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Introduction. Uveal melanoma (UM) is an intraocular tumor that leads to metastatic disease in approximately 50% of afflicted patients. There is no efficacious treatment for metastatic disease in this cancer. Identification of markers that can offer prognostic and therapeutic value is a major focus in this field at present. KAI1 is a metastasis suppressor gene that has been reported to play a role in various human malignancies, although it has not previously been evaluated in UM.Purpose. To investigate the expression of KAI1 in UM and its potential value as a prognostic marker.Materials and Methods. 18 cases of human primary UM were collected and immunostained for KAI1 expression. A pathologist evaluated staining intensity and distribution semiquantitatively. Each case was categorized as group 1 (low staining) or group 2 (high staining).Results. In group 2, two of the 12 cases presented with metastasis. Conversely, in group 1, five out of 6 cases had metastasis. The mean follow-up of patients who did not develop metastasis was 81.81 months (median: 75 months) versus 42.14 months (median: 44 months) for patients with metastasis.Conclusions. KAI1 is a promising candidate marker that may offer prognostic value in UM; it may also represent a therapeutic target in metastatic disease.

Retinoblastoma is the most common primary intraocular tumor in children. Retinoblastoma can be hereditary (familial) or nonhereditary (nonfamilial). Nurses who have an understanding of the genetic patterns for retinoblastoma can participate in the counseling of these patients. A chart is provided as a tool for teaching patients about family patterns of retinoblastoma.

We review the genetics of retinoblastoma and the most recent molecular detection methods. Retinoblastoma, the most common intraocular tumor in children, occurs in either a heritable or non-heritable form. The heritable form, which is highly penetrant and predisposes individuals to a considerably greater second tumor risk, can be distinguished most definitively through the use of genetic testing. Genetic testing for individuals and families suspected of carrying the heritable mutations can help ensure that more comprehensive treatment plans and accurate genetic counseling for affected individuals and their families are provided.

Retinoblastoma (RB) is the most frequent malignant intraocular tumor in childhood. Six hundred and thirty-six cases with 831 RB-affected eyes were diagnosed and treated in our specialist center between 1963-1994. The diagnosis was made by histopathologic examination in 617 cases and clinically in 19 cases. Four hundred and forty-one (69.3%) cases were unilateral and 195 (30.7%) were bilateral. Two hundred and sixty-eight (42.1%) were females and 368 (57.9%) were males. The youngest patient was 20 days old and the oldest was 16 years old at the time of diagnosis (mean: 2.2 years). In thirty-four (5.3%) cases, a family history of RB was present. Ten of these cases were unilateral and 24 were bilateral. The most frequent presenting signs were leukocoria (394 cases, 61.9%), buphthalmos (92 cases, 14.5%), and strabismus (68 cases, 10.7%). The referring initial diagnoses were correct in 519 (81.6%) cases and false-negative in 117 (18.4%) cases. The most frequent initial false-negative diagnoses of the referring physicians were buphthalmos (43 cases, 6.8%), endophthalmitis (37 cases, 5.8%), and retinal detachment (12 cases, 1.9%). Apart from these 636 cases, there were 29 false-positive RB diagnoses during the same study period for which enucleation was performed. False-positive diagnoses included endophthalmitis (9 cases), retinal dysplasia (6 cases), retinal detachment (5 cases), vitreous hemorrhage (4 cases), Coats' disease (4 cases), and toxocariasis (one case). Ancillary testing for metastasis was carried out in all cases with newly diagnosed retinoblastoma. Five hundred and ninety-eight (72%) eyes had intraocular disease and 233 (28%) had extraocular spread. Of these 233 RBS, 58 had systemic disease. Fifty-two out of 58 tumors showing systemic involvement had either optic nerve or extrascleral extension at the histopathologic examination of enucleation material. The remaining six eyes had intraocular Class IV-V RB.

