1/377. 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 = cell (Clic here for more details about this article) |
2/377. 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 = 0.088045408181502keywords = spindle (Clic here for more details about this article) |
3/377. angiomatosis retinae. An ultrastructural study and lipid analysis.A nonfamilial case of agiomatosis retinae (retinal hemangioblastoma) was studied by electron microscopy. In addition to the three major types of cells previously identified within the tumor (endothelial cells, pericytes, heavily lipidized stromal cells), fibrous astrocytes in different stages of lipidization were also found. The endothelial cells were fenestrated, providing the basis for the extravasated exudate that is characteristic of the tumor. The pericytes were completely surrounded by casement membranes and displayed no significant lipidization; in a cellular plaque of vasular tissue at the base of the lesion, however, some of the multilaminar pericytes showed evidence of early smooth muscle differentiation. The stromal cells contained abundant lipid vacuoles and a few organelles, and exhibited granular degeneration of cytoplasmic filaments between the lipid vacuoles. There was spotty basement membrane formation where the stromal cells abutted on the vascular elements. No interconversion could be demonstrated among the endothelial cells, pericytes, and stromal cells. A source for the stromal cells was discovered in the early lipisization of fibrous astrocytes. Analysis of the extracted lipid from the tumor by means of infrared spectroscopy, lipid chromatography, and x-ray diffraction disclosed that the lipid was mostly cholestrol stearate, a plasma lipid. It is suggested that in the retinal lesions the leaky (fenestrated) capillaries of the tumor allowed the passive imbibition of plasma lipid by the fibrous astrocytes, leading to their gradual transformation into the fully lipidized stromal cells.- - - - - - - - - - ranking = 3.6666666666667keywords = cell (Clic here for more details about this article) |
4/377. 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 = 1.6666666666667keywords = cell (Clic here for more details about this article) |
5/377. 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 = 3.6666666666667keywords = cell (Clic here for more details about this article) |
6/377. 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 = 1.6666666666667keywords = cell (Clic here for more details about this article) |
7/377. 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 = 0.33333333333333keywords = cell (Clic here for more details about this article) |
8/377. Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland.PURPOSE: Mucosa-associated lymphoid tissue lymphoma recently has been defined as a distinct subtype of non-Hodgkin's lymphoma with characteristic clinicopathologic features. A 37-year-old woman with systemic lupus erythematosus and unilateral mucosa-associated lymphoid tissue lymphoma of the lacrimal gland is described. methods: The tumor was totally excised by lateral orbitotomy. Immunohistochemical studies were performed with UCHL-1, CD20 (L26), leukocyte common antigen, cytokeratin (CAM5), and kappa and lambda light chain antibodies. RESULTS: The tumor was composed of centrocyte-like lymphocytes, cells with plasmacytoid features, and lymphoepithelial lesions. Most of the cells expressed the CD20 protein and were positive for immunoglobulin kappa light chain. The patient received no supplemental therapy. No systemic dissemination or local recurrence occurred during a follow-up of 26 months. CONCLUSION: The features of this case support the association between systemic diseases and the subsequent development of mucosa-associated lymphoid tissue lymphoma.- - - - - - - - - - ranking = 0.66666666666667keywords = cell (Clic here for more details about this article) |
9/377. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases.OBJECTIVE: Lymphomas of the eye and its adnexa are frequently of B lineage. This study aims to characterize the clinical and histopathologic features of the rare non-B-cell non-Hodgkin lymphomas (NHL) of these locations. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Seven cases of T- and T/NK-cell lymphomas involving the ocular and ocular adnexal tissues. methods: A morphologic, immunohistochemical, and molecular analysis (polymerase chain reaction) of each of the tumors was undertaken. The lesions were classified according to the Revised European-American Lymphoma (REAL) classification. The clinical and follow-up data were collected. RESULTS: The patients included four women and three men ranging in age from 32 to 88 years (mean, 63 years). The presenting ophthalmic symptoms varied from a small nodule on the upper eyelid and conjunctival swellings to dramatic loss of vision associated with gross protrusion of the globe. Five of the cases presented were secondary manifestations of a systemic lymphoma in ocular tissues; two cases represented primary disease. Three cases were "peripheral T-cell lymphomas (PTCL), unspecified" with positivity for CD3, CD8, and betaF1 and negativity for CD56. Two cases were CD3 , CD30 , and CD56- and were classified as "anaplastic large-cell lymphomas of T-cell type" (T-ALCL). The remaining two cases showed an immunophenotype of CD3 , CD56 , and betaF1- and proved to contain Epstein-Barr virus (EBV) by in situ hybridization, consistent with "T/natural killer (NK)-cell lymphoma of nasal type." Clonal T-cell populations were shown in all three of the PTCLs by Southern blot (n = 1) and polymerase chain reaction (n = 2) for the T-cell receptor gamma and beta genes in one case of ALCL but not in the T/NK-cell lymphomas. Five patients died within 2 years; only two patients (one primary PTCL and one cutaneous T-ALCL) were disease free at 4 and 5 years' follow-up, respectively. CONCLUSION: This study shows that a heterogeneous group of T-cell lymphomas can involve the eye and its adnexal tissue. Most T-cell neoplasms are secondary manifestations of systemic disease and carry a poor prognosis. These findings, in conjunction with published data on ocular B-NHL, also indicate that immunophenotypic differentiation between T- and B-NHL in these locations is of clinical importance.- - - - - - - - - - ranking = 6.3333333333333keywords = cell (Clic here for more details about this article) |
10/377. Fine structure of a radiation-induced osteogenic sarcoma.An osteogenic sarcoma arose in the right orbit of a 7-year-old boy some 5 years after the right orbit had been treated by four courses of radiotherapy (total dose approximately 13,000 rads) for a multicentric retinoblastoma. death occurred 6 months after the orbital tumor was first detected. Study of the orbital tumor by electron microscopy revealed a cell population of varied morphology in which two main types were identified. In one group, the cells were large with radiolucent cytoplasm, which contained long branching segments of rough endoplasmic reticulum. In the second group, the cells were smaller with irregular nuclei and an electron-dense cytoplasm, which contained short segments of dilated rough endoplasmic reticulum and numerous mitochondria. The first group of cells closely resembled osteoblasts, while the second group had some features of osteoclasts or their percursors. The branching processes of the tumor cells were separated by an amorphous ground substance, which contained collagen-like fibrils and hydroxyapatite crystals. Crystal deposition was in some instances in close relation to extracellular membrane-bound vesicles.- - - - - - - - - - ranking = 2keywords = cell (Clic here for more details about this article) |
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