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Undifferentiated Pleomorphic Sarcoma Metastatic to the Orbit
Jonathan K Ramsey1, James L Chen2,3, Lynn Schoenfield4
1Department of Ophthalmology and Visual Science.
Ophthalmic Plastic and Reconstructive Surgery
|October 6, 2018
Summary
Undifferentiated pleomorphic sarcoma, previously malignant fibrous histiocytoma, rarely presents as orbital metastasis. This case highlights a rare instance of metastatic orbital involvement in undifferentiated pleomorphic sarcoma.
Area of Science:
- Oncology
- Pathology
- Ophthalmology
Background:
- Undifferentiated pleomorphic sarcoma (UPS), formerly malignant fibrous histiocytoma, is a rare mesenchymal malignancy.
- Orbital involvement by UPS is uncommon, with no reported metastatic cases since tumor reclassification in 2002.
Observation:
- A patient developed a left orbital metastasis two years after treatment for high-grade undifferentiated pleomorphic sarcoma of the right thigh.
- Orbital mass histopathology mirrored the primary tumor, though treatment-induced changes were noted in excisional tissue.
Findings:
- Immunohistochemistry and genetic testing confirmed the metastatic origin of the orbital lesion.
- The patient's tumor exhibited rapid progression despite targeted systemic therapy.
Implications:
- This case represents a rare instance of metastatic orbital undifferentiated pleomorphic sarcoma, challenging previous reporting norms.
- Orbital exenteration was performed due to rapid progression, with no recurrence observed at two-year follow-up.
- Highlights the importance of considering metastatic disease in orbital masses, even rare presentations of UPS.
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