1543Background: Uveal melanoma (UM) is the most common primary intraocular tumor in adult, but its etiology for the most part remains unclear. It has been suggested that the genetic susceptibility ...

Uveal melanoma (UM) is the most frequent intraocular tumor in adults with an annual incidence of about 6/1.000.000. Despite successful treatment of the primary tumor in most cases, approximately 50% of patients die from metastases within 15 years after treatment. This proportion has remained constant during the last decades.

Retinoblastoma (RB) is the most common malignant intraocular tumor of childhood. The condition is treatable if diagnosed early salvaging vision and the eyeball. Late diagnosis leads to local invasion and rarely metastasis causing considerable morbidity and mortality at least in the developing world. Recent advancement in molecular diagnosis and therapeutic options has improved the management of this disease effectively among patients and their families. Extensive research to understand the events that follows two hits in retinoblastoma susceptibility gene (RB1), cell of origin of RB, molecular mechanism of tumor progression and chemoresistance can pave way to identify biomarkers and molecular targeted therapy for early diagnosis and better treatment options in future.

The ability to obtain the proper clinical diagnosis in cases of suspected intraocular tumors has greatly advanced during the past 50 years. The clinical characteristics of most intraocular tumors (size, shape, color, and texture) are detectable by skilled ophthalmoscopic examination and, with the use of adjunctive techniques (mainly ultrasonography), the proper diagnosis can be reached without invasive procedures. Notwithstanding, some intraocular tumors need to be biopsied to obtain a reliable diagnosis. In the cytogenetic era, intraocular tumor management is changing, and tumor-sampling procedures are becoming the main prognostic (and theoretically also diagnostic) tools for uveal melanoma. In spite of the widespread use of biopsies in general surgical practice, in ophthalmic oncology, indications and contraindications for biopsy continue to be under debate. The purpose of this paper is to critically evaluate the role of uveal melanoma biopsy in current clinical practice.

Objetivo: Describir las características ecográficas en los tumores vasoproliferativos del fondo de ojo (TVPFO). Métodos: Se estudiaron retrospectivamente las historias clínicas correspondientes a 8 pacientes afectados de TVPFO. Se analizaron los datos correspondientes a la exploración oftalmológica completa y a la exploración ecográfica. Resultados: Se encontraron masas nodulares afectando a la retina o bien a la retina y la coroides. La superficie tumoral era regular en 5 casos e irregular en los 3 restantes. La media de las dimensiones fue (mm/DE): base mayor, 7,14/2,56; base menor, 6,74/2,48 y altura 2,38/1,26. La estructura interna fue siempre sólida e irregular y la reflectividad era media-alta en 6 casos. No se detectó ni ángulo kappa ni vascularidad interna en ningún caso. Conclusión: De acuerdo con los resultados obtenidos se propone que tanto el diagnóstico como el diagnóstico diferencial de los TVPFO deben ser llevados a cabo no solo con la oftalmoscopia sino también con la exploración ecográfica. Objective: The aim of this work is to describe the ultrasound features in vasoproliferative tumours of the ocular fundus (VPTOF). Methods: The medical records corresponding to eight patients were retrospectively studied. Clinical data from the complete ophthalmologic examination and ultrasonographic findings were analysed. Results: Nodular masses affecting either the retina or both the retina and the choroid were found. The surface contour of the tumour was regular in 5, and irregular in 3 cases. In terms of dimensions (mm/ SD), the average major base was 7.14/2.56; the minor base 6.74/2.48 and the height 2.38/1.26. Internal structure was always solid and irregular and reflectivity was mostly medium to high in 6 eyes. Angle kappa was not present in any case. No vascularity signs were detected. Conclusion: According to the results it is suggested that when a differential diagnosis of VPTOF is carried out, not only should ophthalmoscopic signs be studied, but an ultrasound examination should also be done.

