• 601 Providence Park Drive, Suite O, Mobile, Alabama,36695
    251-650-5437
  • 27961 U.S. 98, Thomas Medical Center, Daphne, Alabama,36526
    251-928-1941
  • 1720 Medical Park Dr, Suite 330a, Biloxi, Mississippi,39532-2131
    228-396-5185

Orbital Tumors - Lymphoma

General

  • almost exclusively in adults
  • continuum including benign reactive lymphoid hyperplasia (pseudolymphoma) to atypical lymphoid hyperplasia to low-grade then high grade malignant lymphoma;
  • also Orbital inflammatory syndrome pseudotumorplasmacytoma (including myeloma)
  • bimodal peak 30’s and 60’s
  • unilateral or bilateral
  • palpable rubbery mass fixed to orbital rim
  • maligant lymphoma & reactive lymphoid hyperplasia cause gradual (over a year or more) progressive, painless proptosis (vs. Obital inflammatory syndrome), lacrimal enlargement
  • usually on conjunctiva, anterior orbit so palpable or visible
  • eyelid or bilateral orbital involvement suggests systemic disease
  • putty-like molding to undisplaced tissues so little Visual Acuity (VA) or EOM loss; usually no bone erosion or infiltration unless high-grade lymphoma
  • lymphoma in retrobubar fat is infiltrative

Imaging

  • all patients w/ orbital lymphoid lesions need exam for systemic lymphoma (by oncology) with orbital, abdominal, chest CT;
    • CBC
    • bone marrow biopsy
    • chest x-ray (CXR)
    • bone/liver/spleen scan

Pathology

  • cytologic factors are more prognostic than mono/polyclonal; but
    • most benign lesions (reactive hyperplasia) are usually mostly T cells with polyclonal Bs;
    • malignant lymphoma usually more monoclonal B cells
  • both polyclonal and monoclonal varieties can develop systemic disease
  • open biopsy for path to give fresh tissue for touch preps; immunohistochemistry; flow cytometry; and gene rearrangement studies; in formalin for micro; gluteraldehyde for electron microscopy

Treatment & Course

  • X-Ray Therapy (XRT) for most orbital lymphoid lesions that are confined to orbit (50% of lymphomas)
  • Chemotherapy for systemic, therapy can be controversial
  • Course
    • up to 25% of patients have systemic lymphoma later on with benign reactive hyperplasia:
    • 40% of patients get systemic involvlement within 5 years with atypical lymphoid hyperplasia:
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