A rare diagnosis of dedifferentiated liposarcoma of the orbit.

ABSTRACT

We report the case of a 56-year-old female who presented to the ophthalmic casualty department with proptosis and diplopia. Initially, non-specific orbital inflammation was suspected while investigations were ongoing. A trial of steroids failed to resolve the proptosis to a significant extent. A CT scan showed a well-defined orbital mass, which on orbital biopsy was shown to be a primary orbital liposarcoma of the dedifferentiated variety. This was confirmed by expert histopathological analysis. An exenteration followed by removal of orbital bone was required to achieve total tumour removal. Radiotherapy was delivered to reduce the chance of micro-metastasis. Orbital liposarcoma is an extremely rare tumour. The dedifferentiated variety is even rarer, with only a few reported cases. Lack of information about this potentially aggressive tumour may create therapeutic dilemmas as to the best treatment approach. Similarly, prediction of an accurate prognosis for the patient may be difficult due to the rarity of this condition.