Objective:

To describe the diagnostic complexity and therapeutic role of radiation therapy, including re-irradiation, in orbital RDD.

Approach:

Key Findings:

• Histopathology confirmed RDD with specific immunophenotypic markers (S100+, CD68+, CD1a–).
• Initial treatment with corticosteroids was partially effective.
• Image-guided radiotherapy (36 Gy in 20 fractions) led to complete remission for five years.
• Re-irradiation (24 Gy in 12 fractions) improved visual acuity and showed stable MRI findings with no late radiation toxicity at two-year follow-up.

Interpretation:

This case highlights the feasibility and efficacy of radiation therapy, including re-irradiation, in managing orbital RDD when medical management fails.

Limitations:

• Standardized management strategies for RDD remain undefined.
• The rarity of the disease limits the generalizability of findings.

Conclusion:

Re-irradiation achieved durable local control and preserved visual function in this case of recurrent orbital RDD.

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