【摘要】目的探讨肝血管平滑肌脂肪瘤的临床病理特点、诊断及鉴别诊断。 方法对3例肝血管平滑肌脂肪瘤患者有关病理检查结果进行回顾性分析。 结果肿瘤位于肝右叶2例，肝左叶1例。肿瘤直径为2～10 cm，平均6.2 cm。3例肿瘤内均见平滑肌、脂肪、畸形厚壁血管，但未见髓外造血灶。对黑色素瘤（HMB45）、结合蛋白（desmin)及肌动蛋白（actin)检查均呈阳性反应。术后随访6～36个月，未见肿瘤复发。结论肝血管平滑肌脂肪瘤由3种成分组成，病理形态变化多样，必须与多种肝肿瘤相鉴别。平滑肌细胞HMB45表达呈强阳性反应是诊断肝血管平滑肌脂肪瘤较可靠的依据。
Objective To investigate the CT imaging features of hepatic angiomyolipoma and its clinical characteristics. Methods The clinical, radiological, and pathological data of 3 hepatic angiomyolipoma (HAML) patients were retrospectively collected, and the imaging and diagnostic features were analyzed.Results All 3 cases were females, 2 complained of abdominal pain and one was absent of symptom. Both the laboratory examinations were normal in 2 cases, and hepatitis B virus markers were positive in one case. For CT features, the mass located in the superior anterior segment of the right hepatic lobe, in the posterior inferior segment of the right hepatic lobe, and in the left medial lobe, respectively. There were components of fat and soft tissue attenuation in these masses, and in which the CT value of the fat component was from -80 HU to -20 HU (mean -50 HU). There were enhancement in different degree and enhanced vascular imaging could be seen within the lesions. During surgery, all 3 masses were outgrowed from the hepatic lobes. Immunostainning showed HMB45 (+), αSMA (+), S100 (+)，which were bly suggestive of HAML.Conclusion HAML is a rare benign tumor with characteristic CT appearance, and its definite diagnosis relies on the immunohistochemical staining of HMB-45.
Objective?To evaluate the value of various CT features in differentiating renal angiomyolipoma (RAML) with minimal fat and renal cell carcinoma (RCC). Methods?The Cochrane Library, PubMed, MEDLINE (OVID), EMBase, and the Chinese Periodical Wed (CNKI, CBM, VIP) were searched. They were searched from Jan 2001 to Nov 2008. Trials screening, quality assessment, and data extraction was conducted according to the inclusion criteria recommended by the Cochrane Collaboration. The SROC curve and meta-analyses were performed by Meta-disc 1.4. Results?Seven trials, involving 482 patients and 513 tumors, were included. The studies were highly homogonous. It was considered that 8 features including single or multiple lesions, scanning density, calcification, angle with cortex, levering-cortex-up sign, lesions pro-trusion, homogeneous enhancement, and prolonged enhancement, played certain roles in differentiating RAML with minimal fat and RCC. Among these features, interface with the cortex was the most important, and the features of homogeneous enhancement and prolonged enhancement were the second most important. Conclusion?Besides measuring lipoid tissue in the tumor, there are another 8 features which are valuable to the differentiation of RAML with minimal fat and RCC.
