Table 1.

Review of the documented cases of adult sporadic Burkitt lymphoma presenting as an orbital mass

Cases	Initial diagnosis/Tx	Presenting ocular sign and symptoms	Histopathology and/or Immunophenotype	Cytogenetic evaluation	EBV status	Systemic disease (Stage)	Survival
24-year-old Chinese male38	Orbital inflammatory disease; Steroid Tx	One day painful lid swelling OS, decreased VA (20/80), nonaxial proptosis, chemosis, ROM. CT scan showed soft tissue mass in the superior, posterior and lateral aspects of the orbit.	Histopathology of biopsy and bone marrow aspirate consistent with BL	ND	ND	Increased WBC; BM	Died 22 weeks post onset
63-year-old Diabetic Black female41	Nausea, abdominal pain and distention; 1 wk eye pain/edema Surgery Tx	One week Hx of progressive OD eye pain, redness, vision loss, swollen lids; OD VA hand motion, right afferent pupillary defect OD, 360° ROM, ptosis, lid edema, chemosis, proptosis, elevated IOP; CT scan showed OD diffuse orbital infiltrate, ethmoid sinus disease/adjacent bone erosion.	Hx: “starry sky” appearance, medium sized atypical lymphocytes; B cell markers; CD10, CD20, CD45 and BCL6; Ki-67	Not specific for BL ND		CT revealed lesions in spleen and left groin	Five months after initial presentation, the patient presented with fever and infection. COD; sepsis
72-year-old Black male41	Possible infection; Antibiotic Tx	Two weeks tooth pain and midfacial swelling; For 1 week, Progressive OD eye proptosis, chemosis, OD VA count finger at 5 feet, 360° ROM, chemosis, elevated IOP; CT scan revealed large mass in inferior orbit and superior maxillary sinus with bone erosion.	Hx: “starry sky” appearance. B cell markers; CD10, CD20, and BCL6, Ki-67; High Mitotic Index	IGH rearrangement; c-MYC translocation [t(8;14)]	ND	No signs of systemic lymphoma stage Ie	Alive at 13 months post initial presentation, but developed skull metastases
78-year-old White female40	Dacroadenitis, lymphoma, lacrimal or gland tumor	Three weeks Hx of increasing swelling of upper OS orbit. 1 mm Proptosis. CT scans revealed left super-temporal orbital mass in the lacrimal gland region causing nasal displacement of the globe.	Hx: high-grade B cell lymphoma; B cell markers; CD10 and CD20	ND	ND	No systemic disease	Free for 5 months; Lost to follow-up; Died 10 months post initial Dx
84-year-old Diabetic White female39	Myositis; Steroid Tx	Diplopia; 2 week swelling of orbit OS; Returned at 2 months with swelling in orbit OS with proptosis, VA (20/30), ROM, diplopia.	Hx: “starry sky” appearance. B cell markers; CD79A, CD20, CD10, CD43, BCL6, Vs38c and Ig	IGH rearrangement; Plasmacytoid BL variant	Neg	CNs, heart, lung, BM; stage IV	Complete orbital tumor regression at 5 months, but persistent lymphadenopathy
49-year-old Diabetic Black female (Current case)	Orbital cellulitis; Antibiotic Tx Pseudotumor; Steroid Tx	Presented to dentist with 5 days Hx of tooth pain and painless lid edema OD; 5 days later at ER – teeth pain, jaw numbness and progressive lid edema, and new onset diabetes; Returned 6 days later with progressive loss VA, ROM and proptosis; CT scans showed progressive right superior orbital mass and sinusitis.	Hx: “starry sky” appearance; high-grade B cell lymphoma; B cell markers; CD19, CD22, CD24, CD30, CD79A, and cytoplasmic kappa light chain	IGH rearrangement; Variant c-MYC translocation [either t(8;22) or t(8;2)]	Neg	CNs, spine, chest, abdomen, pelvis, heart, kidney, urinary bladder. elevated LDH stage IV	Alive, 5 years post Dx with normal ocular function

Abbreviations: Tx, treatment; Hx, history; Dx, diagnosis; VA, visual acuity; ROM, restricted ocular motility; CT, computed tomography; WBC, white blood count; BM, bone marrow; LDH, lactose dehydrogenase; COD, cause of death; ND, not done.