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PubMedDate not supplied

Involution of retinochoroidal shunt vessel after radiotherapy for optic nerve sheath meningioma.

PURPOSE: To illustrate the development and involution of retinochoroidal shunt vessel of the optic disc in a patient with primary optic nerve sheath meningioma. CASE REPORT: A 38-year-old woman presented with gradual onset of blurred vision in her right eye. Examination revealed a right relative afferent pupillary defect and mild edema of the right optic disc. Computed tomography (CT) showed changes consistent with a primary optic nerve sheath meningioma affecting the orbital portion of the right optic nerve. Patient was observed without treatment and subsequently developed a retinochoroidal shunt vessel on her right optic disc. With progression of the meningioma towards the optic chiasm, treatment with stereotactic radiosurgery was done. Following treatment, the optic disc gradually became pale and the retinochoroidal shunt vessel decreased in caliber with complete involution three years after radiotherapy.

Adult↗
PubMedDate not supplied

Intraocular astrocytoma without phacomatosis.

PURPOSE: Astrocytic tumors occur in the retina or in the optic disc usually as a part of tuberous sclerosis complex or other phacomatosis and their isolated occurrence is rare. The authors present two adult patients in whom the diagnosis of intraocular astrocytoma was established but no signs of phacomatosis were revealed.

Astrocytoma↗
PubMedDate not supplied

Retinocytoma associated with calcified vitreous deposits.

PURPOSE: To report a case of bilateral retinocytoma associated with calcified vitreous deposits. METHODS: Case report. RESULTS: On routine examination, a 35-year-old asymptomatic father of a child with bilateral retinoblastoma presented bilateral retinocytoma associated with vitreous calcifications, in the vicinity of the retinocytoma in his left eye. Fundus photographic documentation and fluorescein angiography were performed. The patient has been followed up for 10 years. CONCLUSIONS: The lesions in both eyes have remained stable without signs of growth or malignant transformation. Calcified vitreous deposits are a recently described feature of retinocytoma in addition to the three classic features: translucent retinal mass, retinal pigment epithelial alteration, and calcification.

Adult↗
PubMedDate not supplied

Phthisis bulbi and buphthalmos as presenting signs of retinoblastoma: a report of two cases and literature review.

PURPOSE: To report two cases of bilateral retinoblastoma (RB) with unusual presentations. METHODS: The medical records of 321 patients from the Retinoblastoma Referral Center in Siena were reviewed. A total of 111 patients had bilateral RB, 2 of them presenting with phthisis bulbi and buphthalmos. Both patients underwent bilateral enucleation. Clinical features, imaging studies, and histopathology were reviewed. RESULTS: These 2 cases represent 0.62% (2/321) in our series. Histopathology did not reveal viable tumor cells in the phthisical eyes; in both buphthalmic eyes the tumor was active, infiltrating the choroid and optic nerve. CONCLUSIONS: Phthisis bulbi and buphthalmos are unusual presenting signs of RB. This very rare combination of these two signs in different eyes of the same patient is probably due to a delay in diagnosis.

Child, Preschool↗
PubMedDate not supplied

Ocular and orbital complications following the treatment of retinoblastoma.

PURPOSE: To investigate the ocular and orbital complications observed in children treated for retinoblastoma. SUBJECTS AND METHODS: We retrospectively studied 73 children (39 boys, 34 girls) suffering from retinoblastoma. Thirty-six had bilateral tumor and 37 unilateral disease for a total of 109 eyes affected. The follow-up was 6-180 months (median 36 months). Enucleation was the most common initial treatment approach in the unilateral group, and radiotherapy (by external beam) was the most common initial therapy in the bilateral group. Cryotherapy, photocoagulation, brachytherapy and/or systemic chemotherapy were used as adjuvant treatments when necessary. Ocular complications were recorded at the follow-up examinations. RESULTS: Cataract developed in 20% of the irradiated eyes. The mean time from irradiation until development of cataract was 28.5 months (6-64 months). Radiation retinopathy developed in 12% and was first detected 11-72 months (mean 37 months) after irradiation therapy. Mild transient keratopathy was observed in all eyes undergoing irradiation, and xerophthalmia in one eye. Complications after enucleation included: marked discharge from the socket (11.0%), extrusion of the implant (9.6%), and contraction of the socket (3.0%). No complications were observed after cryotherapy or laser photocoagulation of the tumor. CONCLUSIONS: Ocular complications after treating children with retinoblastoma are common and may seriously affect the quality of life of children surviving the primary malignancy.

