1/617. 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 = eye (Clic here for more details about this article) |
2/617. Von Hippel's disease in association with von Recklinghausen's neurofibromatosis.Ten members of a large family who showed manifestations of either von hippel-lindau disease or von Recklinghausen's neurofibromatosis were examined. Three of 10 members were found to have retinal angiomas which had not been present on fundus examination 3 years previously. These angiomas were associated with ocular and systemic signs of neurofibromatosis. These cases show overlapping manifestations of different phakomatoses and provide support for the concept of a common aetiology for these diseases.- - - - - - - - - - ranking = 0.10495518164261keywords = ocular (Clic here for more details about this article) |
3/617. A case of medulloepithelioma of the left eye.The case of a 12 year old male patient with a diagnosis of medulloepithelioma of the ciliary body is reported. The difficulties in reaching diagnosis and management are discussed. The importance of keeping such a rare disease in mind is stressed.- - - - - - - - - - ranking = 4keywords = eye (Clic here for more details about this article) |
4/617. 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 = eye (Clic here for more details about this article) |
5/617. listeria monocytogenes endophthalmitis with a black hypopyon.A 68-year-old woman had a marked decrease in visual acuity, increased intraocular pressure, and acute iridocyclitis. She developed a pigmented hypopyon simulating an occult intraocular melanoma. Two anterior chamber paracenteses showed growth of listeria monocytogenes. The patient received systemic intravenous penicillin, topical fortified gentamicin sulfate drops, and intraocular injections of cephaloridine. On discharge from the hospital after a two-week stay, visual acuity had improved and intraocular pressure had decreased.- - - - - - - - - - ranking = 0.41982072657044keywords = ocular (Clic here for more details about this article) |
6/617. blindness as a consequence of a paraneoplastic syndrome in a woman with clear cell carcinoma of the ovary.BACKGROUND: paraneoplastic syndromes are rare conditions associated with cancer that result in serious disease states at unique sites. In 1982, a report of bilateral diffuse uveal melanocytic proliferation associated with nonocular cancers which resulted in blindness was reported. We present a case of a woman with recurrent ovarian cancer who developed this paraneoplastic syndrome. CASE: A 55-year-old woman had been diagnosed in 1990 with an ocular melanoma of her right eye and in 1994 with clear cell carcinoma of the ovary. With recurrence of ovarian cancer, new eye lesions were identified in both eyes. After enulcleation of her right eye, an ocular melanoma and diffuse bilateral melanocytic proliferation (BDUMP) were found. The sight in her left eye continued to deteriorate as other signs of BDUMP occurred in the eye. Within 1 month of diagnosis, the patient was blind. She subsequently succumbed to progression of ovarian cancer. CONCLUSION: Recurrent ovarian cancer is usually an intraabdominal disease that results in gastrointestinal dysfunction. This case illustrates a rare paraneoplastic syndrome associated with ovarian cancer that mimics metastatic disease to the eye, but has a different pathophysiology.- - - - - - - - - - ranking = 7.3148655449278keywords = eye, ocular (Clic here for more details about this article) |
7/617. Use of the polymerase chain reaction to detect B- and T-cell gene rearrangements in vitreous specimens from patients with intraocular lymphoma.OBJECTIVE: To determine whether the polymerase chain reaction for B- and T-cell gene rearrangements could be applied to vitreous specimens to aid in the diagnosis of intraocular lymphoma. methods: Vitreous washing specimens from 4 patients were received in balanced saline solution and centrifuged, and a portion of the pellet was used to make routine cytospins. The remainder was used to make a crude extract of dna that was amplified for immunoglobulin heavy chain and T-cell receptor gamma gene rearrangements and the 14;18 translocation by polymerase chain reaction. RESULTS: One patient had 2 specimens 2 years apart. In each, there was an identical band corresponding to the minor cluster region breakpoint of the bcl-2 oncogene, indicating the presence of a 14;18 translocation. One patient showed an immunoglobulin heavy chain gene rearrangement indicating a B-cell lymphoma. Two patients showed rearrangements of the T-cell receptor gamma gene, indicating the presence of a T-cell lymphoma. CONCLUSIONS AND CLINICAL RELEVANCE: Vitreous washing specimens can be used successfully to detect B- and T-cell gene rearrangements by polymerase chain reaction. This may be useful to confirm the diagnosis of intraocular large cell lymphoma in cases suggestive of the diagnosis. Prompt handling of the specimens is necessary to prevent degradation of the dna.- - - - - - - - - - ranking = 0.62973108985567keywords = ocular (Clic here for more details about this article) |
8/617. 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 = 1.1049551816426keywords = eye, ocular (Clic here for more details about this article) |
9/617. Benign pleomorphic adenoma arising from the palpebral lobe of the lacrimal gland associated with elevated intraocular pressure.A 46-year-old man complained of a painless mass in the left upper eyelid. At age 51 years, the patient complained of dull pain in the left eye and diplopia. His left intraocular pressure had increased to 33 mm Hg. No proptosis was noted. The nontender mass in the eyelid was palpable. Computed tomography showed a mass anterior to the orbital rim compressing the globe. The lesion was removed en bloc by a transconjunctival approach. Histopathologic examination of the excised mass showed myxomatous stroma and highly cellular epithelial areas. Postoperatively, the left intraocular pressure normalized. To our knowledge, benign pleomorphic adenomas arising from the palpebral lobe of the lacrimal gland associated with elevated intraocular pressure, as demonstrated in our patient, may be rare.- - - - - - - - - - ranking = 3.7346862714983keywords = eye, ocular (Clic here for more details about this article) |
10/617. Presence of bilateral limbal dermoids and choroidal osteomas in a family with inherited limbal dermoids.We report a case of bilateral limbal dermoids and bilateral choroidal osteomas in a 14-year-old girl with no extraocular anomalies. Histopathological examination of a limbal lesion confirmed the clinical diagnosis of dermoid. Computerized tomography and ultrasonography were compatible with a diagnosis of choroidal osteoma. Limbal dermoids were present in the patient's mother, in a brother with down syndrome, and in an aunt with no choroidal osteoma. The present pedigree is compatible with autosomal dominant inheritance of bilateral limbal dermoids. The same gene may be involved in the pathogenesis of ocular choristomas in same patients.- - - - - - - - - - ranking = 0.20991036328522keywords = ocular (Clic here for more details about this article) |
| | Next -> |