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Journal Français d'Ophtalmologie
Volume 41, n° 3
pages e125-e128 (mars 2018)
Doi : 10.1016/j.jfo.2017.10.005
Posters de la SFO

Pseudo-panuveitis masquerade syndrome associated with an orbital and systemic MALT-type B-cell lymphoma
Lymphome orbitaire et systémique de type MALT se manifestant par une panuvéite

R.K. Khanna a, , N. Vallet b, G. Fromont-Hankard c, P.-J. Pisella a, M.-L. Le Lez a
a Service d’ophtalmologie, CHRU Bretonneau, université François-Rabelais de Tours, 2, boulevard Tonnellé, 37044 Tours, France 
b Service d’hématologie et thérapie cellulaire, CHRU Bretonneau, 37044 Tours, France 
c Service d’anatomopathologie, CHRU Bretonneau, université François-Rabelais de Tours, 2, boulevard Tonnellé, 37044 Tours, France 

Corresponding author. 14, rue de l’Elysée, 37000 Tours, France.14, rue de l’Elysée, 37000 Tours, France.

Orbit and orbital adnexae tumors are rare, comprising only 1% of all non-Hodgkin's lymphoma. In most cases, warning signs are non-specific causing delay in diagnosis and specific treatment. We describe the case of a 49-year-old women sent by her ophthalmologist for right eye redness since six months.

Case report

At initial examination, best-corrected distance visual acuity was 20/25 on right eye and 20/20 on left eye. Near visual acuity was Parinaud 2 in both eyes. Right eye presented conjunctival hyperemia (Figure 1) with non-granulomatous anterior uveitis without synechia or high intraocular pressure. Anterior chamber inflammation was grade 2 according to the Standardization of Uveitis Nomenclature, and the vitreous was infiltrated with presence of cells (grade 1 according to the National Institutes of Health classification). Chorioretinal folds radiating from the optic disc were found at right eye fundus examination. Left eye examination was normal except the presence of small white choroidal lesions at the nasal side of the optic disc (Figure 2). For the right eye, between the macula and the optic disc, fundus autofluorescence (FAF) image demonstrated a granular pattern of slight hypoautofluorescent and hyperautofluorescent spots. FAF also revealed a descending tract with granular hyper-autofluorescence (Figure 3). Between the macula and the optic disc, FA showed hyperfluorescent lesions, with pinpoints, and hypofluorescent spots that corresponded to the hyperautofluorescent lesions (Figure 4). Indocyanine green angiography revealed hypocyanescent choroidal spots on the left eye at the midphase (Figure 5). Anterior chamber puncture was performed: IL-10 to IL-6 ratio ruled out primary intraocular lymphoma. Magnetic resonance imaging showed an orbital intraconal 12 cubic centimeter mass. 18-Fluorodeoxyglucose positron emission tomography revealed high uptake of this mass associated with generalized lymphadenopathy (two cervical lymph nodes and disseminated lymph nodes in both sides of the diaphragm). The patient underwent mass biopsy by lateral orbitotomy. Anatomopathological examination led to the final diagnosis: small B-cell mucosa-associated lymphoid tissue (MALT) orbital lymphoma stage IV Ann Arbor with bilateral choroidal infiltration. Unfortunately, optic nerve was damaged during the biopsy causing functional loss with only light perception remaining. Multidisciplinary cancer management led to rituximab-chloraminophene chemotherapy [1]. Fifteen days later, the patient presented left eye visual acuity loss: 20/40, Parinaud 2. Slit lamp examination revealed conjunctival hyperemia associated with anterior chamber inflammation. Chorioretinal folds and multiple serous retinal detachments were found at the fundus examination. Macular optical coherence tomography was similar to the right eye initial examination: macular subretinal fluid; undulating of retinal pigment epithelium (Figure 6). Oral corticosteroid (prednisone 1mg/day) was given to the patient and resulted in functional and anatomical improvement. However at ocular motility examination, we noticed right eye esotropia and hypotropia. Magnetic resonance imaging showed lateral and superior rectus involvement (Figure 7). Chemotherapy was pursued during a year with progressive decrease of the corticosteroid treatment. Six months after the last chemotherapy cycle, MRI showed partial remission of the right intraorbital intraconal tumoral mass (Figure 8).

Figure 1

Figure 1. 

Anterior segment photography: conjunctival hyperemia.


Figure 2

Figure 2. 

Fundus photography: cream-colored choroidal lesions in the nasal side of the optic disc.


Figure 3

Figure 3. 

Fundus autofluorescence: granular pattern of slight hypoautofluorescent and hyperautofluroescent spots. Descending tract with granular hyper-autofluorescence.


Figure 4

Figure 4. 

Fluoresce in angiography at early phase 1minute after injection: hyperfluorescent lesions, with pinpoints, and hypofluorescent spots. Retinal folds.


Figure 5

Figure 5. 

Indocyanine green angiography at midphase 13minutes after injection: hypocyanescent choroidal spots on left eye.


Figure 6

Figure 6. 

Left eye optic coherence tomography: undulating of retinal pigment epithelium with internal retina layer folds. Sub retinal fluid accumulation.


Figure 7

Figure 7. 

