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Branch retinal artery occlusion following embolization of a maxillary sinus tumor.

Retinal vascular complications following embolization of carotid artery branches have been occasionally reported. Since the result can be catastrophic and is often irreversible, all efforts to prevent this complication should be considered prior to the intervention. We report the occurrence of a branch retinal artery occlusion following embolization of a maxillary sinus tumor. Cerebral angiography pre- and post-embolization; pathology results from the excised tumor; fundoscopic, visual field, and fluorescein angiographic findings are reported. A combination of risk stratification and preoperative evaluation methods which might help in prevention of this serious complication are discussed.

Adenocarcinoma↗

Retinoblastoma: review of 30 years' experience with external beam radiotherapy.

A review of the experience at the Peter MacCallum Cancer Centre (Peter Mac), Melbourne, Australia in treating retinoblastoma with external beam radiotherapy was conducted. Outcomes of particular interest were tumour control, vision preservation and treatment late effects. The review was restricted to patients that had intact eyes treated at Peter Mac from 1965 until 1997 with at least 2 years of follow up. Histories were reviewed regarding patient and tumour characteristics and treatment details. Thirty-five patients were identified in whom 47 eyes were treated. Of the tumours, 47% were Reese-Ellsworth stage IV or V and the majority of others were at high risk for vision loss because of tumour location. The radiation treatment technique became increasingly sophisticated during the study period. Radiation dose and fraction size have similarly evolved but most patients received 30-50 Gy. Since 1989, a highly accurate contact lens immobilization technique has been used to deliver 40 Gy in 20 fractions. Thirteen eyes required additional local therapy. Of the treated eyes, 34 (72%) remain intact and 74% of these have useful vision. One patient died from retinoblastoma and three from second malignant neoplasms. With modern radiotherapy, late toxicities other than growth arrest and non-progressive cataract did not occur during the study period. Tumour control was high and a very acceptable rate of organ and vision preservation was achieved in a relatively high-risk population. Modern radiotherapy continues to develop in an attempt to improve treatment accuracy and minimize late radiation toxicity.

Child, Preschool↗

Purtscher-like retinopathy associated with pancreatic adenocarcinoma.

PURPOSE: To investigate a case of Purtscher-like retinopathy that occurred in association with pancreatic adenocarcinoma. METHOD: Case report. RESULTS: A 63-year-old woman presented with multiple gray patches in the central vision of both eyes. Visual acuity was 20/20 in both eyes. Funduscopy showed large peripapillary yellow-white patches within the superficial retina and small superficial retinal hemorrhages in both eyes. The patient subsequently had abdominal pain. Computed tomography of the abdomen demonstrated a large pancreatic mass with extension into the liver. Histologic examination of a percutaneous needle biopsy specimen showed mucinous pancreatic adenocarcinoma. CONCLUSION: Pancreatic adenocarcinoma should be added to the list of systemic diseases that can be associated with Purtscher-like retinopathy.

Adenocarcinoma↗

Ocular and orbital echography.

Diagnostic ultrasound is a noninvasive, well tolerated, safe procedure with no toxicity which aids the ophthalmologist by demonstrating the integrity of the intraocular contents or the presence of tumors, retinal detachments, vitreous membranes or hemorrhage, foreign bodies or other structural changes in eyes with opaque media which cannot be adequately evaluated optically. Orbital mass lesions and inflammatory changes can be demonstrated and ultrasonic evaluation should be utilized early in the diagnostic evaluation of exophthalmus in combination with other radiographic techniques. The ultrasound examination does require a certain technical skill and experience in interpretation as well as judgment in order for it to be a reliable diagnostic test.

Cataract↗

Fundus abnormalities in Cushing's disease: a preliminary report.

Three patients, 2 males and 1 female, with intrasellar, hypersecretive pituitary adenoma were studied. Blood hypertension was present in all and hyperglycemia in 2 (1 male, 1 female). None had neurological signs of compression of the optic disk or chiasma. One patient showed bilateral circumpapillary and multiple retinal pigment epithelium (RPE) detachment with pooling defects and intraretinal leakage from small retinal arterioles. Another had loss of foveolar, macular and retinal reflexes in the right eye; negligible RPE changes became visible with fluorescein angiography. The third, who had developed malignant hypertension, manifested bilateral hypertensive neuroretinopathy and papilledema. The simultaneous improvement of general and ocular symptoms after removal of the pituitary tumor makes a causal relationship possible and even very likely, between the underlying disorder and fundus abnormalities.

Adenoma↗

Trilateral retinoblastoma with an RB1 deletion inherited from a carrier mother: a case report.

