1
2	History 46 yo woman with 3 months of OS irritation, nonspecific, with no associated visual complaints Ø PMH / POH / ROS / meds /gtts WDWN woman, comfortable-appearing
3	Physical WDWN woman, comfortable-appearing V: 20/20, 20/20 T: 14,15 SLE: WNL Motility: full No proptosis Adnexa: fullness OS upper lid, barely palpable mass beneath orbital rim
4	Adnexa
5	Adnexa
6	Initial Differential Inflammatory Reactive lymphoid hyperplasia Sarcoidosis Orbital pseudotumor Dacryoadenitis Idiopathic sclerosing variant Sjogren’s
7	Initial Differential Neoplastic Lymphoma Primary Epithelial Neoplasia of the Lacrimal Gland Benign Mixed (Pleomorphic Adenoma) Adenoid Cystic (Cylindroma)
8	Initial Differential Infectious TB
9	Labwork and imaging Hbg 11.7 g/dL, WBC 4.1x10³dL^-1, 3% Eos PPD (-) ACE wnl CXR (-)
10	MRI
11	MRI
12	MRI
13	MRI
14	Differential Lacrimal fossa masses ~1/2 are of epithelial origin ~1/2 are benign mixed epithelial ~1/3 are adenoid cystic carcinoma Most of the remainder is lymphoid in origin
15	Differential
16	Intervention:
17	Pathology H&E Crush artifact Diffuse lymphocytic infiltrate
18	Pathology H&E Crush artifact Diffuse lymphocytic infiltrate
19	Pathology Immunohistochemistry CD20 - B cell marker
20	Pathology Immunohistochemistry CD3 - T cell marker
21	Pathology Immunohistochemistry Ki67 - Proliferative index
22	Systemic Evaluation MRI brain/chest/abdomen/pelvis WNL Whole body PET scan WNL Bone marrow - negative flow-cytometry studies Heavy chain gene rearrangement: clonally expanded population
23	Treatment 12 cycles of XRT to left orbit H.pylori eradication therapy Chlamydia eradication therapy (Doxy) Close heme-onc followup Ophthalmology followup - resolution of supraorbital fullness
24	Discussion
25	Orbital Lymphoproliferative Disease Orbital NHL - 9% of all orbital tumors 16/111 in lacrimal fossa (n=114 lacrimal fossa lesions) 8/114 additional lacrimal fossa lesions were “benign reactive hyperplasia” and “atypical hyperplasia”
26	Extranodal MALT, general 8% of Non-Hodgkins Lymphoma Mean age: 60 years 2/3 are Stage I or Stage II at diagnosis 20% B (systemic) symptoms 14% with bone marrow involvement 75% 5-year survival rate
27	Extranodal MALT of the Orbit Prevalence 32 consecutive primary adnexal lymphomas (23 isolated, 3 local tissues, 6 distant spread) 15 MALT (13/15 isolated) 3 lacrimal, 2 conjunctival 8 Diffuse Large B Cell
28	Extranodal MALT of the Orbit Features Median age - 44 years Median duration of symptoms - 11 months Presentation: Mass (65%) / Swelling (35%) / Proptosis (24%) / Irritation (21%) / Diplopia (11%) OD (20) / OS (10) / OU (7) Stage: IAe (25/37) Conjunctiva (51%) / Retrobulbar (24%) / Lacrimal (11%)
29	Extranodal MALT of the Orbit Treatment Rx: radiotherapy alone (29/37), chemo alone (3), surgical excision alone (2) Radiotherapy alone: 1 contralateral orbital relapse after 28 months (median followup 21 months) Median dose: 30 Gy 40 Gy: 2/3 develop xeroophthalmia or cataract
30	Stage Ie or IIe MALT Treatment 85 consecutive patients treated with radiotherapy alone 84 complete remissions 14 relapses 77% Five year disease-free survival
31	Patient-Specific Immunochemistry Positive staining: CD21, CD23, CD20 Negative: CD5, CD23, CD10, BCL-6, CD43
32	Thanks.