Frontiers in oncology · 2026
Long-term management of orbital Rosai-Dorfman disease with re-irradiation for locoregional recurrence: a case report.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Krishna Sanjay Arvind, Nair Anoop Remesan, Perumal Hema Ashok, Eapen Malini, Ullattil Pushpaja Kuttassery, Dutta Debnarayan
Аннотация
INTRODUCTION: Rosai-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.
AIM: To describe the diagnostic complexity and therapeutic role of radiation therapy, including re-irradiation, in orbital RDD.
CASE PRESENTATION: A 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.
CONCLUSION: This 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.
AIM: To describe the diagnostic complexity and therapeutic role of radiation therapy, including re-irradiation, in orbital RDD.
CASE PRESENTATION: A 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.
CONCLUSION: This 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.