Daniel Kook*, Thomas C Kreutzer*, Armin Wolf, Christos HaritoglouDepartment of Ophthalmology, Ludwig-Maximilians University, Munich, Germany*These authors contributed equallyBackground: The purpose of this study was to evaluate the applicability and variability of echographic imaging using 10 mHz and high-resolution 20 mHz B scanning for measurement of intraocular tumors.Methods: This prospective consecutive study comprises 27 eyes in 27 patients with uveal melanoma. Each patient was examined by three experienced examiners independently on three occasions within a two-week time frame in a blinded manner. The height of the lesion was measured by all examiners using the 10 mHz B, 20 mHz B, and 8 mHz A scan probes. Additionally, basal diameter was examined using the 10 and 20 mHz B scan.Results: Tumor height measurements for all examiners using the standardized A scan tended to be higher than for both B scan measurements. Statistical analysis revealed significant differences in tumor height between B and A scan measurements. No difference in tumor height was found between the two B scan techniques (P = 0.239). Basal tumor diameter measurements revealed significant differences between 10 mHz and 20 mHz B scans (P < 0.001 and P = 0.001, respectively). For the 10 mHz B scan, basal diameter results tended to be larger than for the 20 mHz B scan. No difference was found for interobserver variation in all A scan and B scan examinations. The mean standard deviation of the difference in tumor height measurements between the examiners was ±0.24 mm for the 8 mHz A scan, ±0.46 mm for the 10 mHz B scan, and ±0.42 mm for the 20 mHz B scan. Both the 10 mHz and more precise 20 mHz B scan evaluations underestimated tumor height.Conclusion: The 20 mHz ultrasound probe, despite its theoretically higher resolution, is not able to replace A scan measurements of tumor height.Keywords: standardized echography, 20 mHz, B scan, A scan, intraocular tumor

Introduction: Intra-arterial chemotherapy (chemosurgery) for the treatment of retinoblastoma has been performed more than 1600 times (more than 1400 times in Japan and 200 times in New York) over the past 20 years. Despite this treatment's success some eyes cannot be saved and require enucleation. Here we report the histopathologic findings of the remaining intraocular tumor of eyes that were enucleated following treatment that included chemosurgery in New York City. Materials and Methodology: Independent histopathologic review of the enucleated eyes was correlated with the clinical findings that prompted enucleation. Results: Between May 1, 2006 and April 30, 2009, 56 eyes received chemosurgery at our institution, and 10 of these were enucleated subsequently. All were Reese Ellsworth Group 5 at enucleation. Of the 21 eyes that were treated with chemosurgery as the primary treatment, 1 (5%) was enucleated subsequently; its histopathology revealed residual non- necrotic, non-calcified tumor. Of the 34 eyes treated with chemosurgery after other treatments, 9 (24%) were enucleated, and 5 of these eyes contained non-calcified, non-necrotic tumor. None was enucleated for complications of chemosurgery. All patients were alive and free of metastatic disease as of September 2009. Conclusions: A significant number of eyes with advanced intraocular retinoblastoma avoided enucleation as a result of chemosurgery. The rate of eyes that were enucleated was higher when chemosurgery was the secondary rather than the primary treatment. Of the eight eyes enucleated for progressive disease six had non-necrotic, non-calcified tumor cells.

A system has been developed to determine intraocular tumor volume, a characteristic that has been estimated in previous studies from linear dimensions using a variety of models. Volume was determined by tracing tumor boundaries in sequential, parallel ultrasound scans, adding the areas and multiplying by the interslice interval. The in vitro accuracy of the technique was within +/- 2%. The mean difference between volumes determined from replicate in vivo scans of intraocular tumors was 4.3%. Serial scan volumes were, on average, 19% smaller than volumes computed from an ellipsoidal model and 13% smaller than area-rotational volumes. Differences of as much as 50% were observed between serial scan volumes and volumes computed with these models. The results indicate that methods based on either linear measurements or the tumor area in a single cross-section will tend to both systematically overestimate tumor volume and suffer from unpredictable, nonsystematic errors.

