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At least 793 records · Page 44Linked to original sources
PubMed2001

Retinal capillary hemangioma: a comparison of sporadic cases and cases associated with von Hippel-Lindau disease.

OBJECTIVE: To report observations regarding the onset of tumors in patients with retinal capillary hemangiomas (RCH) and comparing sporadic cases with those associated with von Hippel-Lindau (VHL) disease. DESIGN: Retrospective consecutive case series. PARTICIPANTS: Sixty-eight patients with RCH managed at a tertiary referral center. MAIN OUTCOME MEASURES: The data were analyzed for age at diagnosis of RCH, two-hit mutation model, onset of new tumors, and random (Poisson) distribution. RESULTS: Among 68 patients, we identified 174 RCHs in 86 eyes. Thirty-one patients had VHL disease, and 37 patients were sporadic. The median age at diagnosis in cases with VHL disease was 17.6 years (range, 2.8-46.7 years), and in those without VHL disease it was 36.1 years (range, 7.0-74 years). The age distribution of RCH followed a two-hit mutation model. In eyes with VHL disease, the total number of RCH were distributed randomly as calculated by the Poisson distribution (mean number of tumors = 4). Overall onset of 33 new tumors was documented in 11 eyes (13%) over a median interval of 16 months (range, 3-235 months). All new tumors developed before the age of 47 years. The retinal location of new tumors was similar to the previous tumors with most new tumors located in the superotemporal quadrant (42%) and in the midperipheral retina (58%). Of the 50 patients initially manifesting solitary RCH, only 4 had new tumors develop over a median follow-up duration of 6.4 years (range, 0-33 years). All four cases had VHL disease. CONCLUSIONS: The median age at diagnosis of RCH in patients with VHL disease was almost 18 years less than those without VHL disease. The age distribution of RCH followed the two-hit mutation model. In VHL disease, the number of RCH is determined randomly. Most of the new RCH develop by the age of 47 years. In the absence of VHL disease, it is unlikely for patients with solitary RCH to develop new tumors.

Adolescent↗
PubMed2001

Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma.

PURPOSE: To evaluate the efficacy of multiagent chemotherapy in the neoadjuvant treatment of retinoblastoma. DESIGN: Noncomparative, prospective case series. PARTICIPANTS: Twenty consecutive patients with multifocal intraocular retinoblastoma (4 unilateral, 16 bilateral [36 eyes]). INTERVENTION: Eight cycles of chemotherapy with carboplatin and vincristine were administered at 3-week intervals over a 6-month period. Supplemental therapy was withheld until disease progression was documented. MAIN OUTCOME MEASURES: Disease progression (defined as tumor growth, vitreous or subretinal seed progression, and new tumor formation), delay of external beam radiotherapy, and ocular survival. RESULTS: Thirty-six eyes were treated. Eighteen eyes had Reese-Ellsworth group I-III tumors, and 16 eyes had Reese-Ellsworth group IV-V tumors at diagnosis. Two patients, who had unilateral disease at diagnosis, subsequently had tumors develop in the contralateral eye. Nineteen of 20 patients (95%) completed eight cycles of chemotherapy without disease progression. Three eyes of three different patients were successfully treated with chemotherapy alone. Thirty-three of 36 eyes (92%) progressed after completion of chemotherapy: 15 of the 18 eyes (83.3%) with Reese-Ellsworth group I-III and 16 of 16 eyes (100%) with Reese-Ellsworth group IV-V tumors. Seventeen eyes (52%) had growth of a tumor, whereas 14 eyes (42%) had progressive vitreous seeding, and 2 eyes (6%) had new tumors develop. Fifteen eyes (42%) required external beam radiotherapy. Twenty-nine of 36 (80.5%) eyes were salvaged. The median follow-up after chemotherapy was 19 months (range, 3-42 months). CONCLUSIONS: Multiagent chemotherapy alone does not ensure a cure for multifocal intraocular retinoblastoma. Supplemental focal therapy is needed to control disease progression.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Chemothermotherapy in the management of retinoblastoma.

