Clinical features and outcomes of orbital involvement in mantle cell lymphoma: a 15-year retrospective study and literature review

To summarize the clinical features, treatment, and outcomes of mantle cell lymphoma (MCL) with orbital involvement. This descriptive retrospective study included 13 patients with orbital involvement by MCL treated at Beijing Tongren Hospital between January 2010 and May 2025. Clinical and follow-up data were analyzed, and published individual cases and case series were reviewed. The median age was 65 years. 10 patients (76.9%) were male, and 9 (69.2%) had bilateral disease. Orbital disease was the initial presentation in 9 patients, whereas 4 had secondary orbital involvement. The main manifestations were eyelid swelling or mass (12/13; 92.3%), ocular motility disorders (8/13; 61.5%), and proptosis (6/13; 46.2%). All patients had multi-structure orbital involvement, most often affecting the eyelid (12/13; 92.3%), lacrimal gland (11/13; 84.6%), and extraocular muscles (11/13; 84.6%). Among patients with systemic evaluation, lymph node (10/10; 100%) and nasopharyngeal (9/10; 90.0%) involvement were most common. Tumor cells were positive for cyclin D1, CD20, and Bcl-2 in all patients, with typical negativity for CD23, Bcl-6, and CD10. Ten patients received systemic chemotherapy, including 2 who also received local radiotherapy. Among 11 patients with follow-up data, the median follow-up was 48 months; three patients relapsed, and no deaths occurred. The estimated 1-, 3-, and 5-year PFS rates were 90%, 80%, and 67%, respectively. The literature review confirmed male predominance, frequent bilateral disease, advanced-stage presentation, and heterogeneous treatment patterns. The clinical presentation of MCL with orbital involvement lacks specificity, and most patients are diagnosed at an advanced stage. For middle-aged and elderly patients with only transient steroid responsiveness, early biopsy is crucial for accurate diagnosis.