PURPOSE: To describe the first case of intraocular teratoma associated with eyelid coloboma and the second reported case of intraocular teratoma. DESIGN: Interventional case report. METHODS: A left intraocular tumor was surgically resected from a 2-day-old female with an associated lower eyelid coloboma. RESULTS: Pathologic evaluation revealed a completely intraocular tumor comprising derivatives of all three germ cell layers giving a diagnosis of intraocular teratoma. The eyelid coloboma was repaired, and a scleral-wrapped hydoxyapatite-integrated orbital implant was placed. CONCLUSION: To our knowledge, this is the second reported instance of teratoma originating within the globe and the only reported case of teratoma associated with eyelid coloboma. Although exceedingly rare, intraocular teratoma should be added to the differential diagnosis of congenital intraocular tumors.

Lasers are used extensively in ophthalmology for a variety of conditions, including many choroidal and retinal tumors. With technologic advances, current therapy attempts not only to maximize survival with globe-salvaging treatment, but also to preserve vision. Each neoplasm has different indications for primary and adjuvant therapy, as well as differing laser treatment protocols. Additionally, there are numerous laser applications available for use, including laser photocoagulation, transpupillary thermotherapy (TTT), and photodynamic therapy (PDT). The current review outlines the basic principles of laser treatment for intraocular tumors, focusing on the indications, treatment protocols, efficacy, and safety, while also presenting the latest advances in intraocular tumor treatment.

Retinoblastoma, a malignant intraocular tumor of infancy and childhood, originates from primitive embryonal retinal cells. The cause of retinoblastoma is the loss or deletion of genetic information from the long arm of chromosome 13. Retinoblastoma has a hereditary form characterized by bilateral and multifocal tumors, and a nonheritable form that presents with a single tumor.

Among the 934 patients with a choroidal melanoma treated in first intention with an accelerated proton beam since 1984, 41 (4.4%) presented an extrascleral extension, of which 11 were located anteriorly and 30 posteriorly. For these 30 cases, the diagnosis was made by B ultrasonography only when the volume of the extension was important, between 20 and 2280 mm3. The Cox model multi-variant analysis was used to study the statistically significant parameters in the development of an extrascleral extension. It appears that the maximal diameter of the intraocular tumor (p = 0.0012), the localisation of the anterior margin (p = 0.0304) and the age of the patients (p = 0.0097) are statistically meaningful. The 4 years' survival, studied with the Kaplan-Meier curves, is estimated at 60% for the patients with extrascleral extension and at 85% for the patients without. These results are similar to those of literature, whatever treatment is chosen (enucleation and radiotherapy, exenteration).

Purpose To analyze cytogenetic profile of locally invasive posterior uveal melanoma (UM). Methods Twenty consecutive cases of large posterior UM with histopathologically confirmed extrascleral extension were included in this non-comparative cases series. Fine needle aspiration biopsy (FNAB) of the intraocular tumor portion was performed using 25-gauge trans-scleral approach, immediately after enucleation of the globe. FNAB of the extrascleral tumor portion was also performed when it was > 1mm in thickness. Sampled material underwent fluorescence in situ hybridization (FISH). Follow-up was longer than 12 months. Results Six tumors (30%) had both intraocular and extraocular tumor samplings, whereas 14 tumors (70%) showed extrascleral extension less than 1 mm in thickness. Monosomy 3 was found in 5 tumors (25%), whereas disomy 3 in 15 tumors (75%). Cytogenetic profile of the intraocular tumor portion appears to be maintained in the extrascleral extension in all cases (100%). Five patients (25%) developed metastatic disease during follow-up (all had monosomy 3 tumors). Conclusion Extrascleral extension appears more frequent in disomy 3 tumors. Cytogenetic profile of locally invasive posterior UM is maintained in the extrascleral tumor portion and must be considered the most important prognostic factor in locally invasive tumors.

