Chen Ming, Yang Jun, Zhu Lei, Zhou Cao, Zhao Heng
Department of Thoracic Surgery, Shanghai Chest Hospital, School of Medicine, Shanghai Jiao Tong University, 241 West HuanHai Road, Shanghai, China.
J Cardiothorac Surg. 2014 Jul 4;9:119. doi: 10.1186/1749-8090-9-119.
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.
原发性胸内脂肪肉瘤是一种极其罕见的恶性肿瘤，也是胸内肉瘤中罕见的组织学亚型。世界文献中相关报道相对较少。在本研究中，我们探讨了该肿瘤的临床病理特征及预后因素。
我们回顾性分析了2003年1月至2013年3月在上海交通大学附属胸科医院接受治疗的23例原发性胸内脂肪肉瘤患者的临床病理资料。根据肿瘤的不同位置，这些患者被分为三组，包括纵隔、胸膜和肺脂肪肉瘤。此外，这些患者可分为四种类型，包括高分化、黏液样、去分化和多形性脂肪肉瘤。分析了年龄、性别、肿瘤大小、肿瘤位置、肿瘤组织学类型及治疗对患者预后的影响。
不同脂肪肉瘤位置的患者生存率无显著差异。然而，不同脂肪肉瘤类型的患者生存率存在显著差异。与高分化型相比，黏液样、多形性和去分化型的无病生存期（DFS）较差（P = 0.038）。与高分化型相比，去分化、多形性和黏液样型的总生存期（OS）较差（P = 0.027）。根治性手术是OS的一个有利预后因素，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，在包括肿瘤大小、性别和年龄在内的其他临床参数方面，DFS和OS无显著差异。此外，放疗和/或化疗并不能改善接受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，放疗和化疗被认为对生存无效。因此，完全切除原发性胸内脂肪肉瘤以根治该病至关重要。
