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Report of a presentation of bilateral choroidal osteoma without neovascularization in a child following a school screening. Diagnosis was based on funduscopy, optical coherence tomography and B-scan ultrasonography. The child was followed regularly with cycloplegic refraction, funduscopy, macular SD-OCT and EDI-SD-OCT. Fluorescein angiography and OCT-angiography will be performed if necessary., Purpose: To report a peculiar presentation of bilateral choroidal osteoma in a very young child., Methods: Case report., Results: During a school screening, a 4-year-old girl was detected with anisometropia as the first sign of a bilateral choroidal osteoma. The diagnosis was based on funduscopy, optical coherence tomography and B-scan ultrasonography., Conclusion: Choroidal osteomas are rare benign tumors that can lead to severe visual loss due to progression or complications. They are found mostly in young adults, but in our case, the refractive screening allowed an early diagnosis despite the lack of visual symptoms., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

We describe a rare case of bilateral circumscribed choroidal haemangioma in an otherwise healthy male, which mimicked chronic central serous chorioretinopathy (CSCR). A 52-year-old Asian man presented with a one-year history of visual decline in his left eye. The vision in the right eye had been reduced for 15 years. Visual acuity was 6/60 in the right eye and 6/18 in the left eye. Fundus examination of the right eye revealed an area of discoloration with overlying retinal pigment epithelial changes in the macula and evidence of prior surrounding argon laser photocoagulation. The left macula showed a raised choroidal lesion with overlying retinal pigment epithelial changes and associated subretinal fluid. This appearance illustrates how chronic retinal pigment epithelial alterations associated with longstanding subretinal fluid exudation from circumscribed choroidal haemangiomas may mimick the appearance of chronic central serous chorioretinopathy. B-scan ultrasonography, fluorescein angiography, indocyanine green angiography and optical coherence tomography helped to establish the diagnosis. The active lesion in the left eye was treated with verteporfin photodynamic therapy with improvement in vision., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: Uveal melanoma is the most common adult primary intraoculary tumor. Bilateral tumor is very rare, but must not be underestimated because early diagnosis and care improve the survival and the visual prognosis., Observation: We report the case of a patient treated for a left parapapillary choroidal melanoma by disk of ruthenium ((106)Ru), and a follow-up every 3 months for an atypical choroidal large-sized controlateral nævus levelling the macular area. This right nævus changed in 2009 leading to suspicion of a growing melanoma. Because the visual acuity of the left eye was limited to 20/2000, we wanted to confirm this growth before beginning a treatment. But for 3 years the tumor has remained stable and asymptomatic, with a disease staging still negative. To this day, no curative treatment has been started on the right eye., Discussion: In spite of clinical (To Find Small Ocular Melanomas (TFSOM) for example) and paraclinical arguments, it is sometimes difficult to differentiate a large nævus from an early melanoma. Evolution of the tumor often allows to confirm the diagnosis. However, the sole growth of a nævus is not specific of a choroidal melanoma. When confronted with suspicion of a small choroidal melanoma, some authors recommend an immediate treatment for the tumor; others prefer to confirm without a doubt a tumoral growth. It is necessary to weigh out the iatrogenic risks of a conservative treatment on the visual acuity in a monophtalm patient on one side, and the vital risk for the patient on the other side, which seems to be limited in case of small-sized melanoma with a slow evolution., Conclusion: A bilateral choroidal melanoma is rare. Systematic and repeated examinations of both eyes are essential. Management of these complicated cases often leads to discussion., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

