Comprehensive analysis of orbital lymphoma in a Turkish cohort: clinical characteristics, histological subtypes, treatment modalities, prognostic factors, and implications for management

Ann Hematol. 2024 Mar;103(3):905-915. doi: 10.1007/s00277-023-05569-9. Epub 2023 Dec 7.

Abstract

The study analysed the clinical characteristics, treatment approaches, and survival outcomes of 97 consecutive patients with orbital lymphoma (OL) over a 25-year period at. The median age of the patients was 57.6 years, and 59.8% (n = 58) were male. Marginal zone lymphoma constitutes the most prevalent subtype, accounting for 67% of cases, whereas other common subtypes include diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, and T-cell lymphomas. Unilateral involvement was observed in the majority of cases (72.3%). Common clinical presentations included mass (30.9%), swelling (26.8%), and epiphora (11.3%). Of the patients, 7.2% received rituximab alone, 14.4% received radiotherapy alone, 48.5% received chemotherapy, 27.8% received radiotherapy plus rituximab, 22.7% received radiotherapy plus chemotherapy, and 5.2% underwent surgery as the first-line treatment. During a median follow-up of 4.3 years, 15.5% of patients experienced relapse or disease progression. The 5-year and 10-year progression-free survival rates were 84.1% and 79.1%, respectively. This study contributes to our understanding of OLs and provides a foundation for further investigations in this field. Male gender, presence of B symptoms, advanced stage, secondary orbital lymphoma, aggressive histological subtype, and elevated serum lactate dehydrogenase levels were associated with poorer (either inferior or worse) progression-free survival.

Keywords: Marginal zone lymphoma; Ocular adnexal lymphoma; Orbital lymphoma; Orbital neoplasms.

MeSH terms

  • Adult
  • Female
  • Humans
  • Lymphoma*
  • Lymphoma, B-Cell, Marginal Zone* / diagnosis
  • Lymphoma, B-Cell, Marginal Zone* / pathology
  • Lymphoma, B-Cell, Marginal Zone* / therapy
  • Lymphoma, Follicular*
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Orbital Neoplasms* / epidemiology
  • Orbital Neoplasms* / therapy
  • Prognosis
  • Retrospective Studies
  • Rituximab

Substances

  • Rituximab

Supplementary concepts

  • Orbital lymphoma