Clinicopathological Features of Intrathoracic Liposarcoma—A Systematic Review with an Illustrative Case

Abstract

Background: Liposarcoma (LPS) is one of the most common soft-tissue sarcomas. However, intrathoracic LPS is rare, as only 1% of all LPS cases are found in the thorax. Methods: A systematic literature review through PubMed and Embase databases was performed. Only eligible case reports and case series reporting intrathoracic LPS in adult patients were included. Kaplan–Meier curves were calculated to evaluate the survival rate of included patients based on the histological subtype of LPS. Results: 123 studies reporting 197 patients were included. We added a case of a 69-year-old female patient with recurrent giant intrathoracic LPS. The primary tumor measured 15.1cm × 22.9 cm × 21.9 cm and weighed 3100 g. Six months later, the patient was admitted to the hospital with another intrathoracic tumor measuring 9.5 cm × 9 cm× 1.4 cm. The immunohistochemical studies showed expression of murine double minute 2 (MDM2) antigen in both primary and recurrent tumor cells. Conclusions: Dyspnea, chest pain, and cough were the most common symptoms reported in included studies. Overall, the 5-year survival rate was 62%. The highest survival was observed in well-differentiated LPS patients (80%) and the lowest in myxoid LPS (31%).

Resection of a giant mediastinal liposarcoma by median sternotomy with vascular reconstruction—a case report

“…Deregulation and overexpression of MDM2, CDK4 promoting tumorigenesis by dysregulation of apoptotic p53 pathway ( 1 ). The amplification of these two genes is accepted as a diagnostic criterion for distinguishing well-differentiated liposarcoma from lipoma which can be a diagnostic pitfall, as our presented case has shown ( 1 , 6 , 16 ). Chemoresistance and low radiosensitivity of tumors arising from mesenchymal origin favor surgical approach as the only option for radical treatment ( 17 ).…”

“…Liposarcoma is a very rare malignancy of mesenchymal origin, firstly reported by Virchow in 1857 ( 8 ). To the best of our knowledge, less than 200 cases of intrathoracic liposarcoma have been so far published in the literature ( 6 ). The symptomatology of the disease is nonspecific and conditioned by compression or invasion of adjacent structures, such as cough, dyspnea, dysphagia, superior vena cava syndrome or progressive heart failure.…”

“…The World Health Organization (WHO) classification divides liposarcoma into 4 subtypes: well-differentiated, dedifferentiated, myxoid/round cell and pleomorphic ( 16 ). Dedifferentiated and pleomorphic subtype have a poor prognosis ( 6 ). Like well-differentiated liposarcoma dedifferentiated Liposarcoma show amplification of CDK4 and MDM2.…”

“…These tumours are locally aggressive with risk of recurrence but good prognosis as metastases is rare ( 5 ). Due to its anatomical location the tumor is commonly associated with infiltration of surrounding structures ( 6 ). These tumors are often diagnosed as incidental findings without any symptoms.…”

Imaging features and literature review of rare primary giant liposarcoma of the esophagus

“…Liposarcoma arises from primitive mesenchymal cells and immature adipocytes. As per the soft tissue classification outlined by the World Health Organization (WHO) in 2013, liposarcoma encompasses several subtypes [7][8]: well-differentiated liposarcoma, myxoid liposarcoma, dedifferentiatedarcoma, and pleomorphic liposarcoma.-differentiated liposarcoma and myxoid liposarcoma are categorized as low-grade malignant tumors, characterized by a low metastasis rate but a high of local recurrence Conversely, dedifferentiated liposarcoma and pleomorphic liposarcoma are highly malignant tumors, prone to both recurrence and metastasis.…”