The eye is an uncommon subject of cytopathological examination. However, cytopathologic examination may be required for definitive diagnosis in some cases, as malignant tumors of the eye may sometimes be difficult to distinguish clinically from benign disorders. We report a case of malignant melanoma (MM) of the choroid, in which vitrectomy was performed for the initial clinical diagnosis of vitreous hemorrhage. As the dense vitreous hemorrhage was gradually cleared during the vitrectomy, a choroidal mass was discovered and the vitreous fluid was procured for rapid cytologic diagnosis. We used a modified Shorr's stain that can be completed within several minutes. With this method, highly atypical, pleomorphic cancer cells, occasionally associated with melanin pigment granules, were demonstrated. These cytologic findings indicated a diagnosis of MM arising from the choroid. Histologic examination of the enucleated eye confirmed MM of epithelioid type. The advantage and indication of the rapid cytologic diagnosis is discussed., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Introduction: Choroidal metastases are, with melanoma, the most frequent malignant eye neoplasms. Breast carcinoma for women and lung carcinoma for men most commonly metastasize to the eye. We report a rare case of choroidal metastasis from follicular thyroid carcinoma., Observation: A 75-year-old man, with metastatic follicular thyroid carcinoma, presented a decline in visual acuity of the left eye lasting one month. The best corrected visual acuity was 3/10 in his left eye. Biomicroscopic examination of the anterior segment did not reveal anything abnormal. Funduscopic examination showed an amelanotic choroidal mass located at the posterior pole with serous retinal detachment. Tomoscintigraphy with computed tomography confirmed the thyroid origin by uptake of radioiodine in the choroidal mass. External radiotherapy was then proposed., Discussion: Although it is exceptional, diagnosis of choroidal metastases should be considered in any decline in visual acuity in patients with thyroid cancer. The treatment is difficult because metastases from thyroid cancer are very poorly sensitive to radiotherapy and chemotherapy., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

We describe the clinical, angiographic, and echographic aspects of benign choroidal nevi and their differential diagnosis represented mostly by congenital hypertrophy of the pigment epithelium, melanocytoma, and mostly suspicious nevi. Suspicious nevi are defined by the presence of visual symptoms, the presence of orange pigment on the surface, the presence of subretinal fluid sometimes more visible on OCT, the presence of pin-points on angiograms, a thickness of more than 2mm, or a diameter of more than 7 mm. The proximity of the optic disk has also been shown to be a risk factor in several studies. A review of the literature showed that these factors that make the nevus suspicious are associated with a risk of malignant transformation and metastasis, which is correlated to the number of risk factors present at diagnosis. This is why it is important to recommend close follow-up of these lesions with a check-up every 3 months (and radiotherapeutic treatment in case of growth) or early treatment if there are several risk factors and if the lesion is away from the posterior pole., (Copyright (c) 2009 Elsevier Masson SAS. All rights reserved.)

Introduction: Diagnosis of choroidal hematoma, either spontaneous or associated with age-related macular degeneration, is clinical. In some cases of expansive or posterior lesion, hematoma may be misdiagnosed as a tumor. MRI and color Doppler imaging (CDI) are important in ruling out uveal melanoma in these cases., Patients and Methods: We reviewed the clinical, MRI, and ultrasonographic characteristics of 95 patients sent to the Curie Institute for suspected uveal melanoma between 1998 and 2006, whose final diagnosis was a choroidal hematoma. Imaging differences with melanomas are discussed., Results: A total of 95 patients with a diagnosis of hematoma were seen; the age varied from 54 to 92 years with a median age of 77 years; there was a history of macular degeneration in 27 cases and 11 patients were taking an anticoagulant. Intravitreous hemorrhage was noted in 18 cases and the lesion was located in the posterior pole in 28 cases. The thickness of the lesions measured by B scan ultrasonography varied between 1 and 7.8mm, with a mean thickness of 2.86mm. MRI was performed in 27 cases and CDI in ten cases. On CDI, hematomas appeared as linear or regular bulging lesions with no intralesional blood flow. On MRI, hematomas appeared as a high-intensity signal on T1-weighted images, heterogeneous on T2-weigted images in relation to the progression of the clot, but no contrast enhancement was noted inside the lesion. Follow-up examinations showed the progressive involvement of the clot and delayed decreasing size of the lesion., Conclusion: The diagnosis of choroidal hematoma is usually made by fundus examination. In some posterior locations with pseudo-tumoral appearance, CDI and MRI are useful to rule out a uveal tumor.

