Primary orbital pleomorphic liposarcoma in a child: A case report

PURPOSE: To report a rare case of primary orbital pleomorphic liposarcoma and present a relevant literature review. OBSERVATIONS: An 11-year-old boy presented with an enlarging, painless lower right eyelid mass that was noted 4 months ago. Imaging revealed a 3.2 × 2.1 × 3.7-cm-sized well-circumscribed lobulated mass. Biopsy revealed a pleomorphic spindle cell neoplasm that was consistent with a pleomorphic liposarcoma. A systemic evaluation found no evidence of distant metastasis. Despite four chemotherapy cycles, the mass size increased at follow-up. Total orbital exenteration was performed 1 year after confirming a diagnosis of pleomorphic liposarcoma. At the 6-month follow-up, the patient was alive with no signs of recurrence. CONCLUSIONS: Pediatric pleomorphic liposarcoma is a very rare entity. Its definite diagnosis relies on histopathological results. In the absence of systemic metastasis, total orbital exenteration is the optimal approach for local control.