Retained intraocular foreign body simulating choroidal melanoma. A report of two cases

Many different lesions can be difficult to distinguish from malignant melanomas of the choroid. With the use of modern diagnostic facilities the misdiagnosis rate has been greatly reduced. In a recent report from the Collaborative Ocular Melanoma Study Group the incidence of misdiagnosis was only 0.48%. The presence of a retained intraocular foreign body presenting as a raised pigmented choroidal mass similar to a uveal melanoma is rare. We report two cases in which retained intraocular foreign bodies presented clinically as raised pigmented intraocular lesions. Retained intraocular foreign bodies should be considered in the differential diagnosis of ocular tumours, especially if there are any atypical features.     

The ocular melanoma story. LIII Edward Jackson Memorial Lecture: Part II

PURPOSE: To trace the evolution and status of our knowledge of choroidal melanoma with regard to the nature, cause, and treatment of this tumor. METHODS: Historical materials beginning with Georg Bartisch's contributions in 1583 through to the Collaborative Ocular Melanoma Study and recent basic research are reviewed. RESULTS: Many individuals have made important contributions to our knowledge about this tumor. Basic information, however, regarding the natural history of the tumor, the most effective treatment, and its cause is lacking. CONCLUSION: The Collaborative Ocular Melanoma Study will provide important information regarding the choice of treatment between enucleation and radiotherapy as well as natural history information, quality of life, and definitive pathology findings. Definitive treatment of choroidal melanoma will depend on knowledge of the genetic defects that cause the tumor. Within the next 25 years, it is predicted that genetic defects will be determined and tumor samples will be obtained using small-needle aspiration and DNA probes located on microchips. In addition, treatment will then be based on drugs designed to inhibit molecules related to the genetic defect in the tumor.     

Choroidal melanoma: a review and case report

BACKGROUND: Choroidal melanoma is the most common primary intraocular malignancy in adults. The ongoing Collaborative Ocular Melanoma Study (COMS) was designed to provide specific answers for the management of medium and large choroidal melanomas. The present consensus among authorities is that small (< 3 mm thick) suspected choroidal melanomas can be conservatively managed with periodic observation for evidence of growth. CASE REPORT: Dilated fundus examination of a 68-year-old man revealed a pigmented uveal lesion straddling the ora serrata inferiorly at 5:30 in the right eye. It measured 5 mm in diameter with transillumination and 2.89 mm in thickness with B-scan ultrasonography. Questioning a small choroidal melanoma the patient was referred to an ocular oncologist, who confirmed the lesion was suspicious for choroidal melanoma. CONCLUSION: Although there are clinical signs that suggest lesion growth, true growth must be recorded with serial photographs, ultrasound, and transillumination. Active therapy should usually begin once lesion growth is documented.     

Clinicopathologic findings in recurrent choroidal melanoma after transpupillary thermotherapy

OBJECTIVE: This study aimed to report the clinicopathologic findings of recurrent choroidal melanoma after transpupillary thermotherapy (TTT). DESIGN: Two case reports. METHODS: The clinical histories and ophthalmologic findings of two patients with recurrent choroidal melanomas who did not respond to TTT were reviewed. Both patients had their eyes with the melanoma enucleated and processed routinely for light and electron microscopic examination. MAIN OUTCOME MEASURES: The eyes were examined for histopathologic and ultrastructural findings. RESULTS: Histopathologic examination showed choroidal melanomas with extensive tumor necrosis and hemorrhage. In areas, tumor cells appeared histologically intact and presumably viable, with ultrastructural evidence of melanogenesis. The eye from one patient was treated only with TTT and showed hemorrhagic necrosis with cytolysis and no changes to tissues surrounding the melanoma. The eye from the other patient, treated with TTT and a radioactive isotope of iodine (I125) plaque, exhibited areas of intact tumor, tumor necrosis, fibrosis, and radiation retinopathy. CONCLUSIONS: These two cases among a total treated series of 14 patients represent examples of uveal melanoma regrowth after TTT. Opaque media precluded adequate treatment in the first case that showed some TTT effect. The second case showed a combination of TTT and I125 effect and failed because of the aggressive nature of tumor.     