Introduction: Choroidal metastases are often the revealing feature of malignant diseases. We report a rare case of prostatic adenocarcinoma metastases at the choroids, diagnosed and followed by fluorescein angiography (FA), indocyanine-green angiography (ICGA), and optical coherence tomography (OCT-3 Stratus)., Observation: A 54-year-old man was referred to our department for decreased vision in his left eye lasting for 1 month. On ophthalmic evaluation, best corrected visual acuity (BCVA) was 20/20 in his right eye and 20/63 in his left eye. Biomicroscopic examination of the anterior segments did not reveal anything abnormal in either eye. Funduscopic examination revealed two amelanotic choroidal masses in both his right and left eyes, located at the posterior pole and at the periphery. The systemic workup, including hematologic analysis and total-body computed tomography (CT), revealed elevated serum prostate-specific antigen (PSA) and alkaline phosphatase, extensive abnormalities of the axial skeleton, and nodular pulmonary shadows; therefore, prostatic adenocarcinoma was suspected. Needle biopsies (prostatic and pulmonary) confirmed adenocarcinoma of the prostate, with metastatic disease. We decided to submit the patient to intermittent total androgen blockade alone, without adjunctive radiotherapy. The patient responded well to intermittent total androgen blockade with oral bicalutamide and triptorelin injection alone, as documented on fundus pictures, ultrasonography, OCT, FA, and ICGA., Discussion: Prostatic carcinoma should be considered in any male patient with a choroidal mass suspected of being a metastasis. In our patient, FA, ICGA, and OCT clearly documented the complete regression of choroidal metastasis from prostatic carcinoma. Fluorescein angiography, indocyanine-green angiography, and optical coherence tomography are useful tools in the diagnosis and follow-up of prostatic adenocarcinoma metastatic to the choroid.

Introduction: Ring melanoma is a rare form of uveal melanoma characterized by the circumferential involvement of the ciliary body. Unilateral chronic and refractory glaucoma is a classic circumstance of diagnosis., Observation: We report a case of ring melanoma revealed by acute intraocular hypertonia secondary to spontaneous hyphema. Iris and iridocorneal angle were diffusely invaded by the tumor. The fundus was masked but B-scan ultrasonography revealed a voluminous ciliochoroidal tumor. The patient had been enucleated. Pathological examination confirmed the diagnosis of ring melanoma. The tumor involved angle structures. The patient died 1 year later because of metastasis., Discussion: Acute or chronic ocular hypertonia is a classic but rare circumstance of uveal melanoma diagnosis. Many mechanisms exist: neovascular glaucoma, secondary angle closure, involvement of angle structures, and trabecular obstruction by tumor cells or pigment. Acute intraocular hypertonia secondary to hyphema is more exceptional. Our observation highlights that apart from the classic situation of acute angle closure glaucoma, intraocular hypertonia requires meticulous fundus examination, if necessary using B-scan ultrasonography.

Uveal melanoma is very rare in children, and in both adults and children it can in rare cases develop intralesional cavities resembling an intraocular cyst. The presence of a solid mass at the base and a thick wall surrounding the cavity can assist in differentiating cavitary melanoma from a benign cyst. We report the case of a 5-year-old girl who presented with a large intraocular pigmented mass in the left eye, showing multiple hollow cavities on ocular ultrasonography, CT scan, and MRI. The patient was treated by enucleation and the pathology confirmed the diagnosis of choroidal melanoma. FISH revealed no aberration in chromosome 3.

We report the case of a 31-year-old man who developed a combined hamartoma of the retina and retinal pigment epithelium in the left eye. The diagnosis was determined based on alterations discovered on fundus examination: hyperplasia of the retinal pigment epithelium cells and tortuosity of the vessels and glial epiretinal membrane. These modifications made it possible to differentiate the hamartoma from choroidal melanoma. The patient underwent photocoagulation therapy. His death due to stroke 4 years after therapy made it possible to analyze the eyeball. Histopathological examination revealed alterations due to retinal photocoagulation as well as alterations particular to the primary tumor: hypertrophy of the retinal pigment epithelium and glial and vascular overgrowth.

Purpose: Iodobenzamides are reported to possess an affinity for melanoma. A first selected compound, BZA, was studied in a phase 2 clinical trial on 159 patients as an imaging agent for the detection of primary melanoma and metastases with good results. We report the results of a second phase 2 clinical trial on 40 patients with a new radiopharmaceutical BZA2 (an orthoiodinated BZA analog), which was expected to provide quality images sooner after injection and with better imaging contrast., Patients and Methods: Performance was evaluated in 40 patients classified with primary ocular lesions (12), suspicion of metastases of ocular or cutaneous origin (15), or with no known secondary lesion (13), and results were compared with conventional investigation techniques (ophthalmoscopy, ultrasonography, and angiography for ocular melanoma, whole-body CT scan and ultrasonography for metastases)., Results: No adverse events were recorded. The overall results on a per patient basis showed a sensitivity of 78% and a specificity of 95%. The four false negatives observed were ocular lesions (three with a thickness<3mm and one achromic), but all the proven secondary lesions were imaged. Moreover, negative BZA2 scintigraphy in cases of suspicious lesions led to the correction of two diagnoses: the prostatic origin of bone metastases and the endocrine tumor origin (APUD system) of an ocular lesion., Discussion: BZA2 scintigraphy is an easy test with good tolerance. In the diagnosis of ocular primary melanoma, the sensitivity of the test is 64%, although limited by the thickness (3mm) and the pigmentation of the lesion. However, the BZA2 scintigraphy is a very useful test for the detection of melanoma metastases, with a sensitivity of 100% and a specificity of 95%., Conclusion: BZA2 scintigraphy showed good tolerance in patients and it appears promising for differential diagnosis, staging, and restaging of melanoma.

