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

Grand Rounds Conference

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

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Grand Rounds Conference

Eric Downing MDUniversity of Louisville

Department of Ophthalmology and Visual Sciences

SubjectiveCC/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.

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 tearsMeds: Norvasc, ASA, Ativan

Objective

OD OSVA: 20/70 20/20Pupils: 4 4IOP: 19 21EOM: full full

ObjectiveSLE: E/L/L: Superotemporal mass under RUL, second mass under right nasolabial foldC/S: White, quiet OUK: Clear OUAC: Deep and quiet OUI/L: Round, PCIOL OU

Hertel 18, 18

Differential diagnosis

Benign lymphoproliferative disorder Metastatic lesion Inflammatory lesion Infectious lesion Epithelial tumor

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.

Plan

MRI with gadolinium Excision of masses with biopsy

MRI

T1 T2

Surgery

Right orbitotomy with bone flap for lacrimal gland excisional biopsy

Right nasolabial fold mass excision

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

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

workup/therapy

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

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

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

History & Physical Painless proptosis with or without motility deficitsPtosisRarely have decreased VAIf conjunctival, can have the characteristic salmon-patch appearanceCervical or preauricular lymphadenopathy, parotid gland swelling, or abdominal mass can portend systemic disease

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

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%)

Biopsy and Histopathology

Essential to confirm diagnosis Histology and immunophenotype Grading

Monoclonal------>Polyclonal 20%--------->60% risk of systemic

disease

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

Follow up

Every 6 months for 2 years with repeat imaging

Annually thereafter

Prognosis

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

ENMZ: extranodal marginal zone, FL: follicular, MCL: mantle cell, LPL: lymphoplasmatic

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

MRI images

DSDE images

Lymphoma IgG4 disease

References

1. BCSC: Orbit, Eyelids, and Lacrimal System. Pp79-842. Rasmussen P, Ralfkiaer E, Prause JU, et al. Malignant

Lymphoma of the Lacrimal Gland. Arch Ophth. 2011 Oct. Vol 129(10):1275-1280.

3. Eckardt et al. Orbital Lymphoma: diagnostic approach and treatment outcome. World Journal of Surgical Oncology. 2013. 11:17

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):1251-5

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. Eur Radiol. 2014, 24:581-586