■ Intraocular tumors are conventionally diagnosed with noninvasive techniques, namely, clinical characteristics, angiographic and ultrasonographic features. ■ Rarely, intraocular masses require histopathology to increase diagnostic yield. ■ Results from intraocular biopsy can lead to revision of the therapeutic plan. ■ A variety of techniques exist to obtain intraocular tissue, most commonly fine-needle aspiration biopsy (FNAB). ■ Vitrectomy-based biopsy techniques utilizing a 20-gauge aspiration cutter have been performed in an effort to increase the diagnostic yield compared to FNAB, but at the expense of increased complications. ■ Intraocular biopsy based on a 25-gauge aspiration cutter is explored, with the intent of providing the safety benefits of FNAB and the potential benefit of increased diagnostic yield of vitrectomy. ■ Biopsy with 25-gauge aspiration cutter can be performed for anterior segment and posterior segment indeterminate lesions. ■ Albeit with limited follow-up and small number of cases, biopsy with the 25-gauge aspiration cutter did yield adequate amounts of specimen for histopathologic diagnosis, with no significant complications.

Retinoblastoma is a malignant intraocular tumor of childhood that requires accurate diagnosis and prompt treatment. It is well known that several other ocular conditions of childhood can clinically simulate retinoblastoma. Knowledge of the clinical features that serve to differentiate retinoblastoma from simulating lesions may assist the clinician in arriving at the correct diagnosis and preventing misdirected therapy. This review provides a simple classification of those pseudoretinoblastomas and reviews the recent literature on this subject. It emphasizes the clinical features that help differentiate pseudoretinoblastomas from true retinoblastoma.

Orbital and Periorbital Complications of Proton Beam Radiation Therapy for Intraocular Tumor Y.J. Choi, S. Kim, H.K. Choung, and Y.S. Yu; Department of Ophthalmology, Kangdong Sacred Heart Hospital, Hallym University Medical Center, Seoul, South Korea, Department of Radiation Oncology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, South Korea, Department of Ophthalmology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, South Korea, Department of Ophthalmology, College of Medicine, Seoul National University, Seoul, Korea, Seoul, South Korea, Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea, Seoul, South Korea

10068 Background: Retinoblastoma (Rb) is the most common intraocular tumor in childhood. With current modalities, cure rates are high and hence number of survivors is increasing. However, data on q...

e20054 Background: Uveal melanoma is the most common primary intraocular tumor in adults, median age at diagnosis of 60 years. Despite a growing understanding of the molecular characteristics of uv...

We recently demonstrated that splenectomy restores an interaction between CD8+ T cells and macrophages necessary for intraocular tumor elimination. Taking into consideration other studies indicating that intraocular tumor growth does not induce tumor-specific CD8+ T-cell tolerance, our data suggest that splenectomy may influence the phenotype of tumor-associated macrophages.

Microscopic examination of the surgical margins of an en bloc resection of an intraocular tumor is difficult because of the small size of the specimen. A technique to study the surgical margins of en bloc resections is described that minimizes gross handling of the tissue in the laboratory. Step sections at 1/8-mm intervals along the anteroposterior axis of the eye provide sufficient sampling to detect involvement of the surgical margin. They also allow easy anatomic reconstruction of the tumor with glass slides. [Ophthalmic Surg Lasers 1998;29:692-694.]

The optimal treatment of uveal melanoma would destroy the intraocular tumor, retain good vision, and avoid the development of metastatic disease. Unfortunately, inherent limitations make the development of such an optimal therapy unlikely in the near future.1 One, approximately two thirds of choroid

