Long-term clinical course and recurrence pattern of orbital Kimura’s disease: a retrospective case series

BACKGROUND: Kimura's disease (KD) is a rare chronic inflammatory disorder that predominantly affects young Asian males and typically involves the head and neck region. Orbital involvement is uncommon and remains insufficiently characterized, particularly with respect to long-term outcomes. This study aimed to investigate the clinical features, imaging characteristics, treatment strategies, and long-term prognosis of orbital KD. METHODS: We conducted a retrospective case series of patients with histopathologically confirmed KD involving the orbit at a single tertiary referral center between January 2010 and December 2025. Demographic characteristics, clinical manifestations, imaging findings, pathological features, treatment modalities, and follow-up outcomes were reviewed. For suspected recurrence, recurrent clinical manifestations were first identified during follow-up, and orbital imaging was used to confirm lesion recurrence and assess lesion extent when indicated. Pathological confirmation was obtained only when repeat surgery or biopsy was performed. RESULTS: Eight male patients were included, with a mean age of 21.25 ± 17.20 years (range, 6-47 years). Painless eyelid swelling was the most common presenting symptom (62.5%). The eyelid and lacrimal gland were the most frequently involved structures. Histopathological examination revealed dense eosinophilic infiltration and lymphoid follicular hyperplasia in all cases. Surgery combined with systemic glucocorticoid therapy was the primary treatment modality (87.5%). Among the seven patients with available follow-up data, the mean follow-up duration was 87.57 ± 55.42 months. Recurrence occurred in two of the seven patients with available follow-up data (28.6%; 25.0% of the entire cohort), both presenting as local orbital/periorbital recurrence. No distant disease progression, metastasis, or disease-related mortality was observed. CONCLUSIONS: Orbital KD predominantly affects young males and most commonly involves the eyelid and lacrimal gland. Although local recurrence may occur, the long-term prognosis appears favorable. Structured follow-up remains important for detecting recurrence and guiding subsequent management.