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Biomedical subjects

D M Albert

Publications and source records attributed to D M Albert.

At least 253 records · Page 14Linked to original sources

Metastatic carcinoma of the iris simulating iridocyclitis.

A 46-year-old woman with a history of breast carcinoma and no known metastatic disease presented with iridocyclitis and secondary glaucoma. Intraocular inflammation and pressure elevation persisted despite standard medical therapy, and paracentesis was performed. Cytological examination of the aspirate revealed adenocarcinoma. Subsequent studies disclosed no evidence of extraocular metastasis. Two courses of radiation therapy to the involved eye resulted in a dramatic reduction in intraocular inflammation and allowed temporary control of the intraocular pressure. Ultimately, however, progressive glaucoma necessitated enucleation. This case confirms previous statements that iridocyclitis may be the initial clinical manifestation of metastatic malignancy. In addition, this report emphasises the importance of paracentesis in the diagnostic evaluation of selected cases of anterior uveitis of unknown aetiology.

Adenocarcinoma↗

Uveal melanoma: case report of extension through the optic nerve to the surgical margin in the orbital apex.

An unsuspected uveal melanoma arising in a phthisical eye extended through the optic nerve to the surgical margin in the orbital apex without involvement of other orbital structures. Step sections showed the continuous extension through the nerve. Factors likely to predispose to optic nerve extension include epithelioid cell type, large tumour size, peripapillary involvement, and glaucoma. Optic nerve extension carries the risks noted with orbital extension of intraocular tumour, but may in addition lead more directly to intracranial invasion, including a chiasmal syndrome. The role of exenteration and radiotherapy in these cases remains uncertain.

Humans↗

The Alström syndrome: ophthalmic histopathology and retinal ultrastructure.

A case of pigmentary retinal degeneration causing blindness in early childhood, progressive neurosensory hearing loss, diabetes mellitus, acanthosis nigricans, hypogonadism with normal secondary sex characteristics, and kyphoscoliosis without polydactyly and with no mental retardation is reported. The results of endocrinological studies, karyotype analysis, and digital dermatoglyphics supported the clinical diagnosis of the Alström syndrome. The patient had small globes, bilateral posterior subcapsular cataracts, lacy vacuolation of the iris, ciliary process hyalinisation, unilateral asteroid hyalosis, total absence of rods and cones, intraretinal melanin pigment, retinal pigment epithelium atrophy, focal chorioretinal fusion, preretinal fibrosis, bilateral giant optic disc drusen, and optic nerve atrophy. Electron microscopy of the retina demonstrated large numbers of melanolysosomes, numerous folds of basement membrane material, disruption of Bruch's membrane, and numerous bundles of extracellular collagen fibrils in the retinal pigment epithelium.

Adult↗

Tumours of the Moll's glands.

Four cases of tumours arising from Moll's glands, two benign mixed tumours of the skin (chondroid syringomas) and two adenocarcinomas, are reported. Three of the patients were over 40 years old. Contrary to the common assumption that malignant Moll's gland tumours are of low-grade malignancy, the two adenocarcinomas described here were highly malignant. Despite vigorous treatment, including orbital exenteration and lymph node resection, one was rapidly fatal and the other recurred with extensive involvement of the paranasal sinuses.

Adenocarcinoma↗

Establishment of cell lines of uveal melanoma. Methodology and characteristics.

Six continuous cell lines have been established from choroidal and ciliary body melanomas. These lines have been maintained in culture for at least 100 in vitro population doublings for periods over 1 year. They were established initially using a human diploid fibroblast strain MRC-5 as a feeder layer. Cells were grown in Ham's F-12 medium containing fetal bovine and horse sera and supplemented with glucose, cholera toxin, and epidermal growth factor. Culture doubling times ranged from 72-96 hr; cloning efficiencies ranged from 1-5% in the absence of a feeder layer. Six cell lines were studied in detail by electron microscopy, and all were found to have evidence of melanosomes and/or premelanosomes. The morphology of the cells was characteristic of melanomas as defined by the Callender classification, with cell types ranging from spindle A to epithelioid. Karyotypic studies revealed the presence of only human chromosomes with modal numbers ranging from 48-54 in the different lines.

