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At least 109 records · Page 6Linked to original sources
PubMed1979

Postinflammatory and malignant protein patterns in aqueous humour.

Normal aqueous humour and the aqueous humour of patients with cataract is virtually protein-free. Patients having retinoblastoma and non-malignant postinflammatory lesions show significantly high quantities of proteins in the aqueous humour. Retinoblastoma is associated with an increase in the globulin content and an albumin/globulin ratio below unity, while non-malignant postinflammatory intraocular conditions show a rise of the albumin fraction with an albumin/globulin ratio above unity. It seems likely that the leakage of protein into the aqueous is different in the two conditions, and a transcellular route is postulated as being the cause in malignant conditions. The estimation of protein patterns in the aqueous humour may be of value in the diagnosis of intraocular malignancy.

Aqueous Humor↗
PubMed2004

Cancer-related diseases of the eye: the role of calcium and calcium-binding proteins.

The eye provides unique opportunities to study complex biochemical pathways and to describe how components of these pathways contribute to the molecular basis of disease. In this article, the role of calcium-binding proteins in cancer-related diseases of the eye is reviewed. First, paraneoplastic syndromes, or so-called remote effects of cancer, arise from damage to tissues distant from any tumor or its metastases. Many of these syndromes are believed to be immune-mediated. Cancer-associated retinopathy (CAR), a blinding disease due to the degeneration of retinal photoreceptor cells, is one of the best characterized of the paraneoplastic syndromes. The CAR autoantigen has been identified as recoverin, a calcium-binding protein of the EF-hand superfamily. Its features as a calcium-binding protein, along with its function in photoreceptor cells and its role as the CAR autoantigen, are discussed. Next, unlike visual symptoms instigated by a distant tumor, ocular melanoma is the primary malignancy originating in the eye. ALG-2 encodes a pro-apoptotic calcium-binding protein that is down-regulated in ocular melanoma, thus providing these tumor cells with a selective advantage. In addition to background discussion of ALG-2, data describing the expression, cellular localization, and dimerization characteristics of ALG-2 in melanoma cells are presented. Biochemical studies of ALG-2 and its interactions with its target Alix/AIP1 also are presented. Finally, the function of ALG-2 in calcium-induced cell death is discussed. Additional calcium-binding proteins in retina and in ocular tumors are described in relation to different disease entities. Such proteins and their expression in the eye provide valuable examples bridging studies of protein chemistry, cellular function, and human disease.

Animals↗
PubMedDate not supplied

Retinoblastoma.

Retinoblastoma was diagnosed in 74 patients between July 1967 and February 1987. Thirty cases (40%) were bilateral; 39 were female and 35 were male. Treatment in bilateral cases consisted of enucleation of the more involved eye and a combined approach of supervoltage irradiation, cryocoagulation, and photocoagulation to the remaining eye. In unilateral cases the involved eye was enucleated and the uninvolved eye observed. Four patients had bilateral enucleation at their initial presentation and four others had the second eye enucleated after unsuccessful tumor treatment. One patient died from metastatic retinoblastoma, one patient died after intrathecal chemotherapy without the evidence of tumor, and one patient died with trilateral retinoblastoma. Seventy-one of 74 patients (96%) currently survive with a follow-up of 1 month to 19 years. Two-year survival is 65 of 68 (95.5%).

Child↗
PubMed1997

Orbital development in long-term survivors of retinoblastoma.

PURPOSE: To determine the effects of enucleation, irradiation, and age at diagnosis on bony orbital growth in long-term survivors of retinoblastoma using measurements based on computed tomographic (CT) imaging. PATIENTS AND METHODS: We used CT obtained at a median age of 13 years to measure orbital volume and configuration in 54 patients who had been treated for retinoblastoma a minimum of 5 years previously. RESULTS: Enucleation and high-dose orbital irradiation (> 35 Gy) both independently adversely affected orbital development (P = .014 and P = .022, respectively). Orbital volume differences for children treated when < or = 1 year old were no greater than those for children treated when older than 1 year of age. In children treated for bilateral retinoblastoma, the impact of enucleation on orbital development was not statistically different from that of irradiation (P = .13). Small implants (12 to 14 mm in diameter) were more commonly associated with smaller orbital volumes. Migration of orbital implants was associated with the smaller orbital sphere size in children < or = 1 year of age (P < .035). CONCLUSION: Treatment for retinoblastoma compromises orbital development. Resulting orbital asymmetry seems to be at least partially related to the size of the implant. Detailed imaging-based measurements of orbital volume and configuration may aid the planning for cosmetic and reconstructive surgery in those who develop orbital asymmetry.

Age Factors↗
PubMedDate not supplied

Intrasellar primitive neuroectodermal tumor (PNET) in familial retinoblastoma: a variant of "trilateral retinoblastoma".

An infant with bilateral familial retinoblastomas was found at age 6 months to have a large mass within the region of the sella turcica. The histology, immunoreactivity and ultrastructure of the tumor showed primitive neuroectodermal cells. A small number of cells showed positive immunostaining to retinal S antigen. This represents a rare form of so-called trilateral retinoblastoma. In most cases of trilateral retinoblastoma, the intracranial non-metastatic tumor is within the pineal gland. The occurrence of such a tumor in the region of sella turcica is very unusual and its possible significance is discussed in regard to our current understanding of the susceptibility to carcinogenesis conferred by inheritance of the mutant retinoblastoma gene, Rb-1.

Antigens↗
PubMedDate not supplied

Multifocal pigment epithelial detachments by reticulum cell sarcoma. A characteristic funduscopic picture.

Patients with systemic reticulum cell sarcoma often develop evidence of ocular involvement months or several years prior to the onset of neurologic symptoms. This neoplasm appears to have proclivity of arising multicentrically within the subpigment epithelial space, as well as in the vitreous. When this occurs it may produce a peculiar ophthalmoscopic picture of multiple large, solid detachments of the pigment epithelium that is rarely duplicated by any other disease. Three patients illustrating this disease are presented.

Aged↗
PubMed1997

Congenital malignant teratoid neoplasm of the eye and orbit: a case report and review of the literature.

BACKGROUND: Medulloepithelioma is a tumor of the primitive medullary epithelium overlying the ciliary body. Most become evident early in life, and they may be malignant, although distant metastases are rare. The purpose of this report is to describe a unique case of congenital malignant teratoid neoplasm of the eye and orbit. METHOD: A patient with a congenital malignant teratoid tumor of the eye and orbit is described, and a detailed histopathologic study of the ocular findings with a review of the literature is presented. RESULTS: Histopathologic study showed that the lesion was a malignant teratoid neoplasm with a large orbital extension. Several intracranial structural abnormalities were identified. CONCLUSION: The tumor described herein must be added to the differential diagnosis of congenital orbital masses. The clinician should be alert to the association of this lesion with complex intracranial abnormalities.

Diagnosis, Differential↗
PubMed1999

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.

Adult↗