espanolObjetivo: Determinar las caracteristicas clinicopatologicas del melanoma uveal en pacientes mexicanos menores de treinta anos. Metodos: Se revisaron los casos del Servicio de Patologia Oftalmica con diagnostico histopatologico de melanoma uveal de 1957 a marzo de 2002. Se analizaron: edad, sexo, ojo afectado, diagnostico clinico, tamano, tipo celular, indice mitotico, infiltrado linfocitario, patron vascular, necrosis, invasion vascular y/o de estructuras intraoculares asi como extension orbitaria. Resultados: De 122 casos con diagnostico de melanoma uveal, 14 correspondieron a pacientes menores de 30 anos (11,4%). La edad promedio fue de 21,7 anos, con predominio en mujeres. El diagnostico clinico preoperatorio fue melanoma uveal en un 92,85 %, originandose en coroides en la mayoria de los casos. El tipo celular mas frecuente fue el de celulas fusiformes, con predominio de los patrones vasculares 3 y 4 de Folberg. Conclusiones: Nuestros resultados sugieren que los clinicos deben considerar el melanoma uveal como un diagnostico diferencial mas en pacientes menores de 30 anos con un tumor intraocular. Las caracteristicas histologicas observadas se han correlacionado con un mejor pronostico. EnglishPurpose: To analyze the clinicopathologic characteristics of uveal melanoma in Mexican patients under thirty years old. Methods: We reviewed the cases from the Service of Ophthalmic Pathology with histopathologic diagnosis of uveal melanoma from 1957 to March 2002. The data analyzed were: age, gender, affected eye, clinical diagnosis, size, cellular type, mitotic index, lymphocytic infiltrate, vascular pattern, necrosis, vascular and/or intraocular invasion and orbital extension. Results: We reviewed 122 cases diagnosed with uveal melanoma, and 14 patients were younger than 30 years old (11.4%). The mean age was 21.7 years, with female preponderance. Preoperative clinical diagnoses were uveal melanoma in 92.8%, most of the cases originated from choroid. The most frequent cellular type was spindle cells and 3 and 4 Folberg´s vascular patterns. Conclusions: Our results suggest that the clinicians should consider uveal melanoma more frequently as another differential diagnosis in patients under thirty years with an intraocular tumor. The histological characteristics found have been correlated with a better prognosis.

A leiomyoma of the iris is described in an 11-year-old Yorkshire Terrier. This is a rare primary intraocular tumor in dogs and we describe the clinical presentation, gross findings and histopathologic characteristics of this tumor. The diagnosis was made on the basis of light microscopy and immunohistochemical staining using antidesmin antibodies, which is specific for myogenic tissues. An unusual feature of the tumor was the presence of myxoid change. To our knowledge myxoid change has not been previously described in a primary intraocular leiomyoma.

In the first edition of this textbook, we stressed the controversies in management of melanomas of the ciliary body and choroid (posterior uveal melanoma). Many of the controversies have been largely resolved, and there continue to be new developments related to the management of patients with posterior uveal melanoma. Therefore, it seems appropriate in this chapter to provide a brief overview of the current options in management of this important intraocular tumor. Other authors will discuss the details of radiation therapy of uveal melanoma.

The most common primary intraocular tumor is uveal melanoma, with an incidence of 6 cases per million per year (Sphields And Shields 1992a). In the United States, it represents less than 0.2% of all cancers (American Cancer Society 1990).

Introduction: Massive retinal gliosis (MrG) is a rare, benign intraocular tumor that results from the proliferation of well-differentiated glial cells. MrGs develop because of a variety of causes. case report: We encountered a case of a 2-year-old male infant with loss of vision in the right eye. Enucleation was carried out due to an absolute glaucoma. Histologically, the vitreous body had been totally replaced by massively proliferated spindle cells, which had delicate fibrillary cytoplasm without nuclear atypia. Immunohistochemically, the cells were strongly positive for glial fibrillary acidic protein. these findings led to a diagnosis of massive gliosis. conclusion: Massive retinal gliosis (MrG) is a rare, benign intraocular tumor that results from the proliferation of well-differentiated glial cells. to our knowledge, this may be the first report of such an occurrence in a 2-yearold infant. It is important for ophthalmologists to be suspicious of a mass resistant to chemotherapy that resembles a rb in children.