OBJECTIVE: To evaluate the results of chemothermotherapy for the treatment of retinoblastoma. DESIGN: Non-comparative interventional case series. PATIENTS: Fifty-one children (65 eyes and 103 tumors) were treated with chemothermotherapy in a single institution from January 1995 to May 1998. METHODS: Chemothermotherapy consists of a combination of transpupillary thermotherapy delivered shortly after intravenous (IV) injection of carboplatin (560 mg/m(2)). Each tumor is treated separately with a diode laser using a microscope. Laser intensity, spot size, and duration are adapted to the size of each tumor and to the clinical response. After 8 days, thermotherapy alone is repeated. This cycle is performed from one to six times, every 28 days. The treatment data and outcome are analyzed separately. MAIN OUTCOME MEASURES: Assessment of local tumor control. RESULTS: One hundred three tumors were treated in 65 eyes of 51 children. Age at diagnosis was 0 to 60 months (median, 7 months). Median tumor diameter at the time of treatment was 3.5 mm (range, 1.5-12 mm). Laser modalities were as follows: median intensity, 450 mW (range, 150-1000 mW); median spot size, 1.2 mm (range, 0.3-2.0 mm); and median number of cycles required to obtain tumor control, three. Tumor regression was obtained for 99 tumors (96.1%) after a median follow-up of 30 months (17-61 months). Seven tumors relapsed after initial control (6.8%). Salvage treatment (external beam radiation, iodine plaques, or enucleation) was necessary for a total of 11 tumors (10.7%). The only risk factor for relapse was the initial diameter of the lesion greater than 3.5 mm, whereas the other tumor characteristics or treatment variables were not significantly correlated with relapse. Ninety-seven percent of treated eyes were able to be preserved, and 92% of cases were treated without external beam radiation. CONCLUSIONS: Chemothermotherapy is an effective technique to treat small- to medium-sized retinoblastomas in children, avoiding external beam irradiation.

Antineoplastic Agents↗
PubMed2002

Human T-cell lymphotropic virus type-1 associated t-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis.

OBJECTIVE: To report a case of adult T-cell leukemia/lymphoma (ATL) presenting as a bilateral retinal vasculitis and diagnosed by molecular detection of a rearrangement in the T-cell receptor (TCR) and the presence of the human T-cell lymphotropic virus type 1 (HTLV-1) pol gene in the malignant lymphoid cells. DESIGN: Case report. METHODS: Routine histologic and immunohistochemical analyses were performed on the retinal biopsy specimen before referral to the National Eye Institute. Lymphoid cells associated with granulomatous inflammation infiltrating the retina and surrounding retinal blood vessels were microdissected from the paraffin sections of the retinal biopsy specimen. The polymerase chain reaction (PCR) was performed using primers for the TCR gene and HTLV-1 pol and gag genes. RESULTS: Microscopic examination showed a necrotizing granulomatous retinal vasculitis with a predominant T-cell infiltrate detected by immunohistochemistry. Molecular analysis demonstrated a clonal rearrangement of the TCR and the presence of the HTLV-1 pol gene in the microdissected lymphoid cells diagnostic of ATL. CONCLUSIONS: Necrotizing retinitis and retinal vasculitis are rare manifestations of ATL. Human T-cell lymphotropic virus type 1 infection should be considered in the differential diagnosis of patients from endemic areas who have retinal vasculitis at presentation. This case further demonstrates the usefulness of microdissection and PCR for the diagnosis of ocular disease, including HTLV-1 infection.

Adult↗
PubMed2002

Treatment of retinal capillary hemangioma.