Magnetic resonance imaging: right orbital intraconal 12cubic centimeter mass surrounding optic nerve with extension towards lateral and superior rectus.


Figure 8

Figure 8. 

Magnetic resonance imaging: significant orbital mass volume decrease 6 months after chemotherapy.



Ocular involvement in systemic lymphoma is rarely described. Uveal inflammation can be caused by infiltration of lymphoma cells. On the other hand, it can also be found in paraneoplastic syndrome. In our case, any of these two conditions could explain the clinical manifestations, especially the presence of contralateral lesions. The MRI findings of an orbital mass suggested a tumoral process. Final diagnosis is made by anatomopathological examination. The risk-benefit ratio of an orbital mass biopsy has to be considered. It has to be known that the optic nerve can be damaged during the procedure. Thus, cunjunctiva [2, 3] or lymph nodes analyses should be preferred [4]. Close follow-up with body imaging is indicated since systemic lymphoma can occur at a later stage [5] or at diagnosis [6]. Retinal pigment undulation seen on OCT can be explained by the presence of choroidal thickening secondary to tumoral cells infiltration like lymphocytes accumulation in VKH disease [7]. Indocyanine green angiography has to be performed in order to diagnose choroidal infiltration [8]. These foci may represent regions of choroidal space-occupying lymphoma cells. It can be useful to repeat angiography for follow-up in order to evaluate chemotherapy efficiency. In this case, since right eye suffered from functional loss after biopsy, chemotherapy treatment decision was related to the presence of choroidal infiltration at indocyanine green examination on the left eye. On the contrary, in localized orbital lymphoma, radiotherapy could have been an alternative [9]. Some studies report conjunctival MALT-type lymphoma which can extend into the orbit and invade local tissues such as the choroid [2, 10, 11]. Ocular motility disorders can be signs of muscles involvement by an orbital mass, which requires imaging evaluation.


As a conclusion, uveal and orbital lymphoma has to be evoked in presence of chronic ocular inflammation or indirect signs, such as choroidal folds, of ocular compression by a retrobulbar mass.

Disclosure of interest

The authors declare that they have no competing interest.

 This case has been presented as an e-poster at the French Ophthalmology Society congress in 2016.


Aronow M.E., Portell C.A., Sweetenham J.W., Singh A.D. Uveal lymphoma: clinical features, diagnostic studies, treatment selection, and outcomes Ophthalmology 2014 ;  121 : 334-34110.1016/j.ophtha.2013.09.004 [cross-ref]
Ramulu P., Iliff N.T., Green W.R., Kuo I.C. Asymptomatic conjunctival mucosa-associated lymphoid tissue-type lymphoma with presumed intraocular involvement Cornea 2007 ;  26 : 484-48610.1097/ICO.0b013e3180307667 [cross-ref]
Hoang-Xuan T., Bodaghi B., Toublanc M., Delmer A., Schwartz L., D’Hermies F. Scleritis and mucosal-associated lymphoid tissue lymphoma: a new masquerade syndrome Ophthalmology 1996 ;  103 : 631-635 [cross-ref]
Stacy R.C., Jakobiec F.A., Herwig M.C., Schoenfield L., Singh A., Grossniklaus H.E. Diffuse large B-cell lymphoma of the orbit: clinicopathologic, immunohistochemical, and prognostic features of 20 cases Am J Ophthalmol 2012 ;  154 : 87-98010.1016/j.ajo.2012.01.021 [inter-ref]
Demirci H., Shields C.L., Karatza E.C., Shields J.A. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases Ophthalmology 2008 ;  115 : 10.1016/j.ophtha.2008.02.004[1626-31–631.e1-3].
Gaucher D., Bodaghi B., Charlotte F., Schneider C., Cassoux N., Lemaitre C., and al. MALT-type B-cell lymphoma masquerading as scleritis or posterior uveitis J Fr Ophtalmol 2005 ;  28 : 31-38 [inter-ref]
Maruko I., Iida T., Sugano Y., Oyamada H., Sekiryu T., Fujiwara T., and al. Subfoveal choroidal thickness after treatment of Vogt-Koyanagi-Harada disease Retina 2011 ;  31 : 510-51710.1097/IAE.0b013e3181eef053 [cross-ref]
Saatci A.O., Arikan G., Ozcan M.A., Ozkal S., Kargi A., Undar B. Indocyanine green angiographic features of systemic non-Hodgkin's lymphoma and bilateral choroidal involvement Ophthalmic Surg Lasers Imaging 2006 ;  37 : 236-239
Loriaut P., Charlotte F., Bodaghi B., Decaudin D., Leblond V., Fardeau C., and al. Choroidal and adnexal extranodal marginal zone B-cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases Eye 2013 ;  27 : 828-83510.1038/eye.2013.74 [cross-ref]
Sarraf D., Jain A., Dubovy S., Kreiger A., Fong D., Paschal J. Mucosa-associated lymphoid tissue lymphoma with intraocular involvement Retina 2005 ;  25 : 94-98 [cross-ref]
Desai A., Joag M.G., Lekakis L., Chapman J.R., Vega F., Tibshirani R., and al. Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study Blood 2017 ;  129 : 324-33210.1182/blood-2016-05-714584 [cross-ref]

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