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Grand Rounds Conference Eric Downing MD University of Louisville Department of Ophthalmology and Visual Sciences.

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Presentation on theme: "Grand Rounds Conference Eric Downing MD University of Louisville Department of Ophthalmology and Visual Sciences."— Presentation transcript:

1 Grand Rounds Conference Eric Downing MD University of Louisville Department of Ophthalmology and Visual Sciences

2 Subjective CC/HPI: 77M referred by a local oncologist for a painless “bump” on RUL for 2 months. Pt had similar bumps on LUL in 2006, which were excised and identified as benign, per pt.

3 History POH: lump removed over left eye, CEIOL OU, non-ischemic CRVO PMH: Throat cancer (2003) treated with 6 rounds of chemotherapy, multiple kidney stones (2012)TIA (2008), HTN Eye Meds: artificial tears Meds: Norvasc, ASA, Ativan

4 Objective OD OS OD OS VA: 20/7020/20 Pupils: 44 IOP:1921 EOM:fullfull

5 Objective SLE: E/L/L: Superotemporal mass under RUL, second mass under right nasolabial fold C/S:White, quiet OU K:Clear OU AC:Deep and quiet OU I/L:Round, PCIOL OU Hertel 18, 18

6 Differential diagnosis Benign lymphoproliferative disorder Benign lymphoproliferative disorder Metastatic lesion Metastatic lesion Inflammatory lesion Inflammatory lesion Infectious lesion Infectious lesion Epithelial tumor Epithelial tumor

7 Assessment 57M with history of throat cancer (2003), treated with chemotherapy, previously in remission, who presented with a right superotemporal orbital mass for two months. 57M with history of throat cancer (2003), treated with chemotherapy, previously in remission, who presented with a right superotemporal orbital mass for two months.

8 Plan MRI with gadolinium MRI with gadolinium Excision of masses with biopsy Excision of masses with biopsy

9 MRI T1 T2 T1 T2

10 Surgery Right orbitotomy with bone flap for lacrimal gland excisional biopsy Right orbitotomy with bone flap for lacrimal gland excisional biopsy Right nasolabial fold mass excision Right nasolabial fold mass excision

11 Pathology Biopsy: Follicular lymphoma, grade 3A Biopsy: Follicular lymphoma, grade 3A CD 10, CD 20, BCL-2, and BCL-6 positive CD 10, CD 20, BCL-2, and BCL-6 positive Ki67 positive in 30-40% of cells Ki67 positive in 30-40% of cells CD10, BCL-2: Follicular CD10, BCL-2: Follicular CD10/19/20/22/23: B cell CD10/19/20/22/23: B cell Ki67 is a cell proliferation marker Ki67 is a cell proliferation marker CD2/3/4/5/7/8/56: T-cell lymphoma CD2/3/4/5/7/8/56: T-cell lymphoma

12 Post-op Pt doing well Pt doing well VA OD improved slightly from 20/70 to 20/50 VA OD improved slightly from 20/70 to 20/50 Release back to Dr. Woodcock for further workup/therapy Release back to Dr. Woodcock for further workup/therapy

13 Orbital Lymphoma Orbital lymphomas account for 8-15% of extranodal non-Hodgkins lymphomas Orbital lymphomas account for 8-15% of extranodal non-Hodgkins lymphomas MALT lymphomas are the most common (~50%), followed by the follicular type (~25%) MALT lymphomas are the most common (~50%), followed by the follicular type (~25%) Systemic involvement in approx 1/3 of cases Systemic involvement in approx 1/3 of cases 5 year survival rate approaches 100% for follicular lacrimal gland tumors, and 70% overall for extranodal marginal zone tumors 5 year survival rate approaches 100% for follicular lacrimal gland tumors, and 70% overall for extranodal marginal zone tumors Divided into 3 grades which refer to the number of centroblasts per HPF Divided into 3 grades which refer to the number of centroblasts per HPF

14 Pathophysiology Progressive clonal expansion of B, T, or NK cells due to mutations affecting the proto- oncogenes or tumor-suppressor genes Progressive clonal expansion of B, T, or NK cells due to mutations affecting the proto- oncogenes or tumor-suppressor genes Majority are non-Hodgkin B-cell lymphomas (90%) Majority are non-Hodgkin B-cell lymphomas (90%) Often associated with infectious entities, such as Chlamydia psittaci Often associated with infectious entities, such as Chlamydia psittaci

15 Epidemiology Lifetime risk for NHL is 2% Lifetime risk for NHL is 2% Typically affects elderly patients, with mean ages in the seventh decade Typically affects elderly patients, with mean ages in the seventh decade Slight female predominance Slight female predominance Asians/Pacific Islanders>whites>blacks Asians/Pacific Islanders>whites>blacks Increases of >6% annually between Increases of >6% annually between Increasing incidence, most likely due to better diagnostic techniques, the aging population, increased use of immunosuppressive drugs, and HIV/AIDS Increasing incidence, most likely due to better diagnostic techniques, the aging population, increased use of immunosuppressive drugs, and HIV/AIDS