Brachytherapy↗
PubMed2006

Retinoblastoma with cerebrospinal fluid metastasis treated with orbital and craniospinal irradiation using IMRT.

A 4-year-old male child presented with history of leucocoria, photophobia, and progressive visual deterioration in the left eye. Clinical examination and computed tomography revealed a unilateral retinoblastoma involving the left globe. He underwent enucleation of the left eye and was referred for adjuvant radiotherapy in view of optic nerve infiltration up to the line of resection. However, he did not report for treatment and presented six months later with a recurrent mass in the left orbit with intracranial extension. Cerebrospinal fluid (CSF) cytology was positive for malignant retinoblastoma cells. He received multiagent chemotherapy with vincristine, carboplatin, and etoposide along with intrathecal methotrexate. Although the recurrent orbital mass reduced significantly with chemotherapy, malignant cells persisted in the CSF. He was subsequently treated using intensity modulated radiation therapy (IMRT) to treat the left orbital mass along with craniospinal axis irradiation. Computed tomography done at three and nine months after completion of radiotherapy showed complete disappearance of orbital tumor. CSF cytology also showed no evidence of malignant cells.

Cerebrospinal Fluid↗
PubMed2003

Glycosaminoglycans in human retinoblastoma cells: heparan sulfate, a modulator of the pigment epithelium-derived factor-receptor interactions.

BACKGROUND: Pigment epithelium-derived factor (PEDF) has binding affinity for cell-surface receptors in retinoblastoma cells and for glycosaminoglycans. We investigated the effects of glycosaminoglycans on PEDF-receptor interactions. RESULTS: 125I-PEDF formed complexes with protease-resistant components of medium conditioned by human retinoblastoma Y-79 cells. Using specific glycosaminoglycan degrading enzymes in spectrophotometric assays and PEDF-affinity chromatography, we detected heparin and heparan sulfate-like glycosaminoglycans in the Y-79 conditioned media, which had binding affinity for PEDF. The Y-79 conditioned media significantly enhanced the binding of 125I-PEDF to Y-79 cell-surface receptors. However, enzymatic and chemical depletion of sulfated glycosaminoglycans from the Y-79 cell cultures by heparitinase and chlorate treatments decreased the degree of 125I-PEDF binding to cell-surface receptors. CONCLUSIONS: These data indicate that retinoblastoma cells secrete heparin/heparan sulfate with binding affinity for PEDF, which may be important in efficient cell-surface receptor binding.

Chlorates↗
PubMed2006

Retinoblastoma.

Retinoblastoma is a rare eye tumor of childhood that arises in the retina. It is the most common intraocular malignancy of infancy and childhood; with an incidence of 1/15,000-20,000 live births. The two most frequent symptoms revealing retinoblastoma are leukocoria and strabismus. Iris rubeosis, hypopyon, hyphema, buphthalmia, orbital cellulites and exophthalmia may also be observed. Sixty per cent of retinoblastomas are unilateral and most of these forms are not hereditary (median age at diagnosis two years). Retinoblastoma is bilateral in 40% of cases (median age at diagnosis one year). All bilateral and multifocal unilateral forms are hereditary. Hereditary retinoblastoma constitutes a cancer predisposition syndrome: a subject constitutionally carrying an RB1 gene mutation has a greater than 90% risk of developing retinoblastoma but is also at increased risk of developing other types of cancers. Diagnosis is made by fundoscopy. Ultrasound, magnetic resonance imaging (MRI) and computed tomography (CT) scans may contribute to diagnosis. Management of patients with retinoblastoma must take into account the various aspects of the disease: the visual risk, the possibly hereditary nature of the disease, the life-threatening risk. Enucleation is still often necessary in unilateral disease; the decision for adjuvant treatment is taken according to the histological risk factors. Conservative treatment for at least one eye is possible in most of the bilateral cases. It includes laser alone or combined with chemotherapy, cryotherapy and brachytherapy. The indication for external beam radiotherapy should be restricted to large ocular tumors and diffuse vitreous seeding because of the risk of late effects, including secondary sarcoma. Vital prognosis, related to retinoblastoma alone, is now excellent in patients with unilateral or bilateral forms of retinoblastoma. Long term follow-up and early counseling regarding the risk of second primary tumors and transmission should be offered to retinoblastoma patients.