A presentation of intracranial tumor in bilateral and unilateral retinoblastoma with or without family history is termed as trilateral retinoblastoma (TRB). It always occurs either as a pineal tumor or supra/parasellar tumor, which differ in presentation and prognosis. We report here the first case of TRB with transmission of retinoblastoma gene (RB1) deletion from an unaffected mother (a carrier), presenting as concurrent intracranial neoplasm with bilateral retinoblastoma. The presence of RB1 mutation in both child and mother could be responsible for development of intracranial neoplasm which occurred simultaneously with bilateral RB in our patient. Our patient, who had a suprasellar mass, received radiation and intrathecal chemotherapy, and died 6 months after diagnosis. The occurrence of intracranial tumor in an asymptomatic stage can be avoided by routine computed tomography (CT) and magnetic resonance imaging (MRI) scan, and improved survival can be achieved by aggressive multimodality therapy.

Adult↗

The significance of ultraviolet radiation for eye diseases. A review with comments on the efficacy of UV-blocking contact lenses.

Acute and cumulative ultraviolet radiation (UVR) exposure has been proposed as an important causative factor in the development of a whole spectrum of eye diseases. The present review examines the scientific evidence for and against such an association, with special emphasis on recent additions to the literature. The sun is the main UVR source on earth, and it is beyond scientific doubt that the cornea can be harmed by both acute and cumulative ambient exposures. There is also powerful epidemiological support for an association between chronic UVR exposure and the formation of cataracts and pterygia. The evidence in support of UVR linkage to pinguecula, ocular neoplasms and retinal changes is weaker--in part because there are fewer studies reported in the literature. It is concluded that UVR-blocking hydrogel contact lenses and spectacles are two equally effective preventive measures in minimizing unnecessary suffering and health costs, especially for people who spend a significant time outdoors and for those who live in more UV intense environments. UVR-blocking contact lenses and spectacles must not, however, be substitutes in situations that require UVR-blocking safety goggles.

Contact Lenses↗

Retinoblastoma in Turkey: survival and clinical characteristics 1981-2004.

BACKGROUND: In this study, the authors aim to describe the survival and clinical characteristics of 141 retinoblastoma cases treated at Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, between 1981 and 2004. METHOD: The authors retrospectively analyzed the clinical records of 141 children (177 eyes) diagnosed with retinoblastoma and treated between 1981 and 2004. Information on gender, laterality, age at diagnosis, presenting signs, spread of tumor, treatment modality, survival rate, and family history were collected. RESULTS: A total of 105 cases (74.5%) were unilateral and 36 cases (25.5%) were bilateral. The mean age overall at the time of diagnosis was 25 months; in unilateral cases, 29 months; and in bilateral cases, 16 months. The most common presenting signs were leukocoria (116 cases, 82%), strabismus (14 cases, 10%) and proptosis (11 cases, 8%). A total of 28 cases had orbital extension, nine patients had central nervous system invasion, and five cases exhibited bone marrow involvement. In total, 16 patients (11%) had a family history of retinoblastoma. One case developed a secondary neoplasm. The 3 year cumulative survival rate of 141 patients was 89.69% (unilateral, 90.74%; bilateral 87.35% P = 0.9371, P > 0.05, log rank test). CONCLUSION: The study's survival rate was similar to developed countries. The success in higher survival rates is based on the authors multidisciplinary team approach done by the same group and the support of the authors' clinic and government in sponsoring the medical insurance of all patients.

Child↗

Systemic non-Hodgkin's lymphoma with optic nerve infiltration in a patient with AIDS.

PURPOSE: To report the clinicopathologic features of a patient with AIDS and clinically regressed systemic non-Hodgkin's lymphoma who subsequently developed lymphomatous infiltration of the optic nerve and occlusion of the central retinal vein in both eyes. METHODS: The eyes of this patient were examined ophthalmologically and by fluorescein angiography. The eyes, brain, and body were obtained after death and studied by light microscopy. RESULTS: Ophthalmic examination and fluorescein angiography revealed optic nerve swelling and central retinal vein occlusion first in the left eye and shortly thereafter in the right eye. Postmortem histopathologic examination showed dense infiltration of both optic nerves by lymphoma as well as necrotizing vasculitis involving the retinal vessels near the left optic nerve head. Examination of the brain revealed lymphomatous involvement in the paraventricular region with associated necrosis and inflammation. No bone marrow recurrence or other residual systemic lymphoma was present. CONCLUSION: This case demonstrates that infiltrative optic neuropathy may occur as the sole ocular manifestation of disease recurrence in a patient with systemic non-Hodgkin's lymphoma otherwise thought to be in clinical remission.

Biomarkers, Tumor↗

Ocular complications following blast transformation in chronic myelogenous leukemia.