An melanotic line of Bomirski Hamster Melanoma (BHM Ma) was implanted into the anterior chamber of the hamster eye and the ensuring vascular system was studied using scanning electron microscopy (SEM) of vascular corrosion casts. The tumor vasculature, induced mainly from the host iris vessels, showed generally disorganized and irregular patterns. Tortuous tumor capillaries of uneven contour with local dilatations and constrictions were drained by markedly dilated, thin-walled, venous vessels connected with the eye veins. Vascular sprouting and, less frequently, intussusceptive formation of new vessels were observed at the periphery and also within the tumor mass. The presence of numerous nodular outgrowths, varying in size, on the surface of dilated venules and venous vessels represent morphological evidence for continuous remodeling of the tumor vasculature. The observed features of the vascular system seem to provide a pathway for further tumor expansion. Our study showed that BHM Ma line, originating from an aggressive skin melanotic melanoma, implanted to the eye anterior chamber gave rise to rapidly growing tumors with the capability of inducing abundant vasculature which allows metastasis to the lungs, kidneys and regional lymph-nodes.

Ectopia lentis may be a feature of numerous systemic and ocular disorders. Kivela and Tarkkanen described an 8-year-old girl with medulloepithelioma who presented with ectopia lentis and a mass behind the temporal iris. Shields reported 2 children with medulloepithelioma who had ectopia lentis associated with neovascular glaucoma. To date, there has been no report of a child with ectopia lentis as the only presentation of an intraocular tumor. We present 2 children with malignant medulloepitheliomas who presented in this fashion.

We identified and treated a solid, growing fungal tumor mass in a patient with disseminated histoplasmosis. Although the most commonly reported intraocular lesions from disseminated histoplasmosis are areas of inactive chorioretinal scars or areas of localized subretinal neovascular membrane formation, a focus of active fungal growth needs to be ruled out in all such cases. When a solid tumor mass is identified, the most effective way of preserving vision is with systemic antifungal therapy.

Serum superoxide dismutase and catalase assays were performed using spectrophotometry in 60 adults and children with benign or maligrant tumors and in controls. There was a statistically significant difference in the antioxidative status of children with intraocular tumors (primary retinoblastoma) compared with children without tumors, The difference was not significant in adults. These enzymes may be of value in the early diagnosis of malignant intraocular tumor, especially retinoblastoma.

The combination of carboplatin and etoposide has been tested in intraocular retinoblastoma (IORB), in order to avoid enucleation or radiotherapy and to permit local therapy.

Retinoblastoma, the most common primary malignant intraocular tumor of childhood is a great success story in pediatric and ocular oncology. Pathology of retinoblastoma is important to guide the treatment modalities. Differentiated retinoblastoma is commonly seen in younger age group. Since a hundred years, we have been observing two typical true rosettes in retinoblastoma in the form of Flexner-Wintersteiner (FW) and Homer Wright (HW) rosettes and in many occasions pseudorosettes have been documented. In the present case report, a third new type of rosette was identified in a differentiated retinoblastoma which had an unusual anterior segment involvement.

Standardized echography can detect and differentiate intraocular tumors with a great accuracy. However, these tumors may present atypical characteristics. Here we describe a patient with a black mass on the skin of his nose, and retinal detachment due to a solid lesion in his left eye. The echographic characteristics of this lesion and the differential diagnosis with other tumors are discussed.

Primary pigment dispersion syndrome (PPDS) is a bilateral condition that occurs in anatomically predisposed individuals. PPDS may evolve into pigmentary glaucoma, but it is difficult to predict which patients will progress. Secondary pigment dispersion is more often unilateral and acquired as a result of surgery, trauma, or intraocular tumor, but can likewise lead to pigmentary glaucoma. We report two cases of patients with bilateral PPDS who developed secondary pigment dispersion and pigmentary glaucoma in one eye. Patients with PPDS who acquire a secondary mechanism of pigment dispersion may be at an increased risk of progression to pigmentary glaucoma, presumably due to an increased burden of liberated pigment. In addition to regular surveillance for progression to glaucoma from PPDS, secondary causes of pigmentary dispersion in these eyes should be considered when patients present with grossly asymmetric findings. When secondary pigment dispersion is identified in eyes with PPDS, we recommend prompt intervention to alleviate the cause of secondary pigment dispersion and/or aggressive control of intraocular pressure to limit glaucomatous damage.

