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Grand Rounds Conference

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

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Grand Rounds Conference

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  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 VA: 20/70 20/20 Pupils: 4 4 IOP: 19 21 EOM: full full

  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 Metastatic lesion Inflammatory lesion Infectious lesion 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.

  8. Plan MRI with gadolinium Excision of masses with biopsy

  9. MRI T1 T2

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

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

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

  13. Orbital Lymphoma Orbital lymphomas account for 8-15% of extranodal non-Hodgkins lymphomas MALT lymphomas are the most common (~50%), followed by the follicular type (~25%) 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 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 Majority are non-Hodgkin B-cell lymphomas (90%) Often associated with infectious entities, such as Chlamydia psittaci

  15. Epidemiology Lifetime risk for NHL is 2% Typically affects elderly patients, with mean ages in the seventh decade Slight female predominance Asians/Pacific Islanders>whites>blacks Increases of >6% annually between 1975-2001 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 Ptosis Rarely have decreased VA If conjunctival, can have the characteristic salmon-patch appearance Cervical or preauricularlymphadenopathy, parotid gland swelling, or abdominal mass can portend systemic disease

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

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

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

  20. Treatment • Radiotherapy is the treatment of choice (97-100% local control) • Dose of 20-40 cGy • Surgery is usually NOT recommended due to their infiltrative nature • Systemic disease • 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 • Intralesional Rituximab for low-grade lymphomas (Savino) • Complications of radiotherapy: conjunctivitis, cataract, dry eye, corneal ulcer, NVG, optic neuropathy

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

  22. Prognosis ENMZ: extranodal marginal zone, FL: follicular, MCL: mantle cell, LPL: lymphoplasmatic • Decreases with systemic involvement and/or bilateral disease • Major factors • Anatomic location • Stage at presentation • Subtypes (ENMZ(0-20%)>FL(20-37%)>MCL(38-100%>LPL(14-100%) • Immunohistochemical markers • 20-25% will develop disseminated disease within 5 years

  23. Research • Analyzed scans of 23 patients with either orbital lymphoma or IgG4 disease • All patients underwent both MRI and Diffusion-sensitised driven-equilibrium prep (DSDE) • 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

  24. MRI images DSDE images Lymphoma IgG4 disease

  25. References • BCSC: Orbit, Eyelids, and Lacrimal System. Pp79-84 • Rasmussen P, Ralfkiaer E, Prause JU, et al. Malignant Lymphoma of the Lacrimal Gland. Arch Ophth. 2011 Oct. Vol 129(10):1275-1280. • Eckardt et al. Orbital Lymphoma: diagnostic approach and treatment outcome. World Journal of Surgical Oncology. 2013. 11:17 • Savino G, Battendieri R, Gari M, et al. Long-term outcomes of primary ocular adnexal lymphoa treatment with intraorbital rituximab injections. J Cancer Res ClinOncol. 2013; 139(7):1251-5 • 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. EurRadiol. 2014, 24:581-586

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