【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。
Hepatic angiomyolipoma (HAML) is a rare benign mesenchymal tumor of the liver, which has highly variable imaging appearances, often leads to missed diagnosis and misdiagnosis. The images of 2 patients with HAML confirmed by pathology were presented in this study, and the typical imaging features of the HAML, the underlying pathophysiological mechanism, and the differential diagnosis were briefly summarized so as to deepen the understanding of HAML and to improve the diagnosis and differential diagnosis abilities of HAML, then reduce the rates of missed diagnosis and misdiagnosis of the HAML.
: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。
ObjectiveTo summarize clinicopathologic and immunophenotypic features of hepatic epithelioid angiomyolipoma (HEAML) and to explore its diagnostic and differential diagnostic methods.MethodThe clinical and imaging manifestations, pathological morphology and immunohistochemical features of 5 patients with HEAML from August 2011 to December 2017 in this hospital were retrospectively analyzed.ResultsThere were 2 males and 3 females in the 5 patients with HEAML, aged 38–64 years with an average age of 50 years. There were 2 cases of the left lobe tumors and 3 cases of the right lobe tumors. Three cases were diagnosed as the hepatocellular carcinoma and the other two cases were diagnosed as the hepatic hamartoma and (or) hemangioma by the preoperative imaging examination. The diameter of tumors ranged from 1.5 cm to 7.0 cm, with an average of 3.6 cm. Microscopically, the tumors were composed of more epithelioid smooth muscle cells, parenchyma vessels and a small amount of fat. The immunohistochemical results showed that the melan-A, HMB45, and SMA were positive, while the HepPar-1, AE1/AE3, EMA, CD117, Dog-1, CD10, CgA, Syn, and Desmin were negative. The Ki-67 proliferation index was 2%–10%. The patients were all alive without the tumor recurrence after following up for 2–76 months with an average of 31.4 months.ConclusionsHEAML is a rare primary mesenchymal tumor of liver, which should be misdiagnosed for other benign or malignant tumors for influencing clinical treatment. Diagnosis and differential diagnosis can be made by histopathology and immunohistochemical staining.
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.
Cardiac lipoma is rare and benign entities of heart, and often discovered after autopsy incidentally because most patients remain completely asymptomatic. The symptoms of cardiac lipoma depend on their location and size within the heart, such as dyspnea, chest pain, arrhythmia, and even sudden death. Surgical interventions usually have good results. Up till now, there has been no any relevant large-scale randomized controlled trial, and even no precise guideline for treatment. Surgical procedures often depend on patients' clinical manifestations and changes of hemodynamics in cardiac vessels in order to relief the symptoms as well as abort the progress of the disease. Therefore, early diagnosis and close follow-up are necessary for timely treatment. This article aims to summarize the imageological examinations for cardiac lipoma, including echocardiography, computed tomography, magnetic resonance imaging along with their characteristics and advantages, in order to get better clinical strategies.
【摘要】 目的 探讨肝脏血管平滑肌脂肪瘤（hepatic angiomyolipoma，HAML）的多层螺旋CT影像学表现特征及其与病理学基础的相关性，以进一步提高CT诊断的准确性。 方法 收集2008年11月-2010年12月经手术病理证实的16例HAML患者。所有患者均行螺旋CT平扫及动脉期、门脉期增强检查，重点观察HAML的分型及其相应CT表现及影像-病理的相关性。 结果 16例患者共20个病灶，19个为稍低密度病灶，其中11个病灶内可见明显的脂肪密度影；1个为稍高密度病灶。动脉期所有病灶均有不同程度的强化表现，15个病灶内可见到较明显条状及扭曲的血管影。门脉期15个病灶有持续强化。 结论 多层螺旋CT能准确反映HAML的分型及其病理特征，对临床表现不典型患者的诊断和鉴别诊断有较大诊断价值。【Abstract】 Objective To discuss the correlation between the features of multislice spiral CT results for hepatic angiomyolipoma (HAML) and their pathological basis, and to further improve the diagnostic accuracy through CT examination. Methods Sixteen HAML patients diagnosed pathologically between November 2008 and December 2010 in our hospital were enrolled in our study. All patients underwent multi-slice spiral CT scanning of pre-and post-contrast arterial phase, and portal venous phase. Focus was put on observation of HAML types and their corresponding manifestations, and the correlation between CT imaging and the pathologic basis. Results There were 20 lesions in the 16 patients. Among the 19 hypodense lesions, 11 were clearly seen with fat density shadow. One out of the 20 lesions showed as slightly hyperdense. On the arterial phase scanning, all lesions showed enhancement, and obvious vascular shadow could be seen in15 lesions. On the portal venous phase, 15 lesions continued to strengthen. Conclusions Multi-slice spiral CT can accurately reflect the classification of HAML and its pathological features. It has a great value in the diagnosis and differential diagnosis of patients without typical clinical manifestations.
【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.