16 History & Physical Painless proptosis with or without motility deficits Painless proptosis with or without motility deficits Ptosis Ptosis Rarely have decreased VA Rarely have decreased VA If conjunctival, can have the characteristic salmon-patch appearance If conjunctival, can have the characteristic salmon-patch appearance Cervical or preauricular lymphadenopathy, parotid gland swelling, or abdominal mass can portend systemic disease Cervical or preauricular lymphadenopathy, parotid gland swelling, or abdominal mass can portend systemic disease

17 Work-up Labortatory studies Labortatory studies CBC, RPR, ESR, LDH, FTA-Abs, HIV CBC, RPR, ESR, LDH, FTA-Abs, HIV Lumbar puncture Lumbar puncture Imaging Imaging CT: seen as well-defined, high density, lobulated or nodular masses with sharp margins. Usually no bony erosion. CT: seen as well-defined, high density, lobulated or nodular masses with sharp margins. Usually no bony erosion. Usually extraconal Usually extraconal 10-17% involve the lacrima 10-17% involve the lacrima

18 Imaging (con’t) MRI MRI Decreased ability to evaluate for bony destruction Decreased ability to evaluate for bony destruction May miss conjunctival disease May miss conjunctival disease T1: isointense T1: isointense T2: hyperintense T2: hyperintense PET PET Best to check for systemic/nodal involvement Best to check for systemic/nodal involvement Higher sensitivity than CT (86% vs. 72%) Higher sensitivity than CT (86% vs. 72%)

19 Biopsy and Histopathology Essential to confirm diagnosis Essential to confirm diagnosis Histology and immunophenotype Histology and immunophenotype Grading GradingMonoclonal------>Polyclonal 20% >60% risk of systemic disease 20% >60% risk of systemic disease

20 Treatment Radiotherapy is the treatment of choice (97-100% local control) Radiotherapy is the treatment of choice (97-100% local control) Dose of cGy Dose of cGy Surgery is usually NOT recommended due to their infiltrative nature Surgery is usually NOT recommended due to their infiltrative nature Systemic disease Systemic disease Low-grade generally refractory to chemotherapy, but often have a long survival rate, even if untreated Low-grade generally refractory to chemotherapy, but often have a long survival rate, even if untreated Aggressive lesions are treated with radiation and chemotherapy—often Methotrexate +/- procarbazine, vincristine, thiotepa Aggressive lesions are treated with radiation and chemotherapy—often Methotrexate +/- procarbazine, vincristine, thiotepa Intralesional Rituximab for low-grade lymphomas (Savino) Intralesional Rituximab for low-grade lymphomas (Savino) Complications of radiotherapy: conjunctivitis, cataract, dry eye, corneal ulcer, NVG, optic neuropathy Complications of radiotherapy: conjunctivitis, cataract, dry eye, corneal ulcer, NVG, optic neuropathy

21 Follow up Every 6 months for 2 years with repeat imaging Every 6 months for 2 years with repeat imaging Annually thereafter Annually thereafter

22 Prognosis Decreases with systemic involvement and/or bilateral disease Decreases with systemic involvement and/or bilateral disease Major factors Major factors Anatomic location Anatomic location Stage at presentation Stage at presentation Subtypes (ENMZ(0-20%)>FL(20-37%)>MCL( %>LPL(14-100%) Subtypes (ENMZ(0-20%)>FL(20-37%)>MCL( %>LPL(14-100%) Immunohistochemical markers Immunohistochemical markers 20-25% will develop disseminated disease within 5 years 20-25% will develop disseminated disease within 5 years ENMZ: extranodal marginal zone, FL: follicular, MCL: mantle cell, LPL: lymphoplasmatic

23 Research Analyzed scans of 23 patients with either orbital lymphoma or IgG4 disease Analyzed scans of 23 patients with either orbital lymphoma or IgG4 disease All patients underwent both MRI and Diffusion- sensitised driven-equilibrium prep (DSDE) All patients underwent both MRI and Diffusion- sensitised driven-equilibrium prep (DSDE) Used an apparent diffusion coefficient to differentiate Used an apparent diffusion coefficient to differentiate Lymphoma has a lower ADC than IgG4 tissue, appearing darker, and giving us an additional modality to differentiate the two Lymphoma has a lower ADC than IgG4 tissue, appearing darker, and giving us an additional modality to differentiate the two

24 MRI images DSDE images Lymphoma IgG4 disease

25 References 1. BCSC: Orbit, Eyelids, and Lacrimal System. Pp Rasmussen P, Ralfkiaer E, Prause JU, et al. Malignant Lymphoma of the Lacrimal Gland. Arch Ophth Oct. Vol 129(10): Eckardt et al. Orbital Lymphoma: diagnostic approach and treatment outcome. World Journal of Surgical Oncology :17 4. Savino G, Battendieri R, Gari M, et al. Long-term outcomes of primary ocular adnexal lymphoa treatment with intraorbital rituximab injections. J Cancer Res Clin Oncol. 2013; 139(7): Hiwatashi A, Yoshiura T, Togao O. Diffusivity of intraorbital lymphoma vs IgG4 related disease: 3D turbo field echo with diffusion-sensitised drive- equilibrium preparation technique. Eur Radiol. 2014, 24:


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