Child, Preschool↗
PubMed1997

Role of the retinoblastoma protein in the pathogenesis of human cancer.

The retinoblastoma gene (RB-1) was originally identified as the gene involved in hereditary retinoblastoma. However, RB-1 mutations are found in a number of common mesenchymal and epithelial malignancies. The retinoblastoma protein (pRB) acts as a transcriptional regulator of genes involved in DNA synthesis and cell-cycle control. In this regard, the functional interaction between pRB and the E2F transcription factor family appears to be critical. The pRB-E2F interaction is, in turn, regulated by a pathway that includes cyclin D1, cdk4, and p16. Mutations that affect this pathway have been documented in nearly every type of adult cancer. Thus, perturbation of pRB function may be required for the development of cancer. Insights into the biochemical functions of pRB, and its upstream regulators, may form the basis for the development of novel antineoplastic agents.

Adult↗
PubMed2000

Chemoreduction and local ophthalmic therapy for intraocular retinoblastoma.

PURPOSE: To study the effectiveness of combined systemic chemotherapy and local ophthalmic therapy for retinoblastoma with the goal of avoiding enucleation and external-beam radiation therapy (EBRT). PATIENTS AND METHODS: This was a prospective, nonrandomized, single-arm clinical trial. Seventy-five eyes were followed in 47 children. Patients were treated with a six-cycle protocol of vincristine, etoposide, and carboplatin. Most (83%) also received ophthalmic treatment (cryotherapy, laser photocoagulation, thermotherapy, or plaque radiation therapy) during and/or after the chemotherapy. RESULTS: With a median follow-up of 13 months, event-free survival was 74%, with an event defined as enucleation and/or EBRT. Six children required EBRT in seven eyes (9%); five required enucleation of one eye (7%); five required a combination of EBRT and enucleation in six eyes (8%). Reese-Ellsworth groups 1, 2, and 3 eyes had excellent results, with avoidance of EBRT or enucleation in all 39. Treatment of groups 4 and 5 was less successful, with 33% of six eyes and 53% of 30 eyes, respectively, requiring EBRT and/or enucleation. Toxicities from chemotherapy were mild and included cytopenias (89%), fever and neutropenia (28%), infection (9%), and gastrointestinal symptoms, dehydration, and vincristine neurotoxicity (40%). No patients developed a second malignancy, metastatic disease, renal disease, or ototoxicity. CONCLUSION: In retinoblastoma patients with Reese-Ellsworth eye groups 1, 2, or 3, systemic chemotherapy used with local ophthalmic therapies can eliminate the need for enucleation or EBRT without significant systemic toxicity. More effective therapy is required for Reese-Ellsworth eye groups 4 and 5.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

First-line chemotherapy with local treatment can prevent external-beam irradiation and enucleation in low-stage intraocular retinoblastoma.