Necrotic malignant melanoma of the choroid and concurrent intraocular manifestation of malignant non-Hodgkin's B cell lymphoma

An 80-year-old white female developed clinical signs of a large choroidal malignant melanoma in her left eye. There were no signs of metastatic disease, but an asymptomatic chronic lymphatic leukemia was discovered. Histopathologic examination of the enucleated left eye showed a mostly necrotic malignant melanoma of the choroid with areas of spindle B cell differentiation, episcleral extension and secondary angle-closure glaucoma with necrosis of the anterior segment of the eye. On the basis of immunocytochemical studies of the lymphocytic infiltrates in the iridal blood vessels, retinal blood vessels and the choroid, the leukemic disease was classified as B cell lymphoma of low malignancy (lymphoplasmacytoid immunocytoma). A reactive T lymphocytic infiltration of the conjunctival stroma was also noted. Patients with malignant melanomas of the uvea require exclusion of a second malignancy.     

Intravitreal invasion of malignant cells from choroidal melanoma after brachytherapy

We report intravitreal invasion by melanoma cells from a choroidal melanoma after brachytherapy. A malignant melanoma of the choroid with collar-button configuration was treated with iodine 125 brachytherapy. Years later, the collar button developed a dark-chocolate color and began shedding pigmented debris into the vitreous. Coalescence of this debris into spheroidal aggregates suggested the presence of malignant cells; the eye was enucleated. Histologic sections demonstrated a choroidal melanoma with intraretinal and intravitreal invasion by melanoma. Clinical evidence of intraretinal invasion by melanoma cells along with pigmented debris within the vitreous cavity, especially when clustered in spheroidal aggregates, suggests the presence of intravitreal invasion by malignant cells. In this case, intravitreal invasion was verified histologically.     

Intrachoroidal hemorrhage mistaken for malignant melanoma

An 84-year-old man had a choroidal mass in the temporal aspect of his left globe associated with a nonrhegmatogenous retinal detachment of the macula. Because of suspected malignant melanoma of the choroid the eye was enucleated. On histopathologic examination we found that an intrachoroidal hemorrhage, an extremely unusual type of intraocular hemorrhage, accounted for the mass.     

Pigmented squamous cell carcinoma of the skin: report of two cases and review of the literature

Melanoma is the most common malignant tumor in which melanin synthesis occurs, although other nonmelanocytic tumors synthesize melanin or contain nonneoplastic melanocytes. We present two cases of infiltrating pigmented squamous cell carcinoma of the skin and review the clinical, morphologic, and ultrastructural features. Melanin was found in epithelial tumor cells as well as in macrophages and dendritic melanocytes. Interestingly, one of the neoplasms was associated with an adjacent melanocytic nevus and pigmented solar keratosis. Immunohistochemical analysis showed that neoplastic cells stained for keratin and melanin-filled dendritic cells were found to be S-100 protein and HMB45 positive. A careless examination of the immunohistochemical stains for S-100 protein and HMB45 could cause the misdiagnosis of melanoma, a neoplasm that has a more ominous outlook.     

Nutrition and osteoporosis

PURPOSE: To establish an animal model of extrascleral extension of choroidal melanoma. METHODS: Pigmented choroidal tumors were established in nine New Zealand albino rabbit eyes using B16F10 melanoma cell line. The sclerotomy site was not closed in the subgroup of six rabbits where extrascleral extension was desired. For the control group, the sclerotomy site was sutured with 8-0 nylon. Animals were treated with daily injections of cyclosporine and followed by serial fundus examinations, color Doppler imaging, and fundus photography. All tumor-bearing eyes were enucleated at the end of the follow-up period and examined for extrascleral extension. RESULTS: Extrascleral extension of choroidal melanoma occurred in all six animals with open sclerotomy sites. No extrascleral extension was observed in the control group. Color Doppler imaging identified extrascleral extension which was confirmed on gross histology. CONCLUSIONS: Our animal model of extrascleral extension of choroidal melanoma requires minimal surgery to establish, and is reproducible and easy to follow with standard diagnostic equipment.     

Primary bilateral malignant melanoma of the choroid (author's transl)

Report on an extremely rare case of primary bilateral malignant melanoma of the choroid with histological verification of both tumors. The patient was a 54-year-old woman; the presumptive clinical diagnosis was a primary bilateral melanoblastoma. A thorough general examination did not reveal any metastases. Six months later the patient underwent surgery. The left eye, in which the tumor was larger, was enucleated and a B-type malignant melanoma of the choroid with invasion of the sclera was diagnosed histologically. Two months later the patient died of a lung embolism. Histological verification of the tumor of the right eye led to the same diagnosis as in the left eye, but without invasion of neighbouring structures. No distant metastases were found at autopsy. A thin layer of slender spindle nevus cells was found between the tumor and the sclera during the histological study. The author agrees with Yanoff and Zimmerman (1967) that this may be regarded as the origin of the malignant tumor.     