A number of ocular problems compromising vision occurred in a patient with chronic myeloid leukemia following blastic transformation. Hemorrhagic retinopathy developed with systemic relapse and resolved with control of systemic disease. Optic nerve involvement occurred with meningeal leukemia and was controlled with intrathecal cytosine arabinoside and methotrexate. Leukemic retinal infiltrates developed despite control of systemic and meningeal disease and were successfully treated with radiation therapy. Finally, bilateral vitreous hemorrhages occurred, severely impairing vision. Leukemic infiltration of the eye may occur with increasing frequency in CML as the survival following bastic transformation improves. Infiltration should be recognized and treated promptly if serious loss of vision is to be avoided. Central nervous system prophylaxis should be considered in patients achieving a complete response following therapy for transformation.

Adult↗

Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation.

PURPOSE: To report the results of chemoreduction and focal therapy for retinoblastoma with determination of factors predictive of the need for treatment with external beam radiotherapy or enucleation. DESIGN: Interventional case series. METHODS: One-hundred three patients with retinoblastoma (158 eyes with 364 tumors) at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at Children's Hospital of Philadelphia from June 1994 to August 1999 were enrolled for this prospective clinical trial. The patients received treatment for retinoblastoma with six planned cycles (one cycle per month) of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatments (cryotherapy, thermotherapy, or plaque radiotherapy). The two main outcome measures after chemoreduction and focal therapy were the need for external beam radiotherapy and the need for enucleation. The clinical features at the time of patient presentation were analyzed for impact on the main outcome measures using a series of Cox proportional hazards regressions. RESULTS: Using Reese-Ellsworth (RE) staging for retinoblastoma, there were nine (6%) eyes with group I disease, 26 (16%) eyes with group II disease, 16 (10%) eyes with group III disease, 32 (20%) eyes with group IV disease, and 75 (48%) eyes with group V retinoblastoma. All eyes showed initial favorable response with tumor regression. The median follow-up was 28 months (range, 2-63 months). Failure of chemoreduction and need for treatment with external beam radiotherapy occurred in 25% of eyes at 1 year, 27% at 3 years, and no further increase at 5 years. More specifically, external beam radiotherapy was necessary at 5 years in 10% of RE groups I-IV eyes and 47% of RE group V eyes. Multivariate factors predictive of treatment with external beam radiotherapy included non-Caucasian race, male sex, and RE group V disease. Failure of chemoreduction and the need for treatment with enucleation occurred in 13% eyes at 1 year, 29% at 3 years, and 34% at 5 years. More specifically, enucleation was necessary in 15% of RE groups I-IV eyes at 5 years and in 53% of RE group V at 5 years. Multivariate factors predictive of treatment with enucleation included patient age older than 12 months, single tumor in eye, and tumor proximity to foveola within 2 mm. Overall, of the 158 eyes, 50% required external beam radiotherapy or enucleation and 50% were successfully managed without these treatments. No patient developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms over the 5-year follow up. CONCLUSIONS: Chemoreduction offers satisfactory retinoblastoma control for RE groups I-IV eyes, with treatment failure necessitating additional external beam radiotherapy in only 10% of eyes and enucleation in 15% of eyes at 5-year follow-up. Patients with RE group V eyes require external beam radiotherapy in 47% and enucleation in 53% at 5 years.

Antineoplastic Combined Chemotherapy Protocols↗

Cone damage in patients receiving high-dose irofulven treatment.

OBJECTIVES: To describe the clinical, perimetric, and electroretinographic (ERG) results of 4 patients with cone dysfunction following irofulven treatment including the histopathologic and immunocytochemical features of one patient's retinas. DESIGN: Observational case series. METHODS: The patients were examined clinically, including perimetric and ERG evaluations. Eyes from patient 1 and healthy postmortem eyes were processed for histopathologic and immunocytochemistry studies with antibodies specific for cones, rods, and reactive Müller cells. MAIN OUTCOME MEASURES: Clinical signs and symptoms, perimetry, ERG, retinal histopathologic and immunocytochemistry study results. RESULTS: All 4 patients had ERG changes consistent with abnormal cone responses and relatively normal rod responses. Compared with control eyes, the retina of patient 1 had approximately half the normal numbers of macular cones and fewer peripheral cones. The number of rods were normal but all rod and cone outer segments were shortened. CONCLUSION: High-dose irofulven treatment causes cone-specific damage with relative sparing of rods.

Adult↗

Leukemic ophthalmopathy in children.

