目的 探討乳腺脂肪肉瘤的臨床病理特點及鑒別診斷。 方法 分析2010年3月收治的1例乳腺多形性脂肪肉瘤的臨床表現、組織病理學特征及免疫表型特點，并復習相關文獻。 結果 腫瘤由高級別多形性肉瘤和數量不等的多形性脂肪母細胞組成。免疫組織化學：腫瘤細胞呈S-100蛋白陽性表達、CD34灶性陽性表達，細胞角蛋白、上皮膜抗原、巨噬細胞表面抗原、結蛋白、平滑肌肌動蛋白、肌調節蛋白、肌漿蛋白、CD31均呈陰性表達。結論 乳腺脂肪肉瘤是一種少見的原發于乳腺的間葉源性腫瘤，診斷上應首先排除乳腺化生性癌和惡性葉狀腫瘤伴脂肪肉瘤分化，應依據形態學特點和免疫組織化學結果進行鑒別。
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.
【摘要】 目的 探討頸部對稱性脂肪瘤的診斷和治療。 方法 對2004年3月-2010年10月收治的5例頸項部脂肪瘤患者，其臨床癥狀、體征、術前術后處理及結果等臨床資料進行回顧。 結果 5例均為男性，以頸、項部大量皮下脂肪堆積為主要臨床表現，其中1例伴有胸部上分皮下脂肪堆積，呈對稱性隆起。3例患者伴阻塞性睡眠呼吸暫停低通氣綜合征，2例患者有睡眠打鼾但無明顯呼吸暫停。4例患者有脂肪肝和長期酗酒史，但肝功能無異常改變，其中1例（1/4）有多次乙醇中毒史。1例患者無酗酒史，但訴經常作頸部刮痧治療。5例均行外科手術切除，術中見腫瘤為白色無包膜脂肪組織。術后隨訪3個月~2年，1例術后1年復發，未行再次治療，其余未見明顯復發。 結論 頸部對稱性脂肪瘤是脂肪組織彌漫性、對稱性沉積于頸胸部皮下淺筋膜間隙和（或）深筋膜間隙的良性疾病。患者以中年男性居多，長期的酗酒史及典型的臨床表現對于該病的診斷有一定幫助，但酗酒可能并非唯一病因。對于影響美觀及功能的患者，其手術療效較理想。【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.
目的 總結原發性腹膜后脂肪肉瘤（primary retroperitoneal liposarcoma，PRL）的臨床病理特征、診斷及治療方法。 方法 回顧性分析經手術和活檢證實的23例PRL患者的臨床病理資料。結果 首發癥狀及體征表現為腹部腫塊（91.3%，21/23），腹脹（56.5%，13/23）及腹痛（30.4%，7/23）。B超及CT的定位診斷準確率分別為66.7%（12/18）和85.7%（12/14）。首次手術腫瘤完整切除16例（69.6%），其中8例聯合臟器切除； 部分切除3例（13.0%）； 僅行活檢4例（17.4%）。PRL腫瘤完整切除術后復發率為75.0%（12/16）,該12例中再次手術8例（66.7%）可完全切除腫瘤。結論 CT是診斷PRL的重要手段，優于B超； 手術以完整切除腫瘤為主，對侵犯臟器者采用累及臟器一并切除； 術后復發者可再次手術。