Grand Rounds Conference
-
Upload
jescie-moore -
Category
Documents
-
view
23 -
download
1
description
Transcript of Grand Rounds Conference
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
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.
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
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
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