This patient was also noted to have a pigmented iris tumor in his right eye.
Impression
Posterior Iris Pigment Epithelial Cyst
Iris pigment epithelial cysts are typically located behind the iris stroma. As they expand, the cyst wall comes in contact with the natural lens (or IOL) posteriorly and push the iris stroma anteriorly. Gonioscopy and high-frequency ultrasound examination usually reveals focal angle closure. Since most of the trabecular meshwork remains open, most patients do not develop narrow angle glaucoma.
Giant iris pigment epithelial cysts can cause total angle closure warranting intervention. These patients have been treated with Yag-laser iridotomies or surgical iridectomy.
Almost all iris pigment epithelial cysts can be monitored with periodic observation for evidence of secondary glaucoma. Slit-lamp photography, gonioscopy and high-frequency ultrasonography are particularly helpful in evaluating for progressive enlargement. The natural history of iris pigment epithelial cysts is poorly understood.
After seed implantation, an x-ray is used to document radioactive seed locations and allow for in vivo dosimetry.
By Paul T. Finger, MD
Dr. Finger has been using the Brachytherapy Boost Technique (BBT) for over 25 years. It is a multidisciplinary approach that can spare patients from exenteration surgery (removal of the eye and all the orbital contents).
During the BBT procedure, instead of removing the eye lids and orbital tissues, the bulk of the orbital tumor is removed and radioactive seeds or HDR catheters are temporarily placed in the tumor-bed. With this method, the area of tumor infiltration is irradiated more than the rest of the remaining ocular and/or orbital structures. An overlay of a reduced amount of external beam radiation therapy is typically given to the entire orbit or targeted zone.
Dr. Finger considers the brachytherapy boost technique in the following clinical situations
1. When the standard external beam radiotherapy would require using a dose so high that it would result in a blind and painful eye (e.g. orbital melanoma, squamous and basal cell carcinoma, adenoid cystic carcinoma).
Catheters (upper right) for radioactive seed implantation are placed at the time of surgery.
2. When exenteration of the orbit is the only option, but offers historically poor local control rates (e.g. adenoid cystic carcinoma).
A relatively new treatment for retinoblastoma quickly gaining popularity has shown some promise, but a systematic review of reported outcomes published in March provides some reason for caution.
Intra-arterial chemotherapy has rapidly emerged as a treatment for intraocular retinoblastoma, the most common primary childhood eye cancer. It involves inserting a microcatheter into the ophthalmic artery and manually injecting chemotherapy drugs though it directly into the eye.
Centers in some 30 countries have adopted the technique, and a few have completely replaced systemic chemotherapy and radiation with intra-arterial chemotherapy as the primary treatment for retinoblastoma.
The researchers performed comprehensive searches in Medline, Embase, Cochrane, and Web of Science from inception through January 2015. They included any peer-reviewed English-language publication that described outcomes related to toxicity or efficacy in at least four patients. Through their search, the doctors identified 28 publications that met their inclusion criteria. The researchers ultimately evaluated cases encompassing 655 patients, 757 eyes, and a total of 2,350 catheterizations.
While they found the treatment yielded a 66% eye-preservation rate, the number of children who subsequently developed metastases raises concern. The researchers concluded that while the treatment seems promising, more study is needed.
“Metastases have been observed, and long-term follow-up is needed. “Until the results of clinical, prospective studies are available, it is recommended that intra-arterial chemotherapy be offered selectively among other options, with fully informed discussion about all possible risks, benefits, and uncertainties.”
The researchers pointed out that the prior method of systemic chemotherapy addressed the issue of metastases, while intra-arterial chemotherapy does not.
“The most important critique of the IAC literature is the uncertain rate of metastases. Eye salvage is only valuable when achieved without a risk to life. Systemic chemotherapy provides protection against metastases, especially in children who may have unknown high-risk pathologic features in retained eyes, but IAC does not.”
While promising, intra-arterial chemotherapy may not be the panacea some doctors hope. The recently published systematic review of reported outcomes indicates some caution is necessary and more study is definitely needed.
You can read the complete study in the March 2016 issues of the JAMA Ophthalmology. Access it HERE.
This digital image shows a rounded pigmented tumor extending from the anterior chamber angle.
This patient was noted to have a pigmented tumor on his right iris.
