1/58. Iridocorneal melanoma associated with type 1 neurofibromatosis: a clinicopathologic study.OBJECTIVE: A clinicopathologic study of an iridocorneal melanoma associated with type 1 (peripheral) neurofibromatosis is presented. DESIGN: Case report with clinicopathologic correlation. PARTICIPANT: A 32-year-old white woman with type 1 neurofibromatosis presented with long-standing blindness of her right eye due to diffuse intrastromal brown corneal discoloration. INTERVENTION: The patient underwent penetrating keratoplasty and the corneal button was inspected. RESULTS: Histopathologic evaluation of the corneal button after penetrating keratoplasty revealed an intrastromal mixed-type malignant melanoma, which stained positively with HMB-45 and S-100 protein and spared the corneal epithelium and limbus. The corneal graft remained transparent, with best-corrected visual acuity of 20/30. Twenty-two months after surgery, the tumor involved the anterior chamber angle and the iris. Three years later, it caused refractory glaucoma necessitating enucleation. The iris tumor did not extend beyond the iris-lens diaphragm and showed the same cytologic features as the corneal stromal tumor. CONCLUSION: To our best knowledge, this is the first report of iridocorneal melanoma associated with peripheral neurofibromatosis. The location of the tumor in the deep corneal stroma, without initial macroscopic involvement of the angle or iris, may suggest that the corneal portion of the tumor may have developed "in situ" rather than as an extension of iris melanoma. The common origin of melanoma cells and schwann cells from the neural crest and the proliferation of the schwann cells in neurofibromatosis provides additional support for this hypothesis.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/58. Malignant melanoma of the choroid in neurofibromatosis.A 60-year-old white woman with generalized neurofibromatosis and multiple melanocytic hamartomas of the iris developed an unusual choroidal mass, with secondary sensory retinal separation in the left eye. Ophthalmoscopically the tumor had a peculiar donut configuration that was caused by a large focus of central necrosis within a spindle B melanoma.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
3/58. Squamous cell carcinoma with necrotizing scleritis.PURPOSE: To report on a case of limbal squamous cell carcinoma (SCC) with necrotizing scleritis in a young, previously healthy, white Australian male. methods: A 31-year-old man presented with a left limbal lesion intermittently causing a red eye and foreign body sensation. He had enjoyed surfing for many years. Repeat hiv tests were negative and the lesion was biopsied. RESULTS: biopsy showed a well-differentiated SCC apparently arising in an intra-epithelial (in situ) carcinoma of the conjunctiva. The lesion was excised and a corneoscleral graft repair was performed. CONCLUSION: The present case highlights the potential for a significant increase in the prevalence of ocular surface neoplasia in healthy young people who have had excessive UV-B exposure.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
4/58. Ophthalmologic manifestations of granulocytic sarcoma (myeloid sarcoma or chloroma). The third Pan American association of ophthalmology and American Journal of ophthalmology Lecture.The clinicopathologic review of 33 well-documented cases of granulocytic sarcoma on file in the Registry of Ophthalmic pathology confirmed the facts that this tumor is encountered mainly in children, that boys are affected more frequently than girls, and that white Americans appear to be less vulnerable than other ethnic groups. While granulocytic sarcoma is a variant of granulocytic leukemia, the tumor may appear before, after, or concomitantly with hematologic evidence of leukemia. In the present series only four of the 33 patients were already known to have leukemia when they were first seen by an ophthalmologist for their orbital, ocular, or adnexal lessions. The Leder stain has proved extremely helpful in arriving at a definitive histopathologic diagnosis. Accurate diagnosis is important to prevent inappropriate medical or surgical treatment and to indicate the need for vigorous antileukemic chemotherapy. prognosis at best is poor.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/58. Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
6/58. Conjunctival squamous cell carcinoma combined with malignant lymphoma.A 31-year-old white woman with a poorly differentiated malignant lymphoma and a compromised immune system atypically contracted squamous cell carcinoma. Data obtained after her death indicate that her immune system was severely altered by either the primary disease or the chemotherapeutic regimen, or both.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/58. A unique case of primary corneal melanoma.PURPOSE: To report a unique case of primary corneal melanoma. methods: A 59-year-old white woman underwent keratectomy to remove a pigmented rapidly enlarging growth on the corner of her left eye. The specimen was submitted for histopathologic examination. RESULTS: Ophthalmic examination disclosed no association with limbal or conjunctival melanocytic abnormalities. Histopathologic studies demonstrated nodular malignant melanoma. CONCLUSION: Possible development of primary corneal melanoma should be considered in diagnosing all cases with a past history of corneal melanin pigmentation.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
8/58. Metastatic carcinoma of the iris.Of three women and one man with metastatic carcinoma of the iris, the average age of the four was 50.2 years. Two of the tumors originated in the breast and two in the lungs. The average survival from time of diagnosis of the iris lesion was less than six months. Each patient presented with characteristic grayish-white translucent nodules on the iris or in the anterior chamber angle. The patients displayed other features of iris metastases such as secondary glaucoma, rubeosis iridis, iris atrophy, anterior uveitis, hypopyon, and hyphema. As an aid in the clinical evaluation, anterior segment fluorescein angiography was performed, revealing vascular details not seen clinically. Leakage of dye was a constant feature of the angiogram.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
9/58. Retina pathology of a failed external beam-radiated group Vb retinoblastoma.We report on a 7-month-old boy who suffered from retinoblastoma with presentation of a white pupillary reflex in his right eye. Initial examination showed a large subretinal and intraretinal mass nasally with extensive vitreous seeding of tumor cells (Group Vb in the Reese-Ellsworth classification). External beam radiation therapy (EBRT) was applied in hopes of preserving the eye, and significant regression with disappearance of vitreous seedings and a prominently decreased tumor mass with localized calcification were documented. Unfortunately total retinal detachment was subsequently identified 3 months after irradiation. The patient ultimately underwent enucleation, and histopathology revealed significant calcification within the residual tumor without marked necrosis; a preretinal fibrous membrane with focal vascular thickening was noted, which implied a partial but incomplete effect of EBRT for this group Vb retinoblastoma. We describe the histopathological findings of the failure of irradiation for a group Vb retinoblastoma, and emphasize the importance of early application of EBRT treatment for a retinoblastoma.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
10/58. A corneal diffuse neurofibroma as a manifestation of von recklinghausen disease.PURPOSE: To report a case of a primary corneal diffuse neurofibroma in a patient with von Recklinghausen disease (NF-1). methods: Case report. A physical examination and histopathology were performed. The immunohistochemical studies were performed using an avidin-biotin-peroxidase complex technique on formalin-fixed and paraffin-embedded tissue. Histologic sections from corneal tissue were incubated with primary antibodies against vimentin and S-100 protein. A complementary ultrastructural study of the same formalin-fixed and paraffin-embedded tissue was made. RESULTS: The ophthalmologic examination revealed a yellowish-white elevated mass that involved the supratemporal cornea but not the limbus. Histologic study showed a tumor of the peripheral nerve sheath, a diffuse neurofibroma in the corneal stroma, and proliferation of spindle cells with markedly elongated nuclei. Cells comprising the tumor reacted with vimentin and S-100 protein, and the ultrastructural studies revealed myelinated nerve fibers confirming the diagnosis. CONCLUSION: The development of a primary diffuse neurofibroma in the cornea of patients with von Recklinghausen disease is possible. The present case supports the statement that neurofibromas arising from the peripheral nerve sheath may involve any part of the body.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
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