while it was diagnosed.Key Words: Hepatic tumor, angiomyolipoma, diagnosis, surgery     

肝血管平滑肌脂肪瘤的外科治疗

目的 探讨肝血管平滑肌脂肪瘤的临床表现、病理学特征、诊断和外科治疗.方法 回顾性分析我院1990年1月至2006年12月诊治的22例肝血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)患者的临床资料.结果 22例患者中男6例,女16例,平均年龄48.2岁.17例患者无临床症状,所有患者均无肝炎病史,均无肾血管平滑肌脂肪瘤病史.女性患者无口服避孕药史.血AFP、CEA、CA19-9、CA242均正常.术前超声、CT、MRI诊断准确率分别为21%(4/19)、23.5%(4/17)、25%(2/8).肿瘤均为单发,直径4～17 cm,均无完整包膜.22例患者均行手术切除,17例行肝肿瘤不规则切除术,5例行左半肝切除术.1例患者术后第2天死于并发症,1例患者出现轻微胆漏,术后病理免疫组化HMB45均表达阳性,4例见髓外造血明显.随访率91%(20/22),随访时间6个月至17年,术后无肿瘤复发.结论 肝血管平滑肌脂肪瘤无特异性临床症状,术前影像学检查难以确诊,应积极的手术治疗.     

肝血管平滑肌脂肪瘤13例报告

目的 探讨肝血管平滑肌脂肪瘤的临床表现和预后.方法 回顾性分析经手术治疗的13例肝血管平滑肌脂肪瘤病人的临床资料.结果 该肿瘤缺乏特征性临床症状,影像学表现多样,术前仅1例诊断肝脏血管平滑肌脂肪瘤.病理诊断需依赖标本免疫组化指标HMB45阳性.结论 该肿瘤术前容易误诊,首选手术治疗,预后良好.     

Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa

In this study, we investigated HMB-45 expression in epithelioid uterine leiomyosarcomas with clear cell areas. From 12 epithelioid leiomyosarcomas, we selected 5 that had: 1) clear cell areas and 2) spindle cell areas that were at least focally positive for desmin and caldesmon. The patients' ages ranged from 47 to 82 years (mean 64 years). Presenting symptoms were uterine bleeding (three), abdominal pain (one), and a pelvic mass (one). There was no history of tuberous sclerosis or lymphangioleiomyomatosis. One patient had stage II disease, one stage III, and three stage IV. All were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Two received radiotherapy, and three were also treated with chemotherapy. The tumors ranged in size from 4 x 3 x 3 cm to 10 x 7 x 6 cm; all had significant cellular atypia, areas of coagulative necrosis, and between 10 and 90 mitoses per 10 high power fields. Vascular invasion was seen in three cases. The epithelioid component varied from 50% to 90% in each case; and the percentage of clear cells was < 1% in one case, 5% in one case, and 10% to 80% in three cases. Smooth muscle actin and desmin were positive in all cases. Four cases were positive for HMB-45 only in the clear cell areas. The tumor with < 1% of clear cells was negative for HMB-45. All were negative for S-100 and c-kit. Three patients died of disease at 9, 30, and 32 months; one patient is alive with progressive disease at 6 months, and one patient (stage II disease) is alive with no evidence of disease at 8 months. Unequivocal uterine epithelioid leiomyosarcomas may have clear cells positive for HMB-45. These tumors might belong to the group of lesion designated as PEComas; however, it is advisable to designate them as uterine leiomyosarcomas. In uterine smooth muscle tumors, some epithelioid cells most likely undergo clear cell changes and become positive for HMB-45. It would be advisable to perform this stain in all epithelioid smooth muscle tumors of the uterus.