Abstract 5201

Orbital Non Hodgkin Lymphoma (NHL) represents 2 to 5% of extranodal NHL and 8 to 11% of orbital tumors. Published reports usually comprise a reduced number of patients.

Out of a total of 668 extranodal NHL treated in the period 1991–2010, the orbital locations accounted for 63 patients (10.6%).This high value is due to selected referral from ophthalmologic centers. The diagnosis was made by cytology or surgical biopsy and immunohistochemistry. Staging included CT or MRI of the orbit and brain and bone marrow biopsy.

Results:

Age range:17 to 86 years (mean 60), only 5 < 40 years. The ratio M/F: 1/0.9.Location:right orbit: 50%,left: 36%, bilateral: 14%. Conjunctiva: 44.5%, orbit soft tissue: 23.8%, eyelid: 22.2%, lachrymal gland: 9.5%.

Initial symptoms: orbital tumor: 47.6%, proptosis: 26.9%, ptosis: 11.1%, eyelid edema: 6.3%, diplopia: 4.7%, epiphora: 3.2%.

Histology subtypes: MALT: 50.8%, lymphocytic: 30.1% LDGCB: 6.3%, follicular 6.3%, lymphoplasmacytic: 4.7%, angiocentric T: 1.6%. Time to diagnosis was above 12 months in 63.5%.

Treatment in 49 patients (77.7%). Chemotherapy (CT) (CVP, CHOP, CLB) plus radiotherapy (RT) in 57,14 %, CT in 28.57%, radiotherapy: 12.24%, Rituximab:.28% (12.24% + QT, 2.04% alone).

Complete remission (CR) was obtained in 87.77% and PR in 12.23%. Relapse in 16 (32.65%), local homo-or contralateral 9 (56.25%), systemic 6 (37.50%), 1 (6.25%) in breast (MALT). Died in progression: 4 (25%). The 46,93% of patients had survivals of 5 to 10 years, and 12.5% 11 to 15 years.

Conclusions

The outstanding features are: presentation in 5* to 6* decade, low grade subtypes, initial symptoms: painless mass and proptosis, prevalent location in conjunctiva and eyelid. Is remarkable the delay until diagnosis, due to its low frequency and low specificity of the initial symptoms. The high rate of complete remission obtained with chemotherapy and radiotherapy in frontline and relapse and prolonged survival, are characteristics that determine the good prognosis of this location of extranodal NHL.

Disclosures:

No relevant conflicts of interest to declare.

Topics:
argentina, biopsy, bone marrow biopsy, brain, breast, chemotherapy regimen, complete remission, conjunctiva, curie, cyclophosphamide, doxorubicin, prednisone, and vincristine

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2011 by The American Society of Hematology
2011
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