Orbital Mantle Cell Lymphoma Often Presents as Eyelid Swelling, 15-Year Study Finds

Mantle cell lymphoma involving the orbit most often announces itself as eyelid swelling or a mass, and most patients are already at an advanced stage when diagnosed, according to a 15-year retrospective study from Beijing Tongren Hospital published in BMC Cancer. The study analyzed 13 patients treated between January 2010 and May 2025 and paired the case series with a review of previously published individual cases and case series.

The median age of the patients was 65 years. Ten patients (76.9%) were male, and nine (69.2%) had disease on both sides. For nine patients, orbital disease was the initial presentation of the lymphoma, while four developed orbital involvement secondarily. The most common manifestations were eyelid swelling or a mass in twelve of thirteen patients (92.3%), ocular motility disorders in eight (61.5%), and proptosis in six (46.2%).

Every patient had involvement of multiple orbital structures rather than a single isolated site. The eyelid was affected in twelve patients (92.3%), the lacrimal gland in eleven (84.6%), and the extraocular muscles in eleven (84.6%). Among the ten patients who underwent systemic evaluation, all ten had lymph node involvement and nine of ten (90.0%) had nasopharyngeal involvement, indicating that orbital disease in this cohort was typically one facet of widespread disease.

Diagnosis rested on the immunophenotype of the tumor cells. In all patients, the malignant cells were positive for cyclin D1, CD20, and Bcl-2, with typical negativity for CD23, Bcl-6, and CD10.

Ten patients received systemic chemotherapy, including two who also received local radiotherapy. Among the eleven patients with available follow-up data, the median follow-up was 48 months. Three patients relapsed during that period, and no deaths occurred. The estimated progression-free survival rates were 90% at one year, 80% at three years, and 67% at five years.

The literature review confirmed the patterns seen in the cohort: male predominance, frequent bilateral disease, advanced-stage presentation, and heterogeneous treatment patterns across published reports.

The authors conclude that the clinical presentation of mantle cell lymphoma with orbital involvement lacks specificity, and that most patients are diagnosed at an advanced stage. They emphasize that for middle-aged and elderly patients whose orbital disease shows only a transient response to steroid treatment, early biopsy is crucial for reaching an accurate diagnosis.

As a descriptive retrospective study of thirteen patients at a single center, the analysis cannot establish treatment superiority or population-level incidence, and its survival estimates carry the uncertainty inherent to a small cohort. Nevertheless, by consolidating fifteen years of clinical data alongside the published literature, the study documents the clinical features, treatment patterns, and outcomes of orbital involvement in mantle cell lymphoma.

Subject of Research: Clinical features and outcomes of orbital involvement in mantle cell lymphoma

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

Article References: Ren, T., Li, J., Wang, N., Xu, L., Guo, Q., Yan, H., Zhang, Y., Yang, R., Wang, T., Gao, T., Wang, L., & Ma, J. (2026). Clinical features and outcomes of orbital involvement in mantle cell lymphoma: a 15-year retrospective study and literature review. BMC Cancer. https://doi.org/10.1186/s12885-026-17203-8

Image Credits: AI Generated

DOI: 10.1186/s12885-026-17203-8

Keywords: mantle cell lymphoma, orbital involvement, retrospective study, cyclin D1, eyelid swelling, lacrimal gland, proptosis, progression-free survival, early biopsy, BMC Cancer, Clinical, features