The most common primary intraocular tumors are retinoblastoma in children and uveal melanoma in adults. Enucleation (removal of the eye) was the standard treatment until radiotherapy techniques were found to offer patients “eye and vision-sparing” alternatives (STALLARD 1962, 1966, 1968; ZIMMERMAN et al. 1978)

An algorithm has been developed for spectral analysis of backscatteredradiofrequency signals. It has been tested on a tissue model, a gelsuspension of calibrated latex spheres (between 20 and 100 μm in diameter),and on malignant intraocular tumours. The results so far appear promising regarding tissue differential diagnosis.

Two asymptomatic elderly women who underwent cataract extraction 7 or more years previously and with intraocular lens placement presented with a linear bead-like white multinodular mass in the inferior angle simulating iris metastasis versus large inflammatory precipitates. There was no iris infiltration. In the first case, the posterior lens capsule was intact and there was no evidence of gelatinous vitreous in the anterior chamber, whereas in the second case, the capsule was open and there was gelatinous vitreous prolapse. In both cases, there was asteroid hyalosis in the vitreous. Both patients were diagnosed with prolapsed vitreous asteroid hyalosis into the anterior chamber and managed with observation. Vitreous asteroid hyalosis can prolapse into the anterior chamber of pseudophakic elderly patients with or without capsular opening and can simulate an intraocular tumor.

The interest in evaluating the histological features of uveal lesions by non-invasive techniques raises mainly from two issues.

During the last decade, a number of globe-preserving methods have been introduced in the treatment of malignant choroidal melanoma; these include local tumor resection, photocoagulation, phototherapy with hematoporphyrins, diathermy, tumor irradiation, and most recently thermoradiotherapy (Augsburger et al. 1986; Char et al. 1983; Coleman et al. 1986; Finger et al. 1989; Foulds 1983; Gragoudas et al. 1987; Lommatzsch 1983; Packer 1987; Reyman et al. 1984; Riedel 1987; Riedel et al. 1985, 1988; Vogel 1972). Radiotherapeutic techniques to minimize the dose to uninvolved parts of the eye include brachytherapy by means of radioactive scleral plaques (cobalt 60, iodine 125, ruthenium 106) and external beam irradiation using helium ions or protons (Augsburger et al. 1986; Char et al. 1983; Gragoudas et al. 1987; Lommatzsch 1983; Packer 1987).

The differential diagnosis of endobulbar tumors has been performed up to now, prevalently by means of A-scan standardized echography. Thanks to our school, which recognized the use of technological development in regards to false colours, in 1984, today we have computerized B-scan equipment in false colours, with programs that give us the opportunity to perform frozen image elaborations. By means of such programs is possible to study the characteristics of reflectivity, the texture and the contours of the structures that we are examining. Furthermore such equipment is able to perform accurate measurements of dimensions and areas. The authors in this work report their experience on the possibility to perform the differential diagnosis of the principles of endobulbar tumors by means of B-scan in false colours and analyze their various echographical aspects.

Choroidal melanoma is the most common intraocular tumor and is uniquely suited for evaluation by magnetic resonance imaging (MRI) because of the paramagnetic effect of the melanin molecule. The authors performed T1-, T2-, and proton-density-weighted MRI on 34 patients with choroidal melanoma. Nineteen patients received gadolinium contrast, T1-weighted images were superior in both detecting and delineating tumors, showing increased contrast-to-noise ratios over other images. Gadolinium contrast further increased this ratio. These images are presented as evidence that gadolinium-enhanced MRI is valuable in the evaluation of choroidal melanoma.