Clinical and Pathological Features of Primary Orbital Liposarcoma in Chinese Patients

PURPOSE: Liposarcomas are rare in the orbit. We analyzed a series of primary liposarcomas to determine the features unique to the orbit. METHODS: Records from 10 Chinese patients treated for primary orbital liposarcoma at Beijing Tongren Hospital, Capital Medical University, between September 2009 and September 2020 were reviewed. RESULTS: This cohort included four men and six women with age of onset ranging from 18 to 80 years. The pathology was myxoid liposarcoma in five patients, dedifferentiated liposarcoma in two patients, well-differentiated liposarcoma and pleomorphic liposarcoma in one patient each, and dedifferentiated liposarcoma and well-differentiated liposarcoma co-existing in one case. Magnetic resonance imaging (MRI) revealed a well-defined, irregular, or lobulated mass in the orbit, which contained components that were suppressible in the fat-suppression sequence, as well as components that were enhanced by gadolinium enhancement. Nine patients relapsed after surgery, with a mean recurrence of 2.44, and one patient was lost to follow-up. The interval between treatment and first recurrence ranged from 4 months to 16 years; 55.6% of patients with orbital liposarcoma relapsed within one year. Three patients underwent local excision alone, four patients underwent excision combined with radiotherapy, and three patients underwent exenteration. Half of the patients were misdiagnosed in the pathologic diagnosis after their first or multiple surgeries. No distant metastasis, death from tumors, or invasion of adjacent organs was observed after 21-150 months of follow-up. CONCLUSION: Orbital liposarcoma is easily misdiagnosed and prone to recurrence; however, MRI findings may help identify orbital liposarcoma prior to surgery. The optimal treatment choice remains to be discussed.