OBJECTIVE: To report on the methods of treatment and the visual outcome of eyes with retinal capillary hemangioma (RCH). DESIGN: Retrospective consecutive noncomparative case series. PARTICIPANTS: Data on 68 patients with RCH were reviewed for the methods of treatment and visual outcome. METHODS: Statistical analysis using cumulative multinomial logistic regression (generalized estimating equations) was performed to determine significant predictors of visual outcome. RESULTS: Among 68 patients with RCH with von Hippel-Lindau disease (n = 31) and without von Hippel-Lindau disease (n = 37), we identified 174 RCH in 86 eyes. The overall median age at diagnosis of RCH was 30.8 years (range, 2.8-73.8 years). The RCH was bilateral in 18 cases (26%), and a family history of von Hippel-Lindau disease was positive in 19 cases (28%). Twenty-nine of the tumors (17%) touched the optic disc and were classified as juxtapapillary RCH, and the remaining 145 (83%) were extrapapillary in location. Ninety-nine (58%) RCH were 1.5 mm or smaller in size. The RCH were initially managed by observation (46%), laser photocoagulation (25%), or cryotherapy (23%). Small RCH (< or =1.5 mm in size; 63 of 99; 64%) and those touching the optic disc (14 of 29; 48%) were more likely to be initially observed. Sixty-three (82%) of the 77 RCH that were initially observed remained stable for a median follow-up of 84 months. The remaining 14 progressed and were successfully controlled with laser photocoagulation or cryotherapy. Either laser photocoagulation or cryotherapy was effective as the sole method of treatment in controlling 74% (26 of 35) and 72% (28 of 39) of extrapapillary tumors, with a mean number of 1.2 and 1.1 sessions, respectively. In a multivariate model, the only variables that were significantly related to final vision of </= 20/400 were poor initial vision (P = 0.01, odds ratio [OR], 8.5; 95% confidence interval [CI], 1.7,42) and the presence of retinal/vitreous hemorrhage (P = 0.024, OR, 5.7; 95% CI, 1.3, 25.6). CONCLUSIONS: RCH can be safely observed initially in selected cases. Laser photocoagulation and cryotherapy are the mainstays of treatment in most cases. Early detection of RCH and treatment before the onset of severe visual loss is recommended.

Adolescent↗
PubMed2003

Toxicity and dose-response studies of 1-alpha hydroxyvitamin D2 in LH-beta-tag transgenic mice.

PURPOSE: To determine the effectiveness of a vitamin D analog, 1alpha-hydroxyvitamin D(2) (1alpha-OH-D(2)), in inhibiting retinoblastoma in a transgenic retinoblastoma model (LHbeta-Tag mouse) and to evaluate its toxicity. DESIGN: Experimental study using an animal (LHbeta-Tag transgenic mouse) randomized (controlled) trial. PARTICIPANTS AND CONTROLS: Two hundred seventeen LHbeta-Tag transgene-positive 8- to 10-week-old mice total; 179 drug-treated animals, 38 control animals. METHODS: Mice were fed a vitamin D- and calcium-restricted diet and were randomized to treatment groups receiving control (vehicle), or 0.1, 0.3, 0.5, or 1.0 micro g/day of 1alpha-OH-D(2) via oral gavage 5 times weekly for 5 weeks. Body weight was measured at the start of treatment and twice weekly during treatment. Animals were euthanized on the last day of treatment. The eyes were enucleated, processed histologically, and serially sectioned. Representative sections from the superior, middle, and inferior regions of each globe were examined microscopically and tumor areas were measured using Optimas software. Serum was collected for serum calcium levels. Kidneys were removed for histologic processing and were analyzed microscopically for kidney calcification. MAIN OUTCOME MEASURES: Mean tumor area was measured to determine drug effectiveness. Toxicity was assessed by survival, weight loss over the treatment period, serum calcium, and kidney calcification. RESULTS: The mean tumor size in each 1alpha-OH-D(2) group was smaller than controls (all P values < 0.02): control, 90,248 micro m(2); 0.1 micro g, 31,545 micro m(2); 0.3 micro g, 16,750 micro m(2); 0.5 micro g, 30,245 micro m(2); and 1.0 micro g, 16,049 micro m(2). No dose-dependent response curve was evident. The survival percentage for each group was as follows: control, 97%; 0.1 micro g, 91%; 0.3 micro g, 88%; 0.5 micro g, 70%; and 1.0 micro g, 63%. Mortality was higher in the 0.5- micro g and 1.0- micro g doses (P values < 0.01) compared with other treatment groups and with the control group. Serum calcium levels were significant in all treatment groups compared with controls (all P values < 0.0001). CONCLUSIONS: In the LHbeta-Tag mouse, 1alpha-OH-D(2) inhibits retinoblastoma with no significant increase in mortality in lower doses (0.1-0.3 micro g). 1alpha-OH-D(2) has approval by the Food and Drug Administration as an investigative drug for cancer treatment, and has shown efficacy with low toxicity in adult cancer trials. 1alpha-OH-D(2) meets the criteria for human clinical trials.

Animals↗
PubMed1999

Persistent hyperplastic primary vitreous with retinal tumor in tuberous sclerosis: report of a case including tumoral immunohistochemistry and cytogenetic analyses.