:目的:探讨肾上腺髓样脂肪瘤的临床、影像学及病理特点。方法:回顾性分析28例肾上腺髓样脂肪瘤患者的临床资料,结合文献分析其特点。结果:28例患者肿瘤均经手术切除,并经病理检查证实为肾上腺髓样脂肪瘤,随访至今未见复发。结论:肾上腺髓样脂肪瘤的诊断主要依靠B超、CT和MRI,术前诊断困难,确诊需经病理学检查,手术切除是有效的治疗方法。
【摘要】 目的 探讨颈部对称性脂肪瘤的诊断和治疗。 方法 对2004年3月-2010年10月收治的5例颈项部脂肪瘤患者，其临床症状、体征、术前术后处理及结果等临床资料进行回顾。 结果 5例均为男性，以颈、项部大量皮下脂肪堆积为主要临床表现，其中1例伴有胸部上分皮下脂肪堆积，呈对称性隆起。3例患者伴阻塞性睡眠呼吸暂停低通气综合征，2例患者有睡眠打鼾但无明显呼吸暂停。4例患者有脂肪肝和长期酗酒史，但肝功能无异常改变，其中1例（1/4）有多次乙醇中毒史。1例患者无酗酒史，但诉经常作颈部刮痧治疗。5例均行外科手术切除，术中见肿瘤为白色无包膜脂肪组织。术后随访3个月~2年，1例术后1年复发，未行再次治疗，其余未见明显复发。 结论 颈部对称性脂肪瘤是脂肪组织弥漫性、对称性沉积于颈胸部皮下浅筋膜间隙和（或）深筋膜间隙的良性疾病。患者以中年男性居多，长期的酗酒史及典型的临床表现对于该病的诊断有一定帮助，但酗酒可能并非唯一病因。对于影响美观及功能的患者，其手术疗效较理想。【Abstract】 Objective To explore the diagnosis and treatment of symmetric lipomatosis in the neck. Methods We retrospectively analyzed the clinical manifestations, signs, preoperative and postoperative management, and the treatment outcome of five patients with symmetric lipomatosis hospitalized in the Department of Otolaryngology-Head and Neck Surgery of West China Hospital between March 2004 and October 2010. Results All the five patients are male with a large quantity of subcutaneous fat deposit in and around the neck. Among them, one patient demonstrated extending upper thorax mass in the form of symmetrical apophysis; three experienced obstructive sleep apnea hypoventilation syndrome, and two had the symptom of snoring without apnea. Four patients had a long history of alcohol abuse with fatty liver, but had no liver dysfunction. In these four patients, one had alcoholism for many times. One out of the five patients had no history of alcohol abuse, but said to have been treated by a traditional Chinese medical technique GUASHA. All the patients underwent resection surgery, during which a large amount of noncapsulated white adipose tissue was confronted. The duration of follow-up lasted from three months to two years. There was one case of recurrence one year after the surgery and the patient refused re-operation. No obvious recurrence was found in the rest of the group. Conclusions Symmetric lipomatosis is a benign lesion characterized by diffused and symmetric accumulation of adipose tissue in the superficial or deep fascia space in the cervico-thoracic region. It mainly takes place in the middle-aged people. Long history of alcohol abuse and typical clinical manifestations can help to reach the diagnosis, but alcoholism may not be the only cause in etiology. Surgery may be the feasible therapeutic modality up to now.
目的报告8例肝血管平滑肌脂肪瘤的影像学特征及病理学特点，探讨其诊断与治疗方法。方法对8例经手术及病理证实的肝血管平滑肌脂肪瘤的术前影像学表现（B超、CT、MRI、99mTcPMT）、手术情况及病理特点进行分析。结果B超： 肿瘤呈强回声光团6例，低回声光团2例； 边界清楚7例，血供丰富、内部回声不均匀4例，其中1例内部呈分隔网状结构。彩色Doppler超声示肿瘤血供丰富，均测及动脉频谱，阻力指数为0.4～0.5。静脉造影示肿瘤内血流信号明显增加。CT： 平扫示肿瘤呈低密度影7例，不均匀5例，边界清楚7例，肿瘤内见软组织影2例，脂肪成分2例。增强扫描示动脉期明显强化，门脉期及延迟期逐渐呈低密度。MRI: 肿瘤呈短T1、长T2信号，增强后强化明显，脂肪抑制后短T1变成长T1。99mTcPMT示肿瘤呈放射性增强，5 min相肿块区呈放射性缺损，2 h、5 h延迟相肿块区未见放射性填充，肝血池相肿块呈放射性填充。病理及免疫组化： 肿瘤由成熟的脂肪、血管及平滑肌组成，HMB45阳性。术前确诊3例。结论B超示强回声光团，CT呈低密度影中出现软组织影、脂肪成分，增强明显，MRI出现脂肪信号，脂肪抑制后短T1变成长T1，增强明显。99mTcPMT示肿瘤呈放射性增强，5 min相呈放射性缺损，延迟相未见放射性填充，肝血池相呈放射性填充。这些是肝血管平滑肌脂肪瘤的影像学特点，结合病史可作出诊断。此病应尽早手术治疗，行肝部分切除术。最后确诊依靠病理检查及免疫组化分析。