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Man referred for abnormal retinal exam
Chorioretinal folds and an area of globe indentation temporally with overlying subretinal fluid were seen.
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A 77-year-old man was referred to our office for an abnormal retinal exam. Due to trauma to his right eye as a child, he was functionally monocular and followed yearly for regular eye exams. He did not have any recent ocular complaints.
The patient’s ocular history was otherwise significant for cataract surgery in his left eye 3 years prior. His medical history included hypertension, hyperlipidemia and prostate cancer, for which he had a radical prostatectomy 5 years prior. He drank socially and had a history of smoking two packs of cigarettes per day for 30 years but quit more than 25 years ago. His medications included lisinopril and simvastatin.
Examination
The patient’s vision was hand motion in the right eye and 20/30 in the left eye. He had a right relative afferent pupillary defect, full extraocular motility and normal IOP. His color vision and confrontation visual fields were full in the left eye. On physical exam, the patient had a large right exotropia and a dense white cataract in the right eye. His left eye showed 4 mm of proptosis. His anterior segment exam in the left eye was unremarkable except for a posterior chamber IOL. However, dilated exam revealed multiple chorioretinal folds extending from his fovea temporally and an area of globe indentation temporally with overlying subretinal fluid (Figure 1).
Figures 1a and 1b. Alternating yellow and dark lines (choroidal folds) in the macula extending temporally (a). Area of globe indentation in the temporal field of the left eye with overlying subretinal fluid and retinal pigment epithelium changes (b).
Source: Liang MC, Goldman DR, Heher KL, Duker JS
Figures 2a and 2b. Fluorescein angiography at 48 seconds, in the AV phase, showed no obvious leakage at the fovea or elsewhere. The alternating hypofluorescent and hyperfluorescent bands indicate where the retinal pigment epithelium is compressed at the troughs (leading to relative blocking) and where the Bruch’s membrane and retinal pigment epithelium cells are stretched at the peak of the folds (leading to a relative window defect), respectively (a). At 2 minutes, 16 seconds, there was still no leakage in the macula (b).
Figure 3. B-scan ultrasonography of the left eye was significant for scleral thickening and a well-defined homogenous hypoechoic lesion in the orbit apposed against the external surface of the inferior globe.
Fluorescein angiography did not show any leakage or signs of active inflammation, but it prominently illustrated the chorioretinal folds (Figure 2). Indocyanine green angiography revealed a congested choroid but was otherwise unremarkable. B-scan ultrasonography was also performed, revealing scleral thickening and a well-defined homogeneous retro-orbital mass behind the left eye (Figure 3).
What is your diagnosis?
Chorioretinal folds
The differential diagnosis of choroidal or chorioretinal folds is large but was narrowed considerably in this patient given the presence of a retro-orbital mass on ultrasound. The main concerns were inflammatory diseases such as orbital pseudotumor with posterior scleritis and sympathetic ophthalmia given the patient’s history of trauma, tumor such as lymphoma or metastatic cancer, and vascular lesions such as an orbital cavernous hemangioma.
The patient underwent an MRI of the head and orbits, which confirmed the retro-orbital mass conforming to the left globe with focal extension into the choroid and retina (Figure 4). He also underwent a complete physical exam including normal blood work (CBC, CMP, ESR, ANA, ANCA, ACE, CEA) and a chest X-ray that showed a right apical lung opacity.
Figure 4. T1 fat-saturation MRI of the orbits showed minimal left proptosis and a left-side intraconal retro-ocular mass just lateral to the optic nerve.
Figures 5a and 5b. H&E stain (40×) showed a monotonous population of B lymphocytes (a). It was also strongly positive for CD20 (b) and Bcl-2.
Throughout his primary work-up, the patient began to notice a central “spot” in his visual field. New subretinal fluid under the macula was detected, and he was consequently started on 80 mg of oral prednisone. One week later, his vision was 20/70 in the left eye. The fundus exam and fluorescein angiography were otherwise unchanged. Due to the significant change in his vision and no improvement after oral steroids, lateral orbitotomy with biopsy of the mass was scheduled. Pathology of this mass revealed orbital MALT lymphoma (Figure 5).
Discussion
Orbital MALT lymphoma is a low-grade malignancy that generally affects an older age group, from 50 to 70 years. It is the most common lymphoma of the conjunctiva and orbit and is usually indolent and slowly progressive. Eighty percent of cases are unilateral, and 20% of cases are associated with systemic disease at the time of diagnosis. Possible clinical signs are painless proptosis, diplopia, lid edema, ptosis, a palpable mass or a salmon patch on the conjunctiva. Differential diagnosis includes reactive lymphoid hyperplasia, inflammatory pseudotumor, orbital sarcoid, Wegener’s granulomatosis, and orbital tumors such as cavernous hemangioma, optic nerve meningioma or metastasis, among others.
On imaging, MALT lymphomas typically appear as a well-defined homogenous mass that molds to adjacent structures, and it may rarely involve the lacrimal gland or extraocular muscles. Pathology shows infiltrative anaplastic lymphocytes with large cleaved nuclei and a monoclonal proliferation of B cells. Treatment of MALT lymphoma usually includes local radiation therapy or chemotherapy if systemic disease is present. Surgical resection is not recommended due to high rates of recurrence. The visual prognosis of patients with MALT lymphoma is usually favorable, especially if the disease is confined to the orbit. The prognosis for survival is variable, around 60% to 90% at 5 years.
Follow-up
After the diagnosis of MALT lymphoma was made, our patient received 25 Gy of external beam radiation over 14 sessions, with a post-treatment vision of 20/40. His choroidal folds and proptosis resolved; however, a small amount of subretinal fluid continued to persist.
In addition, to follow up on his prior chest X-ray, the patient underwent a chest CT and PET scan that revealed a right apical nodule associated with FDG uptake. A lung biopsy was performed and revealed stage 1 adenocarcinoma unrelated to the MALT lymphoma. He had a curative lobectomy and does not require further systemic treatment.
References:
- Demirci H, Shields CL, Karatza EC, Shields JA. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology. 2008;115(9):1626-1631.
- Ferreri AJ, Ponzoni M, Guidoboni M, et al. Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial. J Natl Cancer Inst. 2006;98(19):1375-1382.
- Panda P, Forooghian F, Goodglick T, Chan CC, Nussenblatt R, Sen HN. Orbital lymphoma masquerading as panuveitis. Ocul Immunol Inflamma. 2010;18(3):181-183.
- Yadav BS, Sharma SC. Orbital lymphoma: role of radiation. Indian J Ophthalmol. 2009;57(2):91-97.
- Zhou P, Ng AK, Silver B, Li S, Hua L, Mauch PM. Radiation therapy for orbital lymphoma. Int J Radiat Oncol Biol Phys. 2005;63(3):866-871.
For more information:
- Michelle C. Liang, MD, Darin R. Goldman, MD, Katrinka L. Heher, MD, and Jay S. Duker, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.
- Edited by Kavita Bhavsar, MD, and Michelle C. Liang, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.