Adult↗

Choroidal melanoma associated with rhegmatogenous retinal detachment.

A case of pigmented choroidal lesion coincident with rhegmatogenous retinal detachment was observed. The patient refused enucleation, and the retinal detachment was successfully treated by diathermy without drainage of subretinal fluid. Clinically the lesion was observed to enlarge, and upon enucleation five years after diathermy the tumor was seen to be a spindle-cell choroidal melanoma. The patient died from metastases of melanoma six years after the enucleation.

Choroid Neoplasms↗

Experimental gypsy moth (Lymantria dispar) ophthalmia nodosa.

Ophthalmia nodosa is the nodular granulomatous inflammatory response of ocular tissue to caterpillar hairs. We experimentally simulated this condition by surgically implanting gypsy moth hairs in the rabbit cornea. We were not, however, able to produce ophthalmia nodosa solely by placing cilia in the rabbit cul-de-sac. The experimentally induced inflammation closely resembled human ophthalmia nodosa in both its clinical and histopathologic features.

Animals↗

Early-onset pauciarticular juvenile rheumatoid arthritis. A histopathologic study.

A young girl with early-onset pauciarticular juvenile rheumatoid arthritis suffered from recurrent severe bilateral iridocyclitis. Despite intensive medical therapy, she gradually lost all useful vision and ultimately required bilateral enucleation at the age of 10 years for control of pain and photophobia. Histopathologic study of the eyes revealed in the iris and ciliary body a dense inflammatory infiltrate composed principally of plasma cells, as well as angle closure, a dense cyclitic membrane, retinal detachment, cystoid macula, and optic atrophy. Immunoperoxidase stains showed little immunoglobulin within or around the plasma cells within the ciliary body, although strong reactions for IgG and kappa- and lambda-light chains were seen in other parts of the eyes.

Arthritis, Juvenile↗

The clinical value of negative temporal artery biopsy specimens.

The clinical course of patients with signs and symptoms suggestive of temporal arteritis but with negative temporal artery biopsy specimens was evaluated. Ninety-one patients undergoing a biopsy formed the basis of this study. Of these, 63 patients had no evidence of arteritis on biopsy, and 28 patients had biopsy specimens showing granulomatous inflammation. False-negative findings from biopsy specimens occurred in 5% of patients who had the disease. Cancer was the final diagnosis in 21% of patients with negative biopsy specimens v 3% of patients with temporal arteritis. Various chronic systemic inflammatory diseases were found in 16% of patients with negative biopsy specimens, while none of the patients with temporal arteritis had additional systemic inflammatory diseases. Patients with proven arteritis were notably older and had higher ESRs than patients without the disease. However, no laboratory test or frequently observed symptom or sign noted on initial examination, considered alone or in combination with other findings, had diagnostic sensitivity or specificity as high as temporal artery biopsy.

Aged↗

Intravitreous silicone injection. Histopathologic findings in a human eye after 12 years.

Histopathologic changes occurred in a human eyeball enucleated 12 years after intravitreous silicone injection for the treatment of complicated retinal detachment with massive preretinal membrane. Silicone "bubbles" (droplets) were found in the chamber angle, iris, ciliary body, retina, and preretinal and subretinal membrane, as well as in the optic nerve. Clinically, glaucoma developed in the eye. A review of the literature indicates that the common late complications of intravitreous silicone injection are cataract, glaucoma, and retinal changes.

Adult↗

Ciliary body and choroidal melanomas treated by proton beam irradiation. Histopathologic study of eyes.

Proton beam irradiation resulted in clinical and/or histopathological regression of large ciliary body and choroidal melanomas in three eyes. Enucleations were performed 6 1/2 weeks, five months, and 11 months after irradiation for angle-closure glaucoma from total retinal detachment, increase in retinal detachment, and neovascular glaucoma, respectively. A direct relationship was found between the length of the interval from irradiation to enucleation and the degree of histologic changes. Vascular changes in the tumors included endothelial cell swelling and decreased lumen size, basement membrane thickening, collapse of sinusoidal vessels, and thrombosis of vessels. Although apparently unaltered tumor cells remained, degenerative changes occurred in some melanoma cells, including lipid vacuoles in cytoplasm, pyknotic nuclei, and balloon cell formation. Patchy areas of necrosis and proteinaceous exudate were present. Pigment-laden macrophages were found near tumor vessels and all had a substantial chronic inflammatory infiltrate. The effect of proton beam irradiation on tumor vessels probably plays an important role in uveal melanoma regression.