Purpose: To report and analyze the circumstances of uveal melanoma detection., Methods: The records of 143 consecutive patients diagnosed in the Ophthalmology Department of Gustave Roussy Institute between September 1994 and September 2001 were analyzed. The study included 66 females and 77 males, aged from 21 to 91 years (mean, 62.75 years)., Results: The first symptom was decreased visual acuity in 37% of cases. In 34.9%, there was no functional sign and a systematic fundus exam provided the diagnosis. Of the 143 patients, 18.8% presented alteration of the visual field or scotoma, 9.9% complained of phosphenes, 9% complained of metamorphopsia, and 6.5% complained of floaters. In 5.5% of cases, there was documented tumor growth. In 2%, the presence of extrascleral exteriorization was the first sign. At the time of diagnosis, anterior tumors tended to be significantly larger than posterior tumors (p<0.007). Smaller lesions were significantly associated with a systematic detection of the tumor (p<0.005). Liver metastasis occurred more frequently with ciliary body melanomas (p<0.001), which were more frequently the largest lesions., Conclusion: These results emphasize the importance of early detection of uveal melanoma. We recommended frequent fundus examination after pupil dilatation.

To the best of our knowledge this is the first description of a choroidal melanoma with documented growth in neurofibromatosis type 2 (NF2). A 20-year-old patient with NF2 presenting deafness due to bilateral acoustic neurinomas and unilateral amaurosis due to a meningioma of the optic nerve developed a pigmented parapapillary choroidal tumor. Despite signs indicating the diagnosis of a melanoma, periodic observation was chosen in order to postpone functional amputation following optic nerve irradiation. The tumor growth was slow during the 5 years that followed, and once progression became rapid, the tumor was treated by accelerated proton beam radiotherapy. One year later, visual acuity diminished due to actinic optic neuropathy and was stabilized at 0.3 for the 2 following years. The tumor presented objective signs of regression, and no sign of metastatic disease was observed. The therapeutic approach in this case provided local control of the tumor while preserving useful visual function.

Purpose: To evaluate the results of proton beam irradiation of choroidal melanomas on a large series of patients., Patients and Methods: Retrospective analysis of a series of patients treated with proton beam irradiation between 1991 and December 1998. The data were analyzed to evaluate the local tumor control as well as the general progression and metastatic rate of the patients. Statistical analysis served to isolate risk factors for relapse or metastasis., Results: We treated 1062 patients during the study period, with a median follow-up of 38 months. Local control was obtained for 97.1% of the patients. Tumors anterior to the equator were at risk for relapse. The survival rate was 92% at 2 years and 78% at 5 years. 73.1% of the 1062 patients died from metastasis, 6.1% of living patients presented with metastatic disease. The risk factors for death were the initial diameter, the age of the patient, and large tumor volume at diagnosis. Metastasis were essentially hepatic (94.6%). Risk factors for metastasis were: a large tumor volume, a lesion anterior or straddling the equator and the age of the patient. Ocular complications may induce a visual loss of 0.1 and less in 47% of the patients, due to optic nerve head and macular ischemia. 6% of the patients required secondary enucleation due to local complications (neovascular glaucoma)., Conclusion: Proton beam irradiation of choroidal melanoma allows good tumor control and eye retention. The survival prognosis is associated with the initial volume of the tumor. The functional results may be improved and new therapeutics are needed to treat metastatic disease.