OBJECTIVE: The authors describe an ocular lesion combining the characteristics of persistent hyperplastic primary vitreous (PHPV) and a retinal tumor in an infant with tuberous sclerosis complex (TSC). STUDY DESIGN: Case report. METHODS: Immunohistochemistry and cytogenetic studies were performed on TSC cells from an intraocular tumor in a 6-week-old infant. RESULTS: Histopathologic examination showed a thick fibrovascular membrane between the aspect of the lens and the astrocytic component of the mass. Glial fibrillary acidic protein (GFAP) showed a variable intracytoplasmic reaction in the astrocytic proliferation, involving approximately 50% of the cells. Tissue culture studies showed a fairly rapid proliferation of fusiform cells, consistent with bipolar astrocytic cells. Cytogenetic studies showed one abnormal clone consisting of three hyperdiploid cells with a loss of chromosome 9 and a gain of chromosomes 6 and 12. CONCLUSION: The atypical localization of the retinal tumor could be explained by the fact that it was trapped during its proliferation by the retinal detachment associated with the PHPV.

Astrocytoma↗
PubMed1999

A phase I/II study of subconjunctival carboplatin for intraocular retinoblastoma.

PURPOSE: To evaluate the efficacy and toxicity of subconjunctival carboplatin for intraocular retinoblastoma. DESIGN: A phase I/II clinical trial (noncomparative case series). PARTICIPANTS: Eleven children with bilateral retinoblastoma who had 13 eyes containing active tumor. METHODS: Subconjunctival carboplatin (1.4-2.0 ml of a 10-mg/ml solution) was administered. Ophthalmologic examinations with the patient under anesthesia were performed before each injection, and response and toxicity were assessed. MAIN OUTCOME MEASURES: Tumor response and toxicities. RESULTS: A median of three injections per eye was administered (range, 1-7 injections per eye) at a median interval of 21 days between injections (range, 14-35 days). The median dose was 20 mg/injection (range, 14-20 mg). Toxicities included transient periorbital edema in four eyes and optic atrophy in one eye that also received laser photocoagulation and cryotherapy. No nonocular toxicities were noted. Three of five eyes with vitreous disease that could be evaluated had major responses. Two of five eyes with retinal tumors had a response, but neither eye with subretinal disease responded. CONCLUSIONS: Subconjunctival carboplatin as used in this protocol is effective for intraocular retinoblastoma. It appears to be safe, but additional study and longer follow-up are required, particularly to determine the risk of optic atrophy.

Antineoplastic Agents↗
PubMed1999

Clinical and immunocytochemical findings in a case of melanoma-associated retinopathy.

OBJECTIVE: To describe an unusual case of melanoma-associated retinopathy (MAR). DESIGN: Retrospective, observational case report and experimental study. PARTICIPANTS: A 61-year-old man with a history of cutaneous melanoma, acquired bilateral central scotomas, and night blindness. INTERVENTION: Serial full-field electroretinography (ERG) and Goldmann perimetry were performed. Serum was screened for cancer-associated retinopathy (CAR) antibodies by Western blotting. Sections of human and rat retina were examined by immunofluorescence microscopy to determine whether retinal cells were reactive with this patient's serum. A metastatic workup was performed. MAIN OUTCOME MEASURES: Electroretinography, Goldmann visual field testing, and immunocytochemistry were performed. RESULTS: The results were as follows: (1) The ERG showed a profound loss of the b-wave amplitude and a "negative" b-wave characteristic of congenital stationary night blindness; (2) a central scotoma and peripheral constriction were identified on Goldmann visual field tests; (3) as in other patients with MAR, bipolar cells in human and rat retinas were immunolabeled with this patient's serum; and (4) a previously unsuspected focus of metastatic melanoma was discovered. CONCLUSIONS: Recognition of this condition may help to identify an occult focus of metastatic melanoma.

Blotting, Western↗
PubMed2001

6p abnormalities and TNF-alpha over-expression in retinoblastoma cell line.

