 Good morning, everyone. It's after eight o'clock. So let's go ahead and get started. We have two speakers today Dr. Zog. He's one of our own. He's the interns and then followed by dr. And body good morning, so I'm going to talk about a subject that I'm sure I'll become more accustomed to learning about as I go on through residency, but I Titled my talk my my left eye is blind We're going to go through a case presentation that we're going to learn what the signs and symptoms are of ocular lymphoma and Options for diagnosis and treatment and then we'll talk about just a few take home points So the first thing we had a 63 year old male who was brought over to my continuity clinic on Wednesday afternoon for a cns lymphoma that's what he'd been diagnosed with and The primary team just wanted us to rule out any intraocular involvement and they reported that the patient had absolutely no visual complaints Which I think is a common thing in that our residents experience. So the chief complaint for the patient was my left eye is blind I Can only see shadows out of that left eye So as we know that the chief complaint is the first part of taking a history and anything and This wasn't done in this patient before he came over to us. So he described a sudden loss of vision about three to five days before Presenting to us. There was no pain He could just see shadows out of that left eye He said his vision hadn't changed much since then no improvement. No worsening his right. I also seemed a little bit blurry to him He had a headache kind of in the back of his head No scalp tinnitus no jaw claudication of any kind He was diagnosed with B cell lymphoma in September. I just saw this patient a few weeks ago He was treated with six cycles of our chop and then two days ago He presented to the ER with new onset of ataxia and altered mental status and they did an MRI at that time and found Some CNS lesions Likely representing lymphoma. This was all presented to us from the patient and the family. This is all their their history So to look back at his past medical history They reported that they were treated for some type of uveitis the patient was back in September That didn't seem to respond to topical prednisone and we couldn't get a whole lot more information about it We didn't have any of his chart information either. So he had Chronic lymphocytic leukemia diagnosed in 2004 which switched over to B cell lymphoma in 2011 He'd also been diagnosed with prostate cancer in 2004 and then just some other minor past medical stuff That's not really pertinent In talking to him about reviews systems. He'd been having fevers weight loss muscle pain He was tired a lot of easy bruising. He was having a hard time walking and just felt overall confused When we checked his vision on his right eye, he was 20 70 pinhold to 20 60 his left eye He was light perception only The right pupil was pretty sluggish and his left. He had a left APD His motility had a little bit of abduction deficit in his left eye, but otherwise they were pretty full He seemed to have confrontational fields in his right eye that were full, but his left eye He couldn't perform anything on We did it perform eventually a Humpery visual field which is so showed kind of global depression in his right eye not really any Focal findings on that and color plates he was one out of 12 in his right eye And he was kind of confused by that because he had apparently had normal color vision this whole life And again, we couldn't really check his left eye He also stated that red things didn't quite seem as red to him in his in his right eye His pressures were upper end of normal 22 and 21 On exam. He didn't really have anything externally his v12 and three were totally intact. No and ophthalmus or proptosis Really not a whole lot on exam even looking at his anterior chamber. He was nice deep and quiet. No cellar flare at all His vitreous. He had a nice clear view. No haze His optic nerves There was no pallor and no edema at all and no hemorrhage is helped healthy looking nerve and then all the retina and everything Actually looked really nice and normal as well So on the MRI Which the primary team had when they sent the patient to us It had leptomaninjual enhancement the posterior fossa and they also had a lot of hyper intense flare in the optic chiasm and also the pre-chiasmatic and retrochiasmatic optic tract and nerve and It had some involvement of the optic radiations as well. So pretty extensive visual pathway involvement on the MRI So we came to the conclusion I saw this patient with dr. Petty and Dr. Henderson helped not Henderson. I always call her Bonnie Henderson Bonnie helped us out the neuro op fellow 63 year old male with a visual pathway involvement of CNS lymphoma, but he didn't have any evidence of intraocular involvement But he had evidence of optic neuropathy really in both eyes with the loss of color vision and red Desaturation is right eye and then the APD on the left. So really the treatment for this is just to treat the underlying lymphoma in his case So with primary CNS lymphoma the typical onset is in the mid 50s It's really rare in children or adolescents. The incidence is increasing for unknown reasons One of the strange things about this is there's not really any lymphatic tissue in the CNS or within the orbit Within the intraocular space at least and so it's kind of a strange Cancer in that sense and it's reported that if somebody has primary CNS lymphoma that they'll have Ocular lymphoma in about 25% of cases. So how are they going to present to us? I think The main thing hopefully is that they'll have some sort of visual complaint They might come to us as a rule out as this guy did in this case, but they might notice visual someone might notice visual field defects difficulty moving their eyes and some sort of optic neuropathy nice stagma something like that They could also present with intraocular inflammation and usually there's two different types. There's vitriol retinal lesions that you'll see in On in the fundus and these are usually associated with an independent primary CNS lymphoma Or you can have this the uveal type which is typically a metastatic origin from a systemic lymphoma And then the other patients are what we get a lot are the patients who are diagnosed with primary CNS lymphoma and They could have ocular involvement whether it's metastatic spread Direct spread or it could be an independent finding as well The classic boards presentation for their for us as residents is some sort of posterior uveitis with a lack of response to Topical steroids which it sounds like this guy had some of that and it's classically one of the masquerade syndromes in uveitis one of the big ones So with primary intraocular lymphoma what you'd normally see is vitreous cell And then looking for the lesions you'll see kind of geographic sub retinal Infiltrate of masses and sometimes you'll have little satellite lesions around the the main cancer It's bilateral in