Abnormalities of the eye were detected in 52 of 657 children (9%) suffering from acute leukemia. The treatment for leukemia was single agent chemotherapy administered sequentially. "Prophylactic" treatment of the central nervous system was not administered. Major manifestations of leukemic ophthalmopathy comprised retinal hemorrhage (in 19) and infiltration of the optic nerve, retina, iris or orbit (29). The ophthalmopathy was treated by topical dexamethasone and radiation therapy. Concurrent bone marrow relapse occurred in most patients. Twenty-seven of 29 patients whose cerebrospinal fluid was examined before or at the time of the onset of leukemic ophthalmopathy demonstrated meningeal leukemia. In contrast, this complication has not been observed in recent patients who received "prophylactic" treatment for meningeal leukemia. Leukemic invasion of the eye should receive appropriate recognition; the posterior pole should be included in the treatment of the central nervous system as a pharmacologic sanctuary.

Child↗

Branch artery occlusion. An unusual complication of external carotid embolization.

A case of retinal branch artery occlusion was caused by migration of emboli, presumably via collateral circulation, during therapeutic embolization of the maxillary artery. Migration of particles to the ophthalmic circulation is unusual with embolization of the branches of the external carotid artery. Meticulous technique, careful angiographic monitoring, and proper selection of embolic material may reduce, but not eliminate, migration of emboli to undesirable locations. Therapeutic embolization of vascular tumors and malformations in the external carotid territory is a recent radiologic innovation that is becoming increasingly popular. Therefore, we may expect to see more ocular complications from aberrant emboli as the use of this technique becomes more widespread.

Adolescent↗

Pleomorphic xanthoastrocytoma of the retina.

We report two cases of pleomorphic xanthoastrocytomas of the retina. The immunoreactivity to CD68, Fe (Pearls' technique), astrocytes (argentic technique), and antibodies to glial fibrillary acidic protein was tested in tumor cells. Both neoplasms contained cells immunoreactive for glial fibrillary acidic protein and CD68. These tumors, observed in the CNS, first reported by Kepes et al. were found in the retina as well-circumscribed masses with a cystic component. Many large pleomorphic cells contained vesicular and enlarged nuclei with a homogeneous eosinophilic cytoplasm, prominent nucleoli, and calcium deposits. Some cells had a markedly swollen lipidized cytoplasm, whereas others were spindle-shaped and contained multiple nuclei. These findings suggest that our two specimens have an astroglial lineage and are similar to pleomorphic xanthoastrocytomas of the CNS brain and spinal cord. Both reported cases were in female patients who were in their 20s and had glaucoma. After 10 years both are free of disease.

Adult↗

Retinitis pigmentosa in association with acromegaly: a case report.

Retinal abnormalities, unrelated to visual pathway compression, in acromegalic patients were originally described by Small in 1972 (Smail JM. Primary pigmentary degeneration of the retina and acromegaly in a case of pituitary adenoma. Br J Ophthalmol 1972; 56: 25-31). He illustrated a case of primary pigmentary degeneration of the retina occurring in a patient with a chromophobe adenoma of the pituitary gland. To the best of our knowledge this remains the sole, published case of this association. We report on two male patients, with an acromegalic appearance, one caused by a pituitary adenoma, the other associated with a Rathke's cleft cyst, presenting to our department of ophthalmology with the clinical picture of pigmentary degeneration of the retina.

Acromegaly↗

Early-onset drusen in a girl with bloom syndrome: probable clinical importance of an ocular manifestation.

Ophthalmic examination of a girl admitted with the complaint of growth failure revealed retinal hard drusen. It was surprising to observe drusen in a child because they represent an age-related degenerative change in normal individuals. After further evaluation, she was diagnosed to have Bloom syndrome, a premature aging syndrome. To the authors' knowledge, this is the first case of Bloom syndrome associated with drusen. It is probable that not only aging but also other fundamental cell processes, especially uncontrolled cell proliferation, might be similarly affected and might follow a more rapid course in this inherited condition presenting with drusen. The authors suggest paying extra attention to drusen during the ophthalmic assessment in the diagnosis of all Bloom syndrome patients; it may be prudent to watch more carefully for the development of cancer in patients with drusen than those without drusen.

Adolescent↗

[Combined use of computerized tomography and echography in ophthalmology (author's transl)].

Computerized tomography does not give as fine an information as echography in the presence of intraocular changes, such as retinal detachments, organized vitreous hemorrhages or the differentiation of a choroidal prominence. Complementary computerized tomography, however, can be helpful in the presence of unclear echographic results in opaque media. Furthermore, an analysis of the value of computerized tomography and echography for the evaluation of orbital lesions is presented based mainly on the results of the preoperative tissue diagnosis in 31 histological proven lesions. The results show that a complementary CT-scan is not necessary in orbital lesions of the anterior third when a clear echographic tissue diagnosis exists. In lesions of the middle third of the orbit, echography and CT complement each other. For lesions situated within the orbital apex or for those which are in connection with adjacent structures, such as the intracranial space or the periorbital region, computerized tomography is superior and of a greater help than standardized A-scan echography aided by contact B-scan.

Eye Diseases↗