Pharmacokinetics of hydroxocobalamin in smoke inhalation victims

PURPOSE: This study was performed to describe the clinicopathologic features of a group of patients with posttraumatic unilateral focal choroidal granulomatous inflammation. METHODS: Enucleated eyes with focal choroidal granulomatous inflammation without clinical signs of contralateral ocular inflammation were identified. Clinical and pathologic features were recorded. RESULTS: Six enucleated eyes that had been injured by projectiles had focal uveal granulomatous inflammation of the posterior choroid. Four eyes had uvea attached to or incarcerated into the wound. Two eyes had foreign material associated with the granulomatous inflammation, and two eyes had a disrupted lens with lens-induced inflammation. Two eyes exhibited the focal granulomatous inflammation at the site of a choroidal rupture. None of the six enucleated eyes contained Dalen-Fuchs' nodules. CONCLUSIONS: Focal choroidal granulomatous inflammation may occur as a result of penetrating ocular trauma. The origin of this condition is unknown, although it likely represents a reaction to a foreign body.     

An error in the fluorescence angiographic diagnosis of a subretinal process (author's transl)

A presentation is given of a malignant melanoma of the choroid of a 15-year-old girl. The tumor was erroneously considered as to be a subretinal hemorrhage produced by a choroidal rupture after an accident. A beginning melanosis bulbi resulted in the enucleation and the histological examination of the eye, which confirmed malignant melanoma of the choroid.     

Ocular involvement in Epstein-Barr virus-associated T-cell lymphoma

PURPOSE: To determine if serous retinal detachment may occur in a case of Epstein-Barr virus-associated T-cell lymphoma. METHODS: We examined a 51-year-old man who had recent loss of vision and poor general health. RESULTS: Ocular involvement consisted of bilateral serous retinal detachment and choroidal infiltrates. The diagnosis of lymphoma was made by liver biopsy. The course of the disease was fulminant. Postmortem histologic examination disclosed a massive infiltration of the choroid and hematopoietic organs by pleomorphic large T cells. Epstein-Barr virus was detected by in situ hybridization. CONCLUSION: Ocular involvement, including choroidal infiltrates and serous retinal detachment, may occur in Epstein-Barr virus-associated T-cell lymphoma.     

Mutations in purine nucleoside phosphorylase deficiency

PURPOSE: The aim of this study was to investigate the incidence of unexpected malignant uveal melanoma in the age of ultrasound diagnostics and to highlight the reasons for misdiagnosis. PATIENTS AND METHODS: All eyes were surgically removed and histologic examination was performed between 1981 and 1995. The eyes were investigated for the incidence of uveal melanoma, and the history of the unexpected malignant melanoma of the uvea or ciliary body highlighted. RESULTS: 225 (18.7%) eyes with malignant melanoma out of 2583 enucleated eyes were found. Eight (3.6%) of 225 were clinically unexpected. The clinical misdiagnoses were secondary angle closure or open angle glaucoma (6), retinal detachment (5), iritis (1), scleritis (1), cataract (4) and an intraocular mass that was believed to be a metastasis of a colon carcinoma. Seven of eight eyes were blind, and one eye had light perception only. The longest follow up before enucleation was 13 years. On three eyes diagnostic ultrasound was reportedly performed without specific diagnosis of uveal melanoma. Surgery was performed on four eyes for reasons of uncontrollable intraocular pressure or retinal detachment up to five years before enucleation. Histologic diagnoses were 3 epitheloid-type, 2 spindel-type and 3 necrotic melanoma of the uvea. Four eyes showed scleral invasion by tumor cells and one eye an invasion into the episcleral space. CONCLUSIONS: Even today the rate of unexpected uveal melanoma, according to our study is 3.6%. Therefore, all blind eyes without visualisation of the posterior pole should be examined with ultrasound in order to diagnose an uveal melanoma prior to enucleation.     

Intraocular invasion by papillary squamous cell carcinoma of the conjunctiva

Two patients with incompletely excised invasive papillary squamous cell carcinoma of the corneoscleral limbal conjunctiva had subtle clinical signs suggesting incomplete transmural extension or localized intraocular tumor invasion. We attempted a wide en bloc resection procedure in each case. Histopathologic examination disclosed that the surgical margins were involved with tumor cells and the eyes were subsequently enucleated. The enucleated eyes disclosed more extensive microscopic intraocular involvement than had been anticipated.     

