A case report of a rare, aggressive and malignant orbital tumour.

A 67-year old male patient presented with a 6-months history of a progressive non-painful proptosis and increasing diplopia.

Examination revealed a decreased visual acuity of 20/10 with a relative afferent pupillary defect. The ocular movement was constricted in upgaze. Slit lamp examination only showed an injected conjuntiva, the optic disc was normal. Computer tomography demonstrated a hypodense extraconal oval shaped lesion localized in the superomedial quadrant of the orbit extending into the apex mimicking a mucocele with similar Hounsfield Units.

We present the first case of a pleomorphic orbital liposarcoma that resolved under extensive surgery, adjuvant chemotherapy and radiotherapy. No recurrence was observed for 18 months following our treatment plan.

Liposarcoma of the orbit is – unlike other locations – an extremely rare malignancy. To the date, only 4 cases of pleomorphic primary orbital liposarcoma have been described. The prognostic factor is depending on tumour size, location, morphologic type, histologic grade and adequacy of initial therapy. Because of the tumour location and its invasive growth pattern, complete resection is mostly not possible. Incomplete surgical management of these tumours is the reason for a high recurrence rate and a low survival time.

To the date, the optimal treatment of these tumours, especially when involving rare sites like the orbit, is very controversial. A multidisciplinary approach of orbital liposarcomas is unevitable to avoid further progression of the disease.