Abstract ID: 26-588
Advanced Primary High-Grade Orbital Leiomyosarcoma: Multidisciplinary Management
Author: Simon Merrick Base Hospital / Institution: St James’s University Hospital, Leeds, UK
Presentation Type: ePoster Presentation
Purpose
To present the management of two cases of high-grade orbital leiomyosarcoma.
Methods
Case 1
A 38-year-old woman presented with diplopia and painless proptosis of her left eye for 5 months. She was found to have 3mm relative proptosis, hypoglobus and limitation of elevation in her left eye. MRI revealed a left orbital mass with invasion through the orbital roof into the left frontal sinus and anterior cranial fossa. She had no alternative primary tumour or metastases. She underwent bicoronal craniotomy with frontal sinus cranialisation, orbital exenteration and radial forearm free flap reconstruction in addition to adjuvant radiotherapy.
Case 2
A 25 year old man presented with new onset socket pain. His history was significant for bilateral retinoblastoma, positive RB1 and BRCA1 gene mutation and previous bilateral enucleation, radiotherapy and chemotherapy. MRI demonstrated a left orbital mass with involvement of the left orbital roof, sphenoid bone and dural disease along the left anterior and middle cranial fossae. He proceeded to neo-adjuvant doxorubicin and dacarbazine chemotherapy followed by orbital exenteration, removal of temporalis, cranio-orbital resection and reconstruction with PEEK implant and with radial free flap reconstruction followed by adjuvant radiotherapy.
Results
Histopathology in both cases confirmed high-grade leiomyosarcoma.
Conclusion
Leiomyosarcomas are mesenchymal tumours of smooth muscle that are extremely rare to present as primaries within the orbit. Their typically painless presentation and risk of development deep within soft tissues can make early diagnosis challenging. Prognosis is thus often poor as advanced local spread or metastases are often established by the point of diagnosis. An MDT approach is needed and treatment options most commonly include surgical excision with or without radiotherapy. The role of chemotherapy in non-metastatic orbital leiomyosarcoma is less clear due to its rarity.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Rebecca | Dawson | Leeds Teaching Hospitals NHS Trust |
| Leila | Touil | Leeds Teaching Hospitals NHS Trust |
| Nick | Phillips | Leeds Teaching Hospitals NHS Trust |
| Jiten | Parmar | Leeds Teaching Hospitals NHS Trust |
| Asim | Sheikh | Leeds Teaching Hospitals NHS Trust |
| Gabriella | Guevara | Leeds Teaching Hospitals NHS Trust |