Radiotherapy with re-irradiation achieves long-term remission in orbital Rosai-Dorfman diseaseRadiotherapy Shows Promise for Orbital Rosai-Dorfman Disease Treatment

Frontiers in Medicine Published June 20, 2026 DOI ↗ Editorial oversight: Dr. Julia Lee, PhD · Oncology, Genomics & Drug Development

AI-generated summary of the cited source, checked by automated accuracy review. How we work

Key Takeaway

Consider radiotherapy, including re-irradiation, as a feasible option for orbital Rosai-Dorfman disease when medical management fails.

This case report details the management of a 58-year-old male with orbital Rosai-Dorfman disease (RDD) who received image-guided radiotherapy (36 Gy in 20 fractions) followed by re-irradiation (24 Gy in 12 fractions) for recurrence. The initial radiotherapy resulted in complete remission for five years. After recurrence, re-irradiation improved visual acuity from 6/60 to 6/36, and no late radiation toxicity was observed at two-year follow-up.

The authors highlight the feasibility and efficacy of radiation therapy, including re-irradiation, for orbital RDD when medical management fails. However, the evidence is limited by the small sample size (n=1) and the lack of robust data supporting re-irradiation in recurrent disease. No adverse events were reported at two-year follow-up.

While the outcome is favorable, clinicians should interpret these results cautiously given the single-patient nature of the report. The case adds to the limited literature on radiotherapy for RDD but does not provide comparative effectiveness data against other treatments.

How this fits prior evidence

This case report adds to the limited evidence on Rosai-Dorfman disease, which, like Kounis syndrome, is primarily documented through case reports. It demonstrates that radiotherapy, including re-irradiation, can achieve prolonged remission and improve visual acuity, contrasting with the lack of benefit seen with immunomodulatory therapies after cardiac arrest. The finding supports the potential role of localized radiotherapy in rare orbital conditions.

This case report describes the treatment of a 58-year-old man diagnosed with orbital Rosai-Dorfman disease. The condition affected his eye area, and medical management alone was not enough to control the disease. Doctors used image-guided radiotherapy followed by a second round of radiation, known as re-irradiation, to treat the area.

The patient experienced complete remission for five years after the first treatment. Following the second round of radiation, his vision improved from 6/60 to 6/36. At the two-year follow-up mark, no late radiation toxicity was reported, and the patient tolerated the procedure well.

Because this report only follows one individual, the results cannot be applied to everyone with this condition. The evidence for using re-irradiation in cases of recurring disease is currently limited. This case highlights that radiation therapy may be a feasible option when standard medical treatments fail for orbital Rosai-Dorfman disease.

What this means for you:

Radiation therapy showed success for one patient with orbital Rosai-Dorfman disease, but more research is needed.

Common questions

What was the result of the radiation treatment?

The patient achieved complete remission for five years after the initial radiotherapy. After the second round of radiation, his vision improved from 6/60 to 6/36. No late radiation toxicity was reported at the two-year follow-up.

Is this treatment safe for the eyes?

In this specific case, the patient showed no signs of late radiation toxicity at the two-year follow-up. However, because this study only involved one person, it is not enough to confirm safety for all patients.

How is this different from standard medical management?

This treatment was used when medical management failed to control the disease. It involved image-guided radiotherapy and a second round of radiation to target the orbital area specifically.

Study Details

Study typeSystematic review

EvidenceLevel 1

PublishedJun 2026

View Original Abstract ↓

IntroductionRosai–Dorfman–Destombes disease (RDD) is a rare non-Langerhans histiocytic disorder with occasional extranodal involvement. Orbital disease can mimic neoplastic or inflammatory conditions, leading to diagnostic and therapeutic challenges. Standardized management strategies remain undefined.AimTo describe the diagnostic complexity and therapeutic role of radiation therapy, including re-irradiation, in orbital RDD.Case presentationA 58-year-old male presented with nasal obstruction, later progressing to orbital involvement. Histopathology confirmed RDD (S100+, CD68+, CD1a–). Initial corticosteroid therapy provided partial relief. Image-guided radiotherapy (36 Gy in 20 fractions) achieved complete remission for five years. On recurrence with proptosis and compressive optic neuropathy, re-irradiation (24 Gy in 12 fractions) was delivered after careful review of prior dose constraints. The patient demonstrated improved visual acuity (6/60 to 6/36), stable MRI findings, and no late radiation toxicity at two-year follow-up.ConclusionThis case highlights the feasibility and efficacy of radiation therapy, including re-irradiation, in orbital RDD when medical management fails. Re-irradiation achieved durable local control, preserved visual function, and adds to the limited evidence supporting its role in recurrent disease.

Radiotherapy with re-irradiation achieves long-term remission in orbital Rosai-Dorfman diseaseRadiotherapy Shows Promise for Orbital Rosai-Dorfman Disease Treatment

How this fits prior evidence

Common questions

Study Details

Transbronchial biopsy with 1.1-mm cryoprobe yields 88.6% diagnosis versus 78.8% with 2.0-mm forceps

Cryoprobe biopsy shows higher success rate for diagnosing lung conditions

Clinical research that matters. Delivered to your inbox.

Radiotherapy with re-irradiation achieves long-term remission in orbital Rosai-Dorfman diseaseRadiotherapy Shows Promise for Orbital Rosai-Dorfman Disease Treatment

How this fits prior evidence

Common questions

More on Rosai-Dorfman disease

Study Details

Transbronchial biopsy with 1.1-mm cryoprobe yields 88.6% diagnosis versus 78.8% with 2.0-mm forceps

Cryoprobe biopsy shows higher success rate for diagnosing lung conditions

Clinical research that matters. Delivered to your inbox.

Related in Oncology

From Other Specialties