Ocular Adnexal Lymphoma of the Extraocular Muscles: Case Series From the University of Iowa and Review of the Literature

Ophthalmic Plast Reconstr Surg. Nov-Dec 2011;27(6):471-6. doi: 10.1097/IOP.0b013e31822e5c1b.


Purpose: To review and statistically analyze the data of patients with discrete involvement of the extraocular muscles with ocular adnexal lymphoma from the tumor registry at the University of Iowa and to compile with current cases in the literature.

Methods: The records of patients with biopsy-proven orbital lymphoma at the University of Iowa Hospitals and Clinics Department of Ophthalmology and the Tumor Registry of the University of Iowa were reviewed. A review of the literature and meta-analysis were conducted.

Results: Eleven patients were identified with biopsy-proven ocular adnexal lymphoma with discrete involvement of the extraocular muscles at the University of Iowa. Additionally, 46 patients were identified in the literature with clinical and radiographic involvement of the extraocular muscles, 31 (67%) with biopsy-proven involvement. In the combined group of 57 patients, 16 (35%) of 45 patients with histopathologic documentation had lymphomas classified as extranodal marginal zone lymphomas. Twenty-five (67%) of the 37 patients with tumor-staging documentation had no extraorbital involvement.

Conclusions: Ocular adnexal lymphoma with discrete extraocular muscle involvement is rare. Most lymphomas found in this area of the orbit are subtypes of B-cell lymphoma. This disease should be included in the differential diagnosis of enlarged extraocular muscles.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Academic Medical Centers
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents / therapeutic use
  • Biomarkers, Tumor / analysis
  • Female
  • Humans
  • Iowa
  • Lymphoma / classification
  • Lymphoma / pathology*
  • Lymphoma / therapy
  • Male
  • Middle Aged
  • Muscle Neoplasms / classification
  • Muscle Neoplasms / pathology*
  • Muscle Neoplasms / therapy
  • Oculomotor Muscles / pathology*
  • Orbital Neoplasms / classification
  • Orbital Neoplasms / pathology*
  • Orbital Neoplasms / therapy
  • Radiotherapy
  • Registries
  • Tomography, X-Ray Computed


  • Antineoplastic Agents
  • Biomarkers, Tumor