Characteristics of Orbital Lymphoma: A Clinicopathological Study of 26 Cases

Int Ophthalmol. 2018 Feb;38(1):271-277. doi: 10.1007/s10792-017-0457-y. Epub 2017 Mar 31.


Objective: Evaluation of the medical data of patients with orbital and adnexal lymphoma.

Design: Cohort study of all cases diagnosed with orbital or adnexal lymphoma at Meir Medical Center between 1993 and 2007.

Participants: Twenty-six patients, with intraorbital or subconjunctival masses with orbital involvement, were examined and followed up between 1 and 8 years.

Materials and methods: Examined data included: clinical presentation, age, gender, imaging, tumor location, surgical management, and pathological diagnosis.

Results: Presenting signs and symptoms included proptosis, eyelid lesions, tearing, chemosis, decreased visual acuity, ptosis, pain, squint, and optic nerve compression. In five cases, lymphoma was misdiagnosed on neuroimaging. Bone changes were seen in four patients. All cases were B cell lymphomas; with the majority (22 cases) of small B cell type; consisting of primary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue and two cases of small cell lymphoma. One small cell lymphomas was of follicular type on a background of CLL, and the other was CLL/SLL type. Fourteen cases were primary orbital disease, and 12 cases were systemic disease. Macroscopic appearance of lymphoma at open biopsy was characteristic in most cases. Flow cytometry phenotyping gave rapid reliable diagnosis of the disease.

Conclusions: Epiphora or chemosis in the presence of an orbital mass should alert the ophthalmologist to suspect lymphoma. Lymphoma may be easily misinterpreted on neuroimaging for other diseases. Bone changes seen on CT are more common than is generally perceived. Macroscopic appearance at open biopsy was characteristic.

Keywords: Adnexal lymphoma; B cell lymphoma; Chemosis; Epiphora; Mucosa-associated lymphoid tissue; Orbital lymphoma.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Biopsy
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Female
  • Flow Cytometry
  • Follow-Up Studies
  • Humans
  • Lymphoma, B-Cell, Marginal Zone / diagnosis*
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Male
  • Middle Aged
  • Neoplasm Staging*
  • Orbital Neoplasms / diagnosis*
  • Orbital Neoplasms / therapy
  • Retrospective Studies
  • Time Factors
  • Tomography, X-Ray Computed / methods*