Adult↗

Intraocular hemangiopericytoma.

A 40-year-old woman had an intraocular lesion consistent with a choroidal hemangioma. Xenon arc photocoagulation was applied to reduce subretinal fluid, and the lesion remained stationary until the patient's death eight years later. The lesion was composed of spindle-shaped and ovoid cells with numerous inconspicuous capillaries. The tumor cells were surrounded by reticulin-positive fibers. The diagnosis of hemangiopericytoma was confirmed by electron microscopy, which demonstrated proliferating pericytes associated with basal lamina. Although hemangiopericytomas are occasionally found in the orbit, to our knowledge this report is the first of an intraocular hemangiopericytoma. Because the pericyte is an integral component of the choroidal vasculature, the origin of this tumor in the choroid is not unexpected. The diagnosis of hemangiopericytoma should be considered when evaluating vascular choroidal lesions.

Adult↗

Mucoepidermoid carcinomas of the lacrimal sac.

Mucoepidermoid carcinoma is a malignant tumor containing both neoplastic mucin-producing cells and epidermoid cells. To the only reported case arising in the lacrimal sac, we add three additional cases. All three were locally aggressive and required orbital exenterations. No deaths from metastatic disease occurred.

Adult↗

A prognostic factor study of disease-free interval and survival following enucleation for uveal melanoma.

Prognostic indicators of uveal melanoma disease-free interval and survival following enucleation were evaluated for 267 patients. The median follow-up time was 17 years. Analysis involved Kaplan-Meier survival curves based on time to tumor-related deaths and multivariate proportional hazards analysis, which provides an estimate of the "force of mortality." Prognostic indicators considered included demographic, clinical, and histopathological factors. Cell type was classified according to the number of epithelioid cells present per high-power field (HPF) on light microscopy. Various classifications of size of the tumor were compared for prognostic value. The five leading predictors of survival in order of importance were as follows: (1) number of epithelioid cells per HPF, (2) largest dimension of the tumor, (3) location of the anterior margin of the tumor, (4) invasion to the line of transection, and (5) degree of pigmentation. Risk categories for survival were constructed based on the three leading prognostic factors.

Epithelium↗

Low incidence of deletion of the esterase D locus in retinoblastoma patients.

Esterase D was quantitatively measured in the red blood cells from three patients from three separate kindreds who had abnormalities of chromosome 13. The esterase D activity was proportional to the number of copies of the q14 region of chromosome 13 present. These findings confirm published data localizing the esterase D gene to chromosome band 13q14, a region which is important in the etiology of retinoblastoma. Fifty-one additional retinoblastoma patients not known to have any chromosomal defect also underwent esterase D determination. In none of these patients did the esterase D measurement detect a 13q14 deletion. The normal esterase D levels in this series of 51 retinoblastoma patients suggest that deletion of an esterase D locus is infrequent in retinoblastoma patients. It must be noted that patients who are mosaics, with a 13q14 deletion in only a fraction of all somatic cells, could possibly have normal red blood cell esterase D levels. Further study is necessary to determine if esterase D determination of all retinoblastoma patients is a worthwhile clinical tool.

Adolescent↗

Choroidal ganglioneuroma in neurofibromatosis.

A twenty-one year old female with previously unsuspected neurofibromatosis presented for evaluation of a blind painful eye. Histopathologic examination of the enucleated specimen revealed choroidal thickening with ovoid bodies and proliferation of connective tissue with pigment-containing cells and ganglion-like cells. Electron microscopic study of the latter cell population revealed typical morphologic features of ganglion cells, including numerous electron-dense intracellular granules and an abundance of mitochondria and rough endoplasmic reticulum. These pathologic findings were interpreted as consistent with the diagnosis of choroidal ganglioneuroma occurring in the context of ocular neurofibromatosis. The literature concerning this unusual tumor is reviewed and the possible relationship of this lesion to neurofibromatosis and other disorders of neural cresent proliferation are briefly discussed.

Adult↗