Orbital metastasis of myxofibrosarcoma

Abstract

Myxofibrosarcoma (MFS), a common sarcoma in adults, typically affects the extremities. Primary orbital involvement is uncommon, and orbital metastasis is exceedingly rare. We describe a case of orbital MFS due to metastasis and provide an updated literature review. A 56-year-old woman presented with 2 months of progressive right eye proptosis. Relevant history included left palmar MFS excised 2 years prior. Examination demonstrated proptosis and restriction in abduction of her right eye without optic nerve compromise. Imaging revealed a well-circumscribed right orbital mass. Initial biopsy showed glandular tissue with chronic inflammation without an evidence of malignancy. She subsequently developed worsening proptosis, complete ophthalmoplegia, and a new afferent pupillary defect. Repeat orbital excisional biopsy with deeper dissection demonstrated high-grade MFS consistent with metastasis. She underwent chemoradiation with resolution of her lesion. Literature review identified 12 previously reported cases of MFS, only two of which involved metastasis to the orbit and were associated with worse prognosis. This report highlights a rare case of orbital metastasis of MFS with high-grade features confirmed on histopathology and provides an updated literature review. Prompt diagnosis and complete removal of MFS located both systemically and in the orbit are essential due to the metastatic potential.

Keywords: Metastasis; myxofibrosarcoma; orbit; orbital tumor; tumor.

Figure 1

External photographs of the patient demonstrating hypoglobus and medial displacement of the right eye with restriction in upgaze and abduction

Figure 2

T1-weighted fat-suppressed magnetic resonance imaging. Coronal (a) and axial (b) images demonstrate an enhancing mass along the lateral aspect of the right orbit as indicated by white arrows

Figure 3

(a) Histopathology of the orbital mass demonstrates a hypercellular neoplasm composed of cytologically malignant pleomorphic spindle cells and epithelioid cells enmeshed in a myxoid stroma that contains arching, interconnecting blood vessels (H and E, original magnification ×100). (b) Mitotic figures are present (arrows), consistent with cytologic malignancy (H and E, original magnification ×400). The findings are morphologically similar to the primary high-grade lesion originating in the soft tissue of the left palm (c) H and E, original magnification ×40, (d) H and E, original magnification ×600, mitotic figures indicated by black arrows

Figure 4

Clinical and radiographic findings at follow-up. (a) External examination of the patient at 15-month follow-up after receiving treatment with orbital radiation and systemic chemotherapy demonstrate clinical resolution of her proptosis. T1-weighted fat-suppressed magnetic resonance imaging with coronal (b) and axial (c) images following treatment demonstrate postsurgical changes without evidence of orbital lesion recurrence