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

Abstract

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.

Multiple recurrent myxofibrosarcoma of the orbit: case report and review of the literature

“…Orbital presentation of MFS is extraordinarily rare, and it was first described in 2008. Since then, only six cases have been published [3][4][5][6][7][8].…”

“…In the six published case reports featuring orbital MFS, the most extended follow-up period was 2 years, which cannot properly reflect relapsing or metastasis. One article has reported a case in which the patient underwent three surgeries as well [7]. Still, no pathological data was described regarding the former operations, making it hard to determine if it was a case of recurrent MFS.…”

Elderly male patient with unilateral proptosis and decreased vision of the left eye

“…[4][5][6][7][8] On CT scan, MFS appears isodense to extraocular muscles, 8 with hyperdensity corresponding to areas of hypercellularity. 9 On MR, the lesion is isointense on T1 weighted, hyperintense on T2 weighted to extraocular muscles. 7 Hypercellular areas are hyperintense on T1 weighted and hypointense on T2 weighted.…”

“…7 Hypercellular areas are hyperintense on T1 weighted and hypointense on T2 weighted. 9 On contrast-enhanced CT scan and MR, areas with increased cellularity show homogeneous contrast uptake. Hypercellularity indicates tumour grade progression.…”

“…Hypercellularity indicates tumour grade progression. 9 For reliable identification of the subtype of sarcoma, immunohistochemical and cytomorphological findings are important. 10 Histological findings include spindle or stellate-shaped fibroblasts within a myxoid stroma, with hyperchromatic tumour cells showing moderate-to-marked pleomorphism.…”