Retinoblastoma is the most common primary intra-ocular tumor in childhood. It has been established that recurrent cytogenetic abnormalities are the hallmark of mostly all malignant tumors. Recurrent atypical cytogenetic abnormalities of the short arm of chromosome 6 were reported in several cases of retinoblastoma, mainly in association with reciprocal translocations. In the present study, alterations of chromosome 6 associated with retinoblastoma were studies in the Y-79 and the WERI-Rb-1 cell lines established from highly malignant retinoblastomas. Fluorescence in situ hybridization (FISH) technique was used to identify the chromosomal breakpoint on 6p in this cell line. To perform this detection, yeast artificial chromosome (YAC) clones from p21 to p22 bands were used and the breakpoint was localized on 6p21.3. Previous studies had suggested that activation of some genes on 6p could be dependent on a translocation mechanism. Expression of a candidate gene localized near the chromosomal breakpoint was measured by immunocytochemistry and flow cytometry techniques. An enhancement of the tumor necrosis factor-alpha (TNF-alpha) protein expression in Y79 cells was detected by this approach. The relationship between TNF-alpha over-expression and the malignancy of retinoblastoma is discussed.

Blotting, Southern↗
PubMed2005

[Functional results of retinoblastoma treatment with local treatment used in isolation or associated with chemotherapy].

INTRODUCTION: Because of long-term complications of external beam radiation in retinoblastoma, a new therapeutic modality using chemotherapy and local treatments is used whenever possible. We conducted a retrospective study to evaluate visual results. PATIENTS AND METHOD: We studied visual acuity and ocular side effects in children presenting uni- or bilateral retinoblastoma in whom we were able to achieve conservative management without external beam treatment. The treatments that were used included chemotherapy, chemothermotherapy, diode laser, iodine 125 plaque brachytherapy, and cryotherapy. The initial characteristics (diameter, subretinal or vitreous seeding, and location) of each tumor, the treatments used, their results, and the visual function after age 4 years were recorded. RESULTS: We treated 429 children for retinoblastoma at the Curie Institute between October 1994 and December 2002. Two hundred twenty-seven eyes had conservative treatment without external beam. We were able to study the visual function in 102 eyes at a median age of 67 months. The median follow-up after the end of the treatment was 5 years. The mean visual acuity was 20/37 and 60% of the children had visual acuity of more than 20/40. Macular alteration was observed in 34 eyes. Statistical analysis showed that a larger tumor diameter of the retinal surface (p<0.0003) and location close to the macula (p<0.0001) were the most significant risk factors for vision loss. CONCLUSION: The visual results of the treatment of retinoblastoma by chemotherapy and local treatments is good when the tumors are not located close to the macula. Larger tumors also have a worse prognosis for vision.

Child, Preschool↗
PubMed2005

[Bourneville's tuberous sclerosis. A case report].

INTRODUCTION: Bourneville's tuberous sclerosis (BTS) is an autosomal dominant phakomatosis characterized by the development of a benign hamartoma-like tumor, which is usually located in the skin, kidney, heart, brain, and eyes. We present here a case of a retinal BTS of late diagnosis. We also discuss the clinical course and progress of this condition. OBSERVATION: A 28-year-old man was a known BTS patient followed in the dermatology clinic. The ophthalmologic exam and the angiography with fluorescein revealed retinal astrocytic hamartomas bilaterally. Dermatologic examination also showed facial angiofibromas. The remainder of the physical examination was unremarkable. The chest X-ray, renal scan, heart scan and a CT scan of the brain failed to show any other localizations of the disease. The lesions described had remained unchanged over a period of 6 years. CONCLUSION: Retinal lesions during BTS are dominated by the presence of astrocytic hamartomas essentially around the papillae. They are often an incidental diagnosis and evolve slowly. Except in complicated cases, their prognosis is usually satisfactory.

Adult↗
PubMed2006

[Multiple bilateral retinomas. A case study].

The case of an asymptomatic 29-year-old male patient with bilateral multiple retinomas is described. Initially misdiagnosed as unilateral pathological myopia 10 years earlier, his ocular tumors were recognized only when examined after detection of bilateral retinoblastoma in his daughter. There was a large gray translucent mass and extensive chorioretinal atrophy in the right fundus and four small discrete tumors in the nasal periphery of the left fundus. There was no progression over 4 years of follow-up. Timely and correct diagnosis of retinoma is essential for appropriate genetic counseling, which would minimize the risk of retinoblastoma in future generations.

Adult↗