Primary intrathoracic liposarcoma: a clinicopathologic study and prognostic analysis of 23 cases.

Primary intrathoracic liposarcoma: a clinicopathologic study and prognostic analysis of 23 cases.
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DOI:
10.1186/1749-8090-9-119
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发表时间:
2014-07-04
影响因子:
1.6
通讯作者:
Zhao H
Zhao H
中科院分区:
医学4区
文献类型:
--
作者:
Chen M;Yang J;Zhu L;Zhou C;Zhao H

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原发性胸内脂肪肉瘤是一种极为罕见的恶性肿瘤,也是胸内肉瘤的一种罕见的组织学亚型。在世界文献中出现的报道相对较少。我们在本研究中探讨了该肿瘤的临床病理特征和预后因素。我们回顾性分析2003年1月至2013年3月在交通大学附属上海胸科医院治疗的23例原发性胸内脂肪肉瘤的临床病理资料。根据不同的肿瘤部位将患者分为纵隔、胸膜和肺脂肪肉瘤三组。脂肪肉瘤可分为高分化型、黏液型、去分化型和多形性4种类型。分析年龄、性别、肿瘤大小、肿瘤部位、肿瘤组织学类型及治疗对患者预后的影响。不同脂肪肉瘤部位的生存率无显著差异。然而,在不同类型的脂肪肉瘤中,生存率有显著差异。黏液型、多形型和去分化型与高分化型相比无病生存期(DFS)较差(P = 0.038)。去分化型、多形型和黏液型的总生存期(OS)低于分化良好型(P = 0.027)。根治性手术是预后OS的有利因素,根治性手术组的OS优于非根治性手术组(P = 0.029)。值得注意的是,DFS和OS在肿瘤大小、性别和年龄等其他临床参数上无显著差异。此外,放疗和/或化疗不能改善非根治性手术或复发患者的预后。组织学类型和根治性手术是影响脂肪肉瘤行为和预后的因素。一般来说,放疗和化疗被认为是对生存无效的治疗方式。因此,完全切除原发性胸内脂肪肉瘤是根治该病的必要措施。
Primary intrathoracic liposarcoma is an extremely rare malignancy as well as a rare histologic subtype of intrathoracic sarcoma. Relatively few reports appear in the world literatures. We explored the clinicopathologic features and prognostic factors of this tumor in this study. We retrospectively analyzed the clinicopathological data of 23 patients with primary intrathoracic liposarcoma who were treated in Shanghai chest Hospital affiliated to Jiao Tong University, from January 2003 to March 2013. These patients were classified into three groups according to the distinct tumor locations, including mediastinum, pleura and lung liposarcoma. Also, these patients could be divided into four types, including well-differentiated, myxoid, dedifferentiated and pleomorphic liposarcoma. The influences of age, sex, tumor size, tumor location, tumor histologic type and therapy on the prognosis of the patients were analyzed. There were no significant difference for survival among distinct liposarcoma locations. However, significant difference for survival among distinct liposarcoma types were observed. Poor disease-free survival (DFS) was observed in the myxoid, pleomorphic and dedifferentiated types as compared to well-differentiated type (P = 0.038). Inferior overall-survival (OS) was observed in dedifferentiated, pleomorphic and myxoid types relative to well-differentiated type (P = 0.027). The radical surgery was a favorable prognostic factor for OS, as demonstrated by the better OS of the radical surgery group as compared to that of the non-radical surgery group ( P = 0.029). Notably, there were no significant differences for DFS and OS in other clinical parameters including tumor size, gender and age. In addition, radiotherapy and/or chemotherapy could not improve the prognosis of the patients receiving non-radical surgery or suffering from relapse. The histological type and the radical surgery are the factors that influence the behavior and prognosis of liposarcoma. In general, radiotherapy and chemotherapy are believed to be ineffective therapeutic modalities for survival. So it is essential to completely resect the primary intrathoracic liposarcoma as radical cure of the disease.
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发表时间: 2000-07-01
期刊: RADIOGRAPHICS
影响因子: 5.5
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发表时间: 1997-07-01
期刊: LUNG CANCER
影响因子: 5.3
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发表时间: 1964-01-01
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DOI: 10.1002/jso.2930190414
发表时间: 1982-01-01
影响因子: 2.5
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