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前纵隔和胸腺脂肪肉瘤。28例临床病理研究

Liposarcoma of the anterior mediastinum and thymus. A clinicopathologic study of 28 cases.

作者信息

Klimstra D S, Moran C A, Perino G, Koss M N, Rosai J

机构信息

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

出版信息

Am J Surg Pathol. 1995 Jul;19(7):782-91. doi: 10.1097/00000478-199507000-00006.

Abstract

We studied 28 cases of anterior mediastinal liposarcoma occurring in 16 males and 12 females with a mean age of 43 years (range, 14-72). Presenting symptoms included dyspnea (four cases) and chest pain (four cases), although 11 tumors were detected incidentally by routine chest radiography. Seven cases were believed to be located within the thymus. Most (i.e., 25) of the cases were of low grade, with the well-differentiated lipoma-like or sclerosing subtypes constituting 60% and the myxoid subtype constituting 28%; the remaining 12% exhibited mixed features. Three cases were pleomorphic type. Several low-grade tumors exhibited widespread, dense aggregates of mature-appearing lymphocytes and plasma cells, which occasionally obscured the mesenchymal nature of the neoplasm, suggesting instead a lymphoid neoplasm or a reactive fibroinflammatory condition. The three high-grade tumors showed combinations of pleomorphic and round cell patterns, with focal myxoid areas. Of the cases grossly arising within the thymus, only one showed extensive thymic tissue within the lesion ("thymoliposarcoma"); six others exhibited residual thymus peripheral to the tumor. Clinical follow-up in 23 cases revealed recurrence in seven patients (31.8%), with a mean interval to recurrence of 3 years. Eight patients died (mean survival, 2.6 years), one postoperatively and three following a recurrence. Fifteen patients were alive (mean survival, 2 years), four with recurrent tumor. The myxoid tumors had a somewhat more aggressive course than the well-differentiated tumors. Metastases were not observed in any of the patients.

摘要

我们研究了28例前纵隔脂肪肉瘤,其中男性16例,女性12例,平均年龄43岁(范围14 - 72岁)。主要症状包括呼吸困难(4例)和胸痛(4例),不过11例肿瘤是在常规胸部X线检查时偶然发现的。7例被认为位于胸腺内。大多数病例(即25例)为低级别,其中高分化脂肪瘤样或硬化亚型占60%,黏液样亚型占28%;其余12%表现为混合特征。3例为多形性类型。一些低级别肿瘤可见广泛、密集的成熟淋巴细胞和浆细胞聚集,偶尔掩盖了肿瘤的间叶性质,提示为淋巴样肿瘤或反应性纤维炎性病变。3例高级别肿瘤表现为多形性和圆形细胞模式的组合,并伴有局灶性黏液样区域。在大体上起源于胸腺内的病例中,只有1例在病变内显示有广泛的胸腺组织(“胸腺脂肪肉瘤”);其他6例在肿瘤周边可见残留胸腺。23例患者的临床随访显示,7例(31.8%)复发,复发平均间隔时间为3年。8例患者死亡(平均生存2.6年),1例术后死亡,3例复发后死亡。15例患者存活(平均生存2年),4例有复发性肿瘤。黏液样肿瘤的病程比高分化肿瘤略具侵袭性。所有患者均未观察到转移。

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