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纵隔原发性脂肪肉瘤

Primary liposarcoma of the mediastinum.

作者信息

Greif J, Marmor S, Merimsky O, Kovner F, Inbar M

机构信息

Pulmonary Division Tel-Aviv Sourasky Medical Center and the Sackler Faculty of Medicine Tel-Aviv University Tel-Aviv Israel.

出版信息

Sarcoma. 1998;2(3-4):205-7. doi: 10.1080/13577149877993.

Abstract

Patient. A 62-year-old man presented with effort dyspnea, non-productive cough and weakness of 4 month duration. He had no findings on physical examination.Discussion. Chest X-ray revealed a large mass in the left anterior mediastinum. Computerized tomography of the chest showed a well-delineated homogeneous mediastinal mass with fat-equivalent density and a small pleural effusion. Fiberoptic bronchoscopy revealed narrowing of the left main bronchus, secondary to external compression. The bronchial mucosa was normal and brush cytology was negative. A CT-guided fine needle aspiration (FNA) of the mass yielded fragments of cells embedded in myxoid background material and closely packed atypical lipoblasts, compatible with liposarcoma. The patient underwent a left lateral thoracotomy and margibnal resection of the mass. The histopathological examination confirmed the diagnosis of mixed-type liposarcoma, consisted of myxoid and pleomorphic liposarcoma. Postoperative two-field radiation therapy was delivered to the mediastinum for a total midplane dose of 40 Gy. After a disease-free interval of 8 months the disease recurred in the mediastinum and pleura. Palliative chemotherapy achieved a short duration partial response but the patient succumbed to local recurrence 2 years after the diagnosis.

摘要

患者。一名62岁男性,出现劳力性呼吸困难、干咳及乏力4个月。体格检查未发现异常。讨论。胸部X线显示左前纵隔有一巨大肿块。胸部计算机断层扫描显示一个边界清晰的均匀纵隔肿块,密度与脂肪相当,并有少量胸腔积液。纤维支气管镜检查显示左主支气管因外部压迫而狭窄。支气管黏膜正常,刷检细胞学检查为阴性。对肿块进行CT引导下细针穿刺抽吸(FNA),获取了嵌入黏液样背景物质中的细胞碎片以及紧密排列的非典型脂肪母细胞,符合脂肪肉瘤表现。患者接受了左外侧开胸手术及肿块边缘切除术。组织病理学检查确诊为混合型脂肪肉瘤,由黏液样和多形性脂肪肉瘤组成。术后对纵隔进行了两野放射治疗,中平面总剂量为40 Gy。经过8个月的无病期后,疾病在纵隔和胸膜复发。姑息化疗取得了短期部分缓解,但患者在诊断后2年死于局部复发。

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