The management of posterior uveal melanoma has evolved tremendously over the past decades and more recently there has been a trend toward more focal conservative treatment. Transpupillary thermotherapy (TTT) with infrared diode laser (810nm) is the newest modality used as primary treatment or as a complement to radiotherapy or surgical resection in selected cases of choroidal melanoma. Plaque radiotherapy or charged-particle irradiation is particularly recommended for medium- or small-sized uveal melanoma not suitable to TTT or resection. Special custom-designed plaque radiotherapy (iodine-125) can be used for the iris and ciliary body, or in juxtapaillary choroidal melanoma. The tumor control rate after plaque or charged-particle radiotherapy appears to be similar but charged-particle irradiation may produce worse anterior-segment complications than plaque radiotherapy. Stereotactic radiation therapy for choroidal melanoma may be effective in controlling tumor growth, but the number of patients treated with this approach is too small to draw solid conclusions. Local tumor resection using trans-scleral resection is mainly suitable for selected iris, ciliary body, or anterior choroidal melanoma, particularly with smaller basal dimensions and greater thickness. Endoresection may preserve central vision or temporal field when radiotherapy would be expected to cause optic neuropathy. Longer follow-up is necessary to establish the efficacy of tumor control. Combined therapies (radiotherapy plus TTT or tumor resection plus TTT) appear to be more effective in decreasing the incidence of intraocular tumor recurrence. Enucleation is still performed for large uveal melanoma when there is no hope of regaining useful vision. Based on the published ophthalmology literature, it seems that enucleation carries the same survival prognosis as each of the conservative treatment modalities.

Indocyanine green angiography (ICG) is a well-known ancillary test for evaluation of choroidal tumors. The authors review the different images seen in choroidal nevus, melanoma, metastasis, hemangioma, osteoma, and vortex vein varix. ICG appears to be useful for the diagnosis of non pigmented choroidal tumors. In addition, ICG provides good information on the limits of pigmented tumors, particularly for the follow-up of a growing tumor and the choice of treatment.

Cancer may affect the eye and orbit as a direct result of metastatic neoplastic infiltration, compression, or circulating antibodies involving paraneoplastic retinal degeneration. A metastatic tumor to the uvea is the most common form of an intraocular metastatic process. The choroid is the most common site for uveal metastasis; metastases to the ciliary body, iris, retina, optic disk, and vitreous are rare. Approximately one-third of patients have no history of primary cancer at the time of ocular diagnosis. Breast and lung carcinomas for women and lung and gastrointestinal carcinomas for men most commonly metastasize to the eye and orbit. The short-term prognosis for vision is usually good after an individualized therapeutic approach (chemotherapy, hormonal therapy, external beam radiotherapy, or plaque radiotherapy), but the systemic prognosis is poor. The visual paraneoplastic syndromes encompass several distinct clinical and pathological entities including carcinoma-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and bilateral diffuse melanocytic uveal proliferation (BDUMP). The CAR syndrome affects photoreceptors, MAR is thought to affect bipolar cell function, and BDUMP targets the uveal tract. Identification of circulating antibodies against retinal proteins (recovering, 23-kDa retinal protein; 46-kDa and 60-kDa retinal proteins) serves to recognize the paraneoplastic nature of the patient's symptoms, which frequently develop before the cancer is diagnosed. Anecdotal therapeutic responses are described after systemic steroids, immunoglobulin injection, and plasmapheresis. Recognition of their visual symptoms and ocular findings should alert the ophthalmologist to the possibility of cancer and systemic evaluation should be pursued.

Uveal effusion exhibits various clinical presentations, some of which may lead to erroneous diagnosis and mimic a choroidal tumor. A 73-year-old female patient displayed a choroidal lesion with a tumoral appearance spontaneously affecting her right eye. Ultrasonography did not establish the diagnosis, leaving doubt for a tumor. In addition to the tumor - like lesion, a moderate inflammation was found in her right eye. The clinical course was favorable and one month after the initial examination, no fundus abnormalities were observed. The delay imposed by the MRI examination contributed to the lack of any active treatment. Apart from cases occurring postoperatively, the diagnosis of uveal effusion may sometimes be difficult to establish. In order to avoid enucleation and even if ultrasonography is helpful in reaching the correct diagnosis, it seems appropriate to wait and regularly follow up the patients when the diagnosis is not certain. The clinical course might be helpful to establish the diagnosis. Our case suggests that in pseudotumoral forms of uveal effusion, we should repeat clinical examination and ultrasonography in order to manage these patients appropriately.