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| Title | Primary orbital low grade fibromyxoid sarcoma in a 12-year-old – A case report |
| Number | 315 |
| Author | Simon Merrick |
| Principal Unit where research was undertaken | St James’s Hospital/Leeds Teaching Hospital NHS Trust |
| Purpose | A 12-year-old boy attended the emergency eye clinic with progressive, painless proptosis and hypoglobus of his left eye over the preceding twelve months. This was associated with limitation of elevation and abduction but good visual acuity, normal colour vision and no relative afferent pupillary defect. |
| Methods | Urgent imaging confirmed the presence of a large, well-defined, homogenous mass lesion involving the orbital roof and extending into the superolateral orbit. This lesion abutted the globe but apart from mild adjacent hyperostosis showed no aggressive features, adjacent inflammation or tissue invasion. Incisional biopsy yielded the histological findings of a whorled spindle cell tumour characterised as a suspected low grade fibromyxoid sarcoma (LGFMS). |
| Results | The patient underwent further debulking and macroscopic clearance of the tumour with excellent restoration of globe position post-operatively. Additional histology confirmed the presence of fibromyxoid tumour within adjacent bone and soft tissue. Paediatric neuro-oncology and specialist sarcoma multi-disciplinary team (MDT) input was sought throughout his care and treatment options of chemotherapy, radiotherapy and further surgery were considered. He currently remains under short interval surveillance with no radiological evidence of recurrence. |
| Conclusion | LGFMS is a very rare mesenchymal tumour that typically affects the trunk and extremities in adolescents and young adults. Orbital involvement is extremely rare with only a few reported cases in the literature to date. Complete surgical excision may not be possible or appropriate in some cases and there is no consensus on the value of systemic therapies owing to its rarity. These tumours have a propensity for indolent behaviour with potential for local recurrence and metastasis multiple decades after initial presentation. This necessitates long term surveillance and involvement of specialist MDTs. |
Additional Authors
| First name | Last name | Hospital / Institution |
|---|---|---|
| Han | Khoo | |
| Bernard | Chang | |
| Kaveh | Vahdani | |
| Gabriella | Guevara |
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