The digital images below will show a rounded pigmented tumor extending from the anterior chamber angle. Gonioscopy revealed no evidence of pigment dusting around the tumor or tumor in the adjacent ciliary body. It was also significant that there was no evidence of distortion of the iris stroma, ectropion uveae, sector cataract or abnormal vascularity (as might be seen with iris melanoma).
A 63 year old male was referred to The New York Eye Cancer Center with a 6 month history of progressive painless proptosis of the right eye.
These magnetic resonance imaging (MRI) studies demonstrate displacement of the optic nerve, a bright T1 tumor image, and a variably bright T2 tumor image. The tumor is noted to involve the orbit, the ethmoid and frontal sinuses.
Despite this large orbital tumor with optic nerve displacement, the patient was 20/20 OU, he had no visual field defect, and no signs of optic neuropathy. A complete medical survey was initiated and the patient was cleared for surgery. A combination of anterior orbitotomy and transnasal ethmoidectomy were performed to evacuate the mucus and allow for future drainage.
Though recurrence is possible, this surgery is typically curative. We recommend sending the mucoid contents for culture and sensitivity and cytopathology.
A 67 year old male was referred to The New York Eye Cancer Center with a 18 x 18 mm base, and 9.3 mm high dome-shaped tumor in his left eye.
A dynamic 10 MHz ultrasound reveals the “twinkling” of blood as it flows within a choroidal melanoma.
Note
This is a large file which may take up to 5 minutes to download (at 56K). If you are using dial-up Internet access, you will see a static image for 5 minutes.
Note the “twinkling” within the tumor.
This is caused by blood circulating within the melanoma. You can use this observation to differentiate between benign and malignant tumors. In general, malignant tumors have an active circulation as compared to “pseudo-melanomas” like a choroidal hemorrhage. The “twinkling” represents intrinsic vascularity or blood flow. Blood flow and vascularity will be found to diminish after radiation therapy.
Note the pigmented tumor presenting at the pupillary margin (arrow).
A 64-year-old female was referred to The New York Eye Cancer Center with a small iris tumor visible at the pupillary margin
Impression
Iris Pigment Epithelial Cyst
Iris pigment epithelial cysts are commonly located at the iridociliary junction and are round or oval. When observed by slit-lamp examination, they have a smooth brown surface. Visualization of the tumor can be improved by dilation of the pupil.
The iris pigment epithelial cyst seen after dilation of the pupil.
Iris pigment epithelial cysts have thin walls and sonolucent contents (as imaged by UBM). It is generally accepted that the high reflectivity of the cyst wall is caused by its epithelial cell lining and that its sonolucent core is consistent with a liquid content.
Iridociliary cysts typically displace the iris root anteriorly. This can induce a focal plateau-iris configuration with or without angle-closure. Though single cysts are more common, multiple cysts are found in at least one third of cases. When multiple cysts involve more than 180 degrees of the iris, as it does in 10% of patients, angle-closure glaucoma may develop.
The natural history of iris pigment epithelial cysts is poorly understood. Therefore serial observation is warranted.
Recommendation:
Observation
Laser cystotomy for progressive angle closure, threatening acute glaucoma
References
Differential Diagnosis of Anterior Segment Cysts by Ultrasound Biomicroscopy. Authors: Marigo FA, Esaki K, Finger PT, Greenfield DS, Liebmann JM, Ritch R. Ophthalmology 1999;106:2131-35
Anterior Segment Tumors: Current Concepts and Innovations. Marigo FA, Finger PT. Survey of Ophthalmology 2003;48:569-593.
There was keratinization of the overlying corneal epithelium, corneal neovascularization, and punctate keratopathy (arrow).
A 71-year-old white female presented with a growth which appeared on her left eye 3 months prior to examination. Slit lamp examination revealed a gray, gelatinous, slightly elevated neovascular mass measuring 2.4 x 3.6 mm at the limbus centered in the 8 o’clock meridian.
An exfoliative biopsy was performed and a plug was placed in the lower punctum. Histopathology confirmed the diagnosis of CIN (corneal and conjunctival intraepithelial neoplasia).
The known risks and potential benefits of topical Interferon Alfa-2b (IFNa2b) and traditional forms of treatment (including but not limited to surgical excision and cryotherapy) were extensively discussed. After informed consent was obtained, treatment was given with topical IFNa2b (Schering Plough, Kenilworth, NJ).
