目的 总结原发性腹膜后脂肪肉瘤（primary retroperitoneal liposarcoma，PRL）的临床病理特征、诊断及治疗方法。 方法 回顾性分析经手术和活检证实的23例PRL患者的临床病理资料。结果 首发症状及体征表现为腹部肿块（91.3%，21/23），腹胀（56.5%，13/23）及腹痛（30.4%，7/23）。B超及CT的定位诊断准确率分别为66.7%（12/18）和85.7%（12/14）。首次手术肿瘤完整切除16例（69.6%），其中8例联合脏器切除； 部分切除3例（13.0%）； 仅行活检4例（17.4%）。PRL肿瘤完整切除术后复发率为75.0%（12/16）,该12例中再次手术8例（66.7%）可完全切除肿瘤。结论 CT是诊断PRL的重要手段，优于B超； 手术以完整切除肿瘤为主，对侵犯脏器者采用累及脏器一并切除； 术后复发者可再次手术。
目的 探讨乳腺脂肪肉瘤的临床病理特点及鉴别诊断。 方法 分析2010年3月收治的1例乳腺多形性脂肪肉瘤的临床表现、组织病理学特征及免疫表型特点，并复习相关文献。 结果 肿瘤由高级别多形性肉瘤和数量不等的多形性脂肪母细胞组成。免疫组织化学：肿瘤细胞呈S-100蛋白阳性表达、CD34灶性阳性表达，细胞角蛋白、上皮膜抗原、巨噬细胞表面抗原、结蛋白、平滑肌肌动蛋白、肌调节蛋白、肌浆蛋白、CD31均呈阴性表达。结论 乳腺脂肪肉瘤是一种少见的原发于乳腺的间叶源性肿瘤，诊断上应首先排除乳腺化生性癌和恶性叶状肿瘤伴脂肪肉瘤分化，应依据形态学特点和免疫组织化学结果进行鉴别。
【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.
ObjectiveTo analyze the ultrasonic manifestations of myxoid liposarcoma (MLS) in soft tissues.MethodsWe retrospectively analyzed the ultrasonic characteristics of 10 cases of soft tissue MLS which were pathologically confirmed between January and June 2006. Results Ultrasonic manifestations: 8 cases occurred in muscles and 2 in subcutaneous tissues and muscles; the maximum mass diameter was (71.1±39.4) mm; 10 cases had clear border, 8 had regular shape, and 9 had posterior echo enhancement; 10 cases were all solid masses with mucous liquid zone (9 cases had mainly weak echo, among which 5 had strong echo areas; 1 case had mainly strong echo); bloodstream grade: 3 cases belonged to level 0, 3 level Ⅰ, 2 level Ⅱ, and 2 level Ⅲ. Pathologic findings: 2 cases had low differentiation; 1 case was a mixture of MLS and atypical lipoma with mainly strong ultrasonic echo; the rest 7 cases had middle or high differentiation.ConclusionsUltrasonic manifestations of soft tissue MLS are mostly large masses in the muscular layer with clear border, regular shape, posterior echo enhancement and mucous liquid zone. Various degrees of strong echo zone exist in the masses. Bloodstream and mucous liquid zone are correlated with differentiation degree, and ultrasonic manifestations can be influenced if other factors exist.
ObjectiveTo understand the pathogenesis and the research progress of comprehensive treatment of primary retroperitoneal liposarcoma (PRLPS) and to provide evidence for clinical diagnosis and treatment.MethodThe recent literatures on the pathological classification, pathogenesis of PRLPS, and comprehensive treatment including the surgery, radiotherapy, chemotherapy, and molecular targeted therapy were reviewed.ResultsThe pathological types of PRLPS were highly differentiated, dedifferentiated, mucoid/round cell, polymorphic, and mixed. The main molecular pathogenesis was the synergistic effect of MDM2 with related genes, abnormal expressions of c-myc gene and microRNAs, Prune-nm23-H1 mechanism, and abnormal protein products of FUS-CHOP fusion gene which regulated the growth of tumor. The treatment of PRLRS included the radical resection, extended resection, and palliative resection combined with radiotherapy, chemotherapy, and molecular targeted therapy.ConclusionsPRLPS is a rare malignant tumor with high recurrence rate, but early diagnosis and treatment are difficult. With the further study of the molecular mechanism of PRLPS, the treatment of PRLPS has been transformed into a comprehensive treatment based on surgery, adjuvant radiotherapy and chemotherapy, and molecular targeted therapy.