Giant myxofibrosarcoma of the orbit: a rare case and a review of the literature.

A 65-year-old man presented with giant orbital myxofibrosarcoma with no extraorbital extension. Imaging was suggestive of mesenchymal malignancy with significant vascularity. Incisional biopsy was suggestive of low-grade fibromyxoid sarcoma. The clinical and imaging features did not support the pathological diagnosis. Histopathology of the exenterated sample revealed features of high-grade myxofibrosarcoma. To the best of our knowledge, this is the largest reported myxofibrosarcoma of the orbit. Adjuvant radiotherapy was advised. Correlating the case history, imaging and histopathology, this case can best be summarised as a natural history of conversion from a low to high-grade myxoid malignancy. We emphasise that the overlapping features of myxoid malignancy (fibromyxosarcoma vs myxofibrosarcoma) and regional variation in tumour morphology in biopsy specimen may mislead the clinician. Therefore, knowledge about the key differentiating features as well as incisional biopsy of each radiologically different area can increase the possibility of correct diagnosis and management of each case. 2016 BMJ Publishing Group Ltd.