a majority of cases eighty to ninety percent Less common findings are intra retinal hemorrhages And retinal artery obstructions on f a the classic finding is hypo fluorescence early and then late hyperfluorescence I don't know how well this Projects okay, you can kind of see the white yellow areas Indicating sub retinal involvement with they call it kind of this stippling over the top of these white lesions Just another fundus photo of kind of similar things This one's a little bit too dark, but it was a good montage of kind of a full retina what it might look like if you See a patient with this This is an f a showing some hyperfluorescence Some patients may present if they have excessive intraocular inflammation with hypo pion as well The primary uvial lymphoma they describe this a little bit differently It's multifocal creamy yellow chorotal infiltrates and again It's associated with lymphoma. That's more of a systemic origin. It's metastatic and a lot of times this has few vitreous cells and Then f a you might see blockage from the pigment clumps On ultra sound usually what you'll see is just diffuse uvial thickening kind of just behind the retina This might light up on the ultra sound This is a picture of kind of a Like I could I'd have that Dr. Harry help us with that one But it just looks like there's something behind the eye something bright back there So how do we diagnose this a lot of times these patients come with a CBC that might be suspicious But MRI of the brain and LP Really you got to get some cells and you might find them on lumbar puncture or You might be able to buy out see a lesion that you find outside of the eye on imaging But really for us where we might be involved is with paris planar retracted me and you send that for cytology A lot of times the first sample can be negative And so if you have a high clinical suspicion you do need to get a second sample And you can also do a choreo retinal incisional biopsy on an area that you might not think will have any visual potential in the future As well to get that and there's some studies about IL-6 and IL-10 concentrations If the IL-10 to IL-6 ratio is greater than one that's suggestive of lymphoma So kind of a diagnostic tree here first you have to have clinical suspicion for ocular lymphoma You do a funness exam If you can't really see The vitreous very well you might do a fluorescein angiography a lot of these patients will get one of those anyway And you'll see the lesions there. This kind of brings you back to One of the main things that you need to do is get an MRI Make sure there's no CNS involvement if somebody presents with primary intraocular lymphoma Their chance of having CNS lymphoma is a lot higher for some reason it's up in the 50 to 60 percent range in a lot of studies But so try to get some tissue lumbar puncture is probably the first thing that you want to do if you can get some Cells that are positive there, then you'll get an oncologist involved Otherwise you might turn to vitrectomy if that's positive then you turn to treating that if that's negative Then you might have to repeat that vitrectomy if you're really suspicious or biopsy one of the areas that is really suspicious on pathology You'll see malignant lymphoid cells and the features of these are usually the large cell lymphomas and they're they're generally B cell type And basically a lymphoid cell would have pleomorphic nuclear and scant And you might see a lot of necrotic lymphocytes as well This is a typical Cytology so this might be a par's plan of retrectomy with some cells that are smeared out on a plate You can see in the Kind of at the top there a mitotic figure and just a lot of atypical looking lymphocytes with the clump chromatin nuclear line that sort of thing Another kind of close-up of some atypical lymphocytes there On histology, this is a view of the retina maybe a coriorentinal biopsy that you might do The R obviously is the retina the C is the coroid and the arrow is pointing to the limp Lymphoma cells and you can see a separation there between Brux membrane and RPE and a lot of times these patients have kind of an RPE detachment that you can see on Imaging and also on on fluorescein angiography So that's kind of a typical what a typical biopsy would look like and the coroid in this picture on the right has a lot of You can see a lot of blue cells in there Indicative of chronic inflammation as well So the treatment of primary intraocular lymphoma Generally speaking it seems like radiation therapy is the typical treatment to the eye and What I've found is that the prognosis is not very good you the survival is about five years just in intraocular lymphoma and the recurrence rate is pretty high from the studies that I was reading So some groups are moving more towards chemotherapy and actually intravitural injection of like methotrexate and things like that It seems like the success rates a little bit better with that type of treatment So generally a good initial response to radiation, but relapse occurs in a majority of cases You end up with a lot of atrophic sub-retinal scars if you're able to get this into remission So you'll lose some some sight out of those areas Pretty much it there. So I think what I got out of this number one dr. Vitaly kind of Gets this into our minds pretty early on that you need we need to diagnose uveitis and not just treat it initially But if you have something that is not responding to treatment then think about one of the masquerade syndromes of uveitis and especially this Excuse me lymphoma and really tissue is the issue in all Oncology, so you need to get cells by the tractomy or lumbar puncture And I think for me not all rule-outs are asymptomatic despite what they come to you as and then make sure that you just take a look at the Whole patient when you're examining the eye because you might make a systemic diagnosis that will really change the patient's life So that's it any questions Yeah So the real key is just to get them it just needs to be sent to pathology for cytology That's the real key and I don't dr. Mamos do you get these at all or do they go over to the hospital when they're So when I was reading about it, they usually do recommend like a 40 cc tap Because there's a lot of studies you'll send it for cytology or send it for flow cytometry And then just kind of the regular in your differential. You might send it for infectious things PCR all those kind of things. Yeah, so we we did not think his prognosis was very good We basically told him that it was unlikely that he would get any better despite treatment, but We're gonna have him come back and just see how he's doing. He's I checked up on his chart last night He's had chemotherapy and they seem he seemed to have a good symptomatic response to the chemotherapy as far as their written neuro exam But I don't know ocular They're generally B cell lymphoma, but there were there were probably five or six case reports in the literature that were T cell