PURPOSE: To evaluate the efficacy of first-line chemotherapy (CT) in preventing external-beam radiotherapy (EBR) and/or enucleation in patients with retinoblastoma (Rbl). PATIENTS AND METHODS: Twenty-four patients with newly diagnosed unilateral or bilateral Rbl received CT associated with local treatment (LT). Two to five courses of etoposide and carboplatin were administered at 3- to 4-week intervals, depending on tumor response, and were completed each time by LT. RESULTS: Tumor response was observed in all eyes. Twenty-one of 24 patients showed a complete response (CR) that persisted at a median follow-up (FU) of 31 months (range, 4 to 41 months). Among the three patients who relapsed, two were lost to FU and one died of progressive disease. CR was achieved by CT and LT alone in 15 (71.4%) of 21 patients with less advanced disease (groups I to III). Six other patients with advanced disease (groups IV and V) experienced treatment failure and needed salvage treatment by EBR and/or enucleation. The difference between the two patient groups with regard to disease stage was statistically significant (P <.0001). EBR could be avoided in 13 (68.4%) of 19 patients, who presented with groups I to III (15 eyes) and group V (one eye) disease, whereas enucleation could be avoided in only two (40%) of five. CONCLUSION: CT combined with intensive LT is effective in patients with groups I to III Rbl, permitting the avoidance of EBR in the majority of these young children and, thus, reducing the risk of long-term sequelae. This is in contrast with the disappointing results for patients with groups IV and V Rbl, in whom EBR and/or enucleation was needed.

Adolescent↗
PubMed2001

A 13-year-old boy with cognitive impairment, retinoblastoma, and Wilson disease.

A developmentally delayed child manifested retinoblastoma at age 4 years and Wilson disease at age 11, a previously unreported association. Cytogenetic and molecular analysis showed an interstitial deletion in the long arm of the paternally derived homologue of chromosome 13 (13q14.2-13q22.2), which encompasses the retinoblastoma and Wilson disease loci. The authors postulate that the co-occurrence of retinoblastoma and Wilson disease was the consequence of an acquired somatic mutation at the retinoblastoma locus and an inherited mutation at the Wilson disease locus of the maternally derived chromosome 13, superimposed on the hemizygosity associated with the paternally derived deletion.

Brain↗
PubMed1997

A case of nonfunctioning pituitary adenoma resembling so-called silent corticotroph adenoma.

It has been indicated that some subsets of clinically nonfunctioning pituitary adenoma exhibit immunohistochemical and ultrastructural features of somatotroph or corticotroph cells, which are described as silent somatotroph and corticotroph adenomas, respectively. We here describe a 70-year-old woman with nonfunctioning pituitary adenoma presenting with visual disturbance. None of the pituitary hormones show abnormal finding. Magnetic resonance imaging study revealed a pituitary tumor which developed inferiorly into the sphenoidal sinus. Tumor resection was performed by a transsphenoidal approach, and resulted in a partial resection of the tumor. The specimen indicated chromophobic adenoma. Immunohistochemical examination revealed a characteristic distribution pattern of cytokeratin staining for ACTH cell adenoma, although immunostaining for ACTH was only weakly positive. After the operation, only ACTH secretion was impaired, requiring replacement therapy with glucocorticoid. Bromocriptine was administered in order to prevent the recurrence of the tumor. It remains to be elucidated whether the present case could be classified as silent corticotroph adenoma, which was originally indicated to have aggressive characteristics, i.e., progressive visual defect, high incidence of infarction and frequent recurrence.

Adenoma, Chromophobe↗
PubMed1999

Utility of pupillary dilation for detecting leukocoria in patients with retinoblastoma.

In the United States, 50% of all retinoblastoma cases are diagnosed after the observation of leukocoria by a family member or primary care physician. However, leukocoria produced by retinoblastoma lesions can often be missed by direct ophthalmoscopic examination through an undilated pupil. The purpose of this study is to demonstrate the utility of pupillary dilation for the detection of leukocoria in suspected cases. Seven patients (10 eyes), aged 2 days to 20 months, with retinoblastoma were examined for leukocoria using a direct ophthalmoscope with the pupils first undilated and then after pharmacologic dilation with 0.5% cyclopentolate and 2.5% phenylephrine. Leukocoria was detected by direct ophthalmoscopy on undilated examination in 3 of 10 eyes (30%). In contrast, leukocoria was observed after pupillary dilation in 10 of 10 eyes (100%). The retinoblastoma lesions, from 2 to 10 mm in diameter, were located within the posterior 45 degrees of the retina. Pupillary dilation is a safe and effective tool that can enhance the ability of the examiner to detect leukocoria. Dilation may afford early diagnosis and treatment, and therefore should be considered on patients in whom the diagnosis of retinoblastoma is entertained.

Dilatation↗