Treatment of Orbital Desmoid-type Fibromatosis With Sorafenib

Area of Science:

• Ophthalmology
• Oncology
• Pediatric Oncology

Background:

• Desmoid-type fibromatosis (DF) is a rare fibroblastic tumor.
• Orbital involvement is exceptionally rare, with fewer than 10 reported cases.
• Primary orbital DF poses diagnostic and therapeutic challenges, especially in infants.

Observation:

• An infant presented with rapid onset of right eye (OD) proptosis, disc edema, and hyperopic shift.
• A retrobulbar mass consistent with desmoid-type fibromatosis was diagnosed.
• Complete surgical excision carried a high risk of vision loss.

Findings:

• The orbital desmoid-type fibromatosis was treated with sorafenib, a tyrosine kinase inhibitor.
• Tumor stabilization and subsequent regression in size were observed during sorafenib therapy.
• This represents the first reported case of orbital desmoid-type fibromatosis treated successfully with sorafenib.

Implications:

• Sorafenib may be a viable therapeutic option for managing orbital desmoid-type fibromatosis, particularly when surgery is contraindicated.
• This case expands the understanding of treatment modalities for rare orbital tumors in pediatric patients.
• Further research is warranted to evaluate the long-term efficacy and safety of sorafenib in this context.