Three Month Follow-up Photograph – Dose Given = 1 million units/cc, 1 drop, four times daily. She was followed biweekly and by the 3-month follow-up, the lesion had completely resolved. At 15 months of follow-up, there has been no recurrence.
Treatment of CIN has traditionally involved wide excision of the tumor with application of cryotherapy, topical Mitomycin-C, or radiation.
At The New York Eye Cancer Center, currently almost all patients with squamous conjunctival neoplasia can be treated without surgery, using topical chemotherapy eye drops. Depending on the type of chemotherapy eye drops, there may be local side effects (conjunctival hyperemia, follicular conjunctivitis) which generally resolve within 1 month of therapy.
This patient has done well, with no apparent side effects. It is my impression that topical interferon should the primary treatment for most cases of ocular surface squamous carcinoma and moderate to severe dysplasia.
Impression:
Topical Interferon Chemotherapy for Squamous Conjunctival Intraepithelial Neoplasia
References
Schechter BA, Schrier A, Nagler RS, Smith EF, Velazquez GE. Regression of Primary Conjunctival and Corneal Intraepithelial Neoplasia with Topical Interferon Alfa-2b. Cornea 2002; 21(1):6-11.
Wilson MW, Czechonska G, Finger PT, Rausen A, Hooper ME, Haik BG. Chemotherapy for eye cancer. Survey of Ophthalmology 45(5):416-444, 2001.
A RetCam digital image reveals an amelanotic subretinal tumor with intrinsic vascularity. Dependent exudative, bullous, nonrhegmatogenous retinal detachments are present in the inferior quadrants (Patient in supine position for RetCam). Image by Julian Garcia, MD
This 45 year old HIV positive patient was noted decreased vision in his left eye for 3 weeks duration. He had a past medical history of renal cell carcinoma with lung metastasis.
Impression
Choroidal Metastasis: of Renal Cell Carcinoma Origin
Treatment
This risks and benefits of chemotherapy and external beam radiation therapy were discussed in detail. Though he was to receive systemic chemotherapy, external beam irradiation was initiated due to the severity of his disease.
Comment
A RetCam digital image reveals an amelanotic subretinal tumor with intrinsic vascularity. Dependent Three-dimensional Ultrasound (3DUS) Image – This 3D reconstruction clearly demonstrates the variably reflective choroidal tumor as well as the secondary retinal detachment in the inferior quadrant.
This case presents multiple findings consistent with metastatic choroidal tumors: uveal mass and secondary retinal detachment. Multifocality and bilaterality can also be noted.
The presence of metastatic tumor in his lungs was a risk factor for uveal extension. All patients with intraocular metastasis should undergo radiographic imaging of their head, chest, and abdomen or total body PET/CT.
This case is unusual in that the most common primary cancers are breast in women and lung in men. Choroidal metastasis of renal cell origin are relatively rare.
In this case, our patient presented with a known primary cancer. Eighteen percent of patients will present with no known primary, most will have their primary discovered by subsequent systemic evaluations, and a few will have to undergo ocular fine-needle aspiration biopsy to help determine its source
Leukocoria with tumor visible through the right pupil. Note the clear cornea and lack of orbital signs.
This 16-month old patient was referred for bilateral leukocoria (white pupil), acute glaucoma OS, with evidence of anterior chamber seeding and possible optic nerve/orbital invasion. The left pupil was nonreactive.
Impression
Retinoblastoma
Note
This risks and benefits of observation, enucleation, chemotherapy and external beam radiation therapy were discussed in detail. Single agent cisplatinum chemotherapy (3 cycles due to definite choroidal and possible orbital and optic nerve involvement) and enucleation of the left eye was performed. Histopathologic evaluation of the left eye revealed retinoblastoma without optic nerve or orbital extension. The right eye was enucleated after failure of systemic chemoreduction to produce a treatable tumor.
Comment
This case presents multiple classic findings of retinoblastoma: leukocoria (white pupil), secondary glaucoma, orbital inflammation, anterior segment seeding, strabismus, and intraocular calcification. Other findings of retinoblastoma (not found in this case) can include pinealoma, and extraocular tumor extension. In North America today, when the tumor is confined to the eye more than 95% of children survive their retinoblastoma.
"Very well treated by Dr. Finger. He explained everything I needed to know about my issue with detail and attention, putting me at ease and giving me confidence to handle this problem for the rest of my life.”
– N.N.
