Alveolar soft part sarcoma of orbit: A rare diagnosis.

OBJECTIVE

Alveolar soft part sarcoma (ASPS) is an aggressive, rare tumour with unique morphological and histopathological features.

METHODS

We report a rare case of orbital ASPS and its management in a young male who presented with painless proptosis and progressive loss of vision.

RESULT

Twenty-two year male presented with a history of gradually increasing proptosis with loss of vision since 12months. He underwent radical re-excision of mass with right orbital exenteration and reconstruction using temporalis muscle flap. Adjuvant radiotherapy to a dose of 64Gy in 32 fractions over 6.5weeks was planned in view of positive surgical margins. Patient is free of disease and currently under follow up in multidisciplinary clinic.

CONCLUSION

Function preserving surgery remains the standard treatment approach in localised disease however the complex anatomy and locally aggressive nature makes it difficult to achieve clear surgical margin. Adjuvant radiotherapy has shown to improve local control in patients with positive surgical margins.