Uveal melanoma, choroidal hemangioma, and simulating lesions. Role of MR imaging

Malignant uveal melanomas are the most common primary intraocular malignant tumors in adults. Choroidal hemangiomas are congenital, benign, vascular lesions that may be confused with malignant uveal melanomas on clinical examination. The MR imaging characteristics of uveal melanomas and choroidal hemangiomas are different, making diagnostic differentiation possible with a high degree of accuracy. This article describes the MR imaging features of uveal melanoma, choroidal hemangioma, and other simulating lesions.     

Immunocytologic findings in a case of Vogt-Koyanagi-Harada syndrome

BACKGROUND: Vogt-Koyanagi-Harada (VKH) syndrome is a form of chronic panuveitis that often has a poor long-term visual outcome. To the authors' knowledge, there have been only two previous reports describing immunocytologic findings in the eyes of patients with VKH syndrome, all studied in relatively late stages of the disease. METHODS: The authors performed immunocytologic studies on the enucleated eyes of a patient with active VKH syndrome, using monoclonal antibodies to mononuclear subsets, major histocompatibility antigens, and viral and treponemal agents, to better understand the pathophysiologic mechanisms underlying the disease. Emulsified chorioretinal tissue also was processed for viral isolation in tissue culture and for Treponema pallidum by rabbit inoculation studies. RESULTS: The dense uveal infiltrates in active VKH syndrome are composed predominantly of T lymphocytes and HLA-DR+ macrophages. Scattered T cells and plasma cells were present in the retina. In addition, nondendritic-appearing CD1 (Leu-6) positive cells were localized in the choroid, in close proximity to choroidal melanocytes. No microbial agents were identified. CONCLUSION: Whereas the etiology of the VKH syndrome remains an enigma, the authors' immunocytologic findings are consistent with a T-cell-mediated disorder. In demonstrating CD1 (Leu-6) positive cells in the infiltrates, the authors propose a potential role for these cells in the etiology of VKH syndrome.     

Clinically unsuspected malignant melanomas of the posterior uvea (author's transl)

Out of 1300 eyes enucleated between 1966-1974, 300 subsequently revealed histologically-proved malignant melanomas of the uvea. In 264 cases the clinical diagnosis was correctly made. In 36 cases the i.o. malignant melanomas were clinically unsuspected. The incorrect clinical diagnoses included: secondary glaucoma (30), retinal detachment (2), iritis (2), and end/panophthalmitis (2). The clinical symptomatology and morphology of the secondary glaucoma caused by the i.o. melanomas did not differ from that due to other, non-neoplastic etiologies. In almost all of the 36 cases the eyes revealed unilateral severe opacity of the media. It is important that one consider an intraocular melanoma in cases of secondary glaucoma, retinal detachment, or intraocular inflammation in which the etiology is uncertain. This is also true in cases of blind, painful eyes in which there is no obvious etiology. Early diagnosis is of vital interest to the patient because this group, in which the malignant melanoma was unsuspected, may be, as suspected in this study, is characterised by extreme aggressivness and invasive capacity. One third of these eyes revealed extension of tumor through the sclera and optic nerve. Also these tumors often revealed more malignant cell types.     

Coexistence of 3 tumors of neural crest origin: neurofibroma, meningioma, and uveal malignant melanoma

OBJECTIVE: To describe the clinical findings in a patient who developed a neurofibroma, meningioma, and choroidal melanoma. METHODS: Clinical and histopathological findings of the case are reviewed and presented. RESULTS: The patient had a right superolateral periorbital neurofibroma, a right sphenoid wing meningioma, and a left choroidal juxtapapillary malignant melanoma. All 3 tumors are derived from neural crest cells. CONCLUSIONS: To our knowledge, this is the first report of a patient with this combination of 3 neural crest-derived tumors. This case is most appropriately classified as a complex neurocristopathy, a disorder involving the aberrant and pathological proliferation of multiple tissues derived from neural crest cells.     

Intraocular neoplastic cyst from mucoepidermoid carcinoma of the conjunctiva

An 89-year-old woman with recurrent conjunctival mucoepidermoid carcinoma developed intraocular inflammation and an elevated fundus lesion that simulated choroidal detachment in her affected right eye. Intraocular invasion of squamous cell carcinoma was suspected and the eye was enucleated. Pathologic examination of the enucleated eye showed intraocular invasion by conjunctival mucoepidermoid carcinoma that formed a suprauveal cyst lined with malignant epithelial cells. The patient developed an orbital recurrence 1 year later and underwent orbital exenteration. She died 2 years later from an unrelated cause. Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and produce an intraocular neoplastic cyst.