Primary sclerosing liposarcoma of the ovary: Case report and a review of the literature.

INTRODUCTION: Primary soft tissue sarcoma of the ovary is uncommon, with most literature reports being in the format of a single case report or small case series. PRESENTATION OF CASE: We report a case of a primary sclerosing liposarcoma of the ovary in a 67-year-old lady. She presented with a rapidly growing ovarian mass, which was surgically excised. Histologically, the lesion appeared nodular with alternating hypercellular and low-cellularity areas, with areas of necrosis. It comprised atypical spindled, oval and stellate cells set in abundant myxoid and fibrous hyalinised stroma. Immunohistochemistry showed expression of p16, CDK4, CD34, S-100 and MDM2. The case was sent to a referral centre where MDM2 gene amplification was detected by interphase fluorescent in-situ hybridisation (FISH) analysis, rendering a diagnosis of well-differentiated sclerosing liposarcoma with focal low-grade dedifferentiation. DISCUSSION: To the best of our knowledge, this is the first reported example of a sclerosing variant of liposarcoma in the ovary. Differential diagnosis includes microcystic stromal tumour (MST), sclerosing stromal tumour (SST), atypical spindle cell lipoma, myxoid liposarcoma, myxofibosarcoma, solitary fibrous tumour and low grade fibromyxoid sarcoma. CONCLUSION: Ovarian sarcomas are characterised by a rapid and aggressive clinical course, requiring multidisciplinary team input for ongoing management. Complete surgical resection with clear margins is the mainstay of treatment.