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Cytomegalovirus retinitis and low-grade non-Hodgkin's lymphoma: case report and review of the literature.

Patients with non-Hodgkin's lymphoma may develop retinal or choroidal lesions during the course of their disease. New immunosuppressive therapies currently used in reticuloendothelial malignancies have increased the incidence of opportunistic infections in this patient population. The differentiation of lymphomatous infiltration from opportunistic infection as a cause of chorioretinal infiltrates is critical, as the treatments are fundamentally different. We report a case of a patient with non-Hodgkin's lymphoma who developed a chorioretinal infiltrate that was initially thought to represent progressive disease. The patient received radiation treatment appropriate for intraocular lymphoma. The lesion progressed further and after reevaluation a diagnosis of cytomegalovirus retinitis was made and therapy initiated. Review of the literature for intraocular lymphoma and cytomegalovirus retinitis is provided and diagnostic strategies are described. We recommend analysis of intraocular fluid when there is difficulty in clinically differentiating intraocular lymphoma from opportunistic infection.

Antiviral Agents↗

Isolated metastases to the retina or optic nerve.

Isolated metastases to the retina or optic nerve are extremely rare. Retinal metastases arise from cutaneous malignant melanoma and carcinomas (lung, gastrointestinal, genitourinary, and breast). Most patients present with reduced vision. The clinical appearance of these metastases is variable. Melanoma metastases are brown or black, whereas carcinoma metastases are white. Frequently, retinal hemorrhages and exudates are seen. Optic nerve metastases arise from carcinomas (lung, breast, and gastrointestinal) in adults and acute leukemias in children. As is true of retinal metastases, most patients present with reduced vision. Though in most cases there is a visible optic nerve mass, some patients have optic disc edema only. Investigations are directed by a known history of malignancy in many patients and by physical examination. Cytological workup of vitreous may be required. Treatment options include observation, radiotherapy in eyes with visual potential, and enucleation for pain control. The visual prognosis is poor. In patients with retinal or optic nerve metastases, median survival is approximately 9 months after the onset of visual symptoms, but is slightly longer in cases in which the primary lesion is breast carcinoma.

Adult↗

The search for the retinoblastoma cell of origin.

The cellular effects of the genetic defects associated with tumorigenesis are context dependent. To better understand the reasons that different cell types require distinct combinations of mutations to form tumours, it is essential to identify and characterize a tumour's 'cell of origin'. Retinoblastoma, a rare childhood cancer of the retina that is caused by RB inactivation, is a good model in which to search for a tumour cell of origin, because retinal development is well understood and the initiating genetic lesion is well characterized. Identifying the cell of origin for this tumour would advance our understanding of how cellular context affects the requirement of specific mutations for cancer initiation and progression.

Animals↗

Variations in the presentation of primary intraocular lymphoma: case reports and a review.

Primary intraocular lymphoma is a distinct subset of primary non-Hodgkin's lymphoma of the central nervous system (CNS). Diagnosis can be difficult and is often delayed, as the clinical presentation can mimic a number of other ocular conditions. This report describes four different presentations of intraocular lymphoma and focuses on its modes of clinical presentation. Primary intraocular lymphoma can present with a wide variety of manifestations frequently mimicking diffuse uveitis that is refractory to corticosteroids. Subretinal pigment epithelium tumors may be seen. However, other presentations may include multiple deep white dots in the retina secondary to tumor infiltration; retinal infiltration, causing a necrotizing retinitis; or infiltration of the retinal vasculature, causing arterial or venous obstruction. Finally, optic nerve invasion may be seen. CNS lymphoma develops in the majority of patients before, in conjunction with, or after the development of eye disease. Intraocular lymphoma often has a fatal outcome, but recognition of its modes of presentation facilitates early diagnosis and treatment that may improve prognosis.

Adult↗

T1 Gd-enhanced compared with CISS sequences in retinoblastoma: superiority of T1 sequences in evaluation of tumour extension.

BACKGROUND: As adequate therapy for retinoblastoma in young children depends on infiltration of extra-retinal structures, diagnostic modalities play an essential role. METHODS: In this widely extended study, 80 children with retinoblastoma were studied with MRI (standard fat-suppressed Gd-enhanced T1, T2 thin-slice sequences (additionally with small loop surface coil), constructive interference in steady state (CISS) sequence covering the orbita). The images were analysed by two blinded neuroradiologists. Histology was used as the gold standard. RESULTS: MRI assumed infiltration of extra-retinal structures in 13 of 80 patients of which ten were confirmed by histology. Affected extra-retinal structures were: optic nerve (five, of which two were on CISS and three on T1 with higher image resolution using the surface coil), scleral infiltration (five, of which four on CISS and T1) and ciliary body infiltration (one on CISS and T1). Another 61 enucleated patients did not have any extra-retinal infiltration in histology. The CISS sequence with multiplanar reconstruction was mainly helpful in revealing exact three-dimensional tumour extension with excellent clinical acceptance and pre-surgical planning but T1 fat-suppressed Gd-enhanced images were superior in revealing exact tumour extension. CONCLUSION: CISS sequences allow to produce excellent anatomical images and to perform multiplanar reconstruction to better demonstrate tumour extension. However, T1-weighted sequences after contrast application are more sensitive (60 versus 40%) in detecting infiltration of the optic nerve but equal in detecting scleral infiltration.

Child↗

Detection of optic nerve involvement in retinoblastoma with enhanced computed tomography.

PURPOSE: The study was designed to evaluate the value of enhanced computed tomography (CT) as a non-invasive test in the detection of optic nerve invasion in retinoblastoma. METHODS: Nineteen eyes in 17 consecutive retinoblastoma patients underwent CT studies performed with high spatial resolution (512 x 512) and 1.5 mm sections, both with enhancement and without enhancement. If the central retinal vessels (CRV) were subjectively visualised with intravenous enhancement, the optic nerve was considered to be free of retinoblastoma. Nineteen enucleated globes were processed for histopathology, and the optic disc and nerve examined with light microscopy for the presence or absence of tumour and the level of involvement. RESULTS: The correlation between the visualisation of CRV and the presence or absence of optic nerve involvement histopathologically was found to be highly significant (p = 0.0006, Fisher exact test). CONCLUSION: In high spatial resolution CT with enhancement and 1.5 mm or thinner sections, non-visualisation of the CRV appears to be a reliable indicator of optic nerve invasion with retinoblastoma.

Child↗

Evaluation of orbital and eye lesions by radiographic examination, ultrasound, and computerized tomography.

This article examines three imaging procedures for the evaluation of orbital and eye lesions. In the radiographic section we discuss the normal anatomy, the projections to show the anatomy, and the interpretation of bone changes caused by benign and malignant lesions. In the next section we describe the equipment employed in the ultrasonic evaluation of the eye and orbit and the principles applied in the diagnosis of lesions of the eye and orbit by ultrasound. We introduce the CT section by explaining the methodology of computerized tomography of the orbit. The criteria for evaluating lesions of the eye and orbit are presented with appropriate cases for illustration. The discussion and summary detail the relative merit of these imaging procedures ad their usefulness in the diagnosis work-up of pathological conditions of the eye and orbit.

Adult↗