Abstract

BackgroundPrimary 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.MethodsWe 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.ResultsThere 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.ConclusionsThe 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.

Highlights

  • Primary intrathoracic liposarcoma is an extremely rare malignancy as well as a rare histologic subtype of intrathoracic sarcoma

  • There were no significant differences for disease-free survival (DFS) and OS in other clinical parameters including tumor size, gender and age (Table 3)

  • Our study demonstrates that the most common histologic types of intrathoracic liposarcoma appear to be well-differentiated (34.78%, 8/23) and myxoid cases (34.78%, 8/23), which is consistent to reports of the literatures

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Summary

Introduction

Primary intrathoracic liposarcoma is an extremely rare malignancy as well as a rare histologic subtype of intrathoracic sarcoma. We explored the clinicopathologic features and prognostic factors of this tumor in this study. Primary intrathoracic liposarcoma is Liposarcoma has been subclassified histologically into well-differentiated, myxoid, pleomorphic, and dedifferentiated types according to the 2012 NCCN classification of liposarcoma. Mediastinal liposarcomas are extremely rare, making up from 0.1%–0.75% of all mediastinal tumors [3]. The pleural and pulmonary liposarcoma are more scarce location. The prognosis of this group sarcomas for distinct histological subtypes and different locations is still unclear. We carried out this study to explore the correlation between the survival and distinct tumor locations/histological types of this rare malignancies for the first time

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