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Fluid state of malignant choroidal melanoma growth presenting as exudative retinal detachment.

The extension of a malignant choroidal melanoma of an epithelioid cell type all through the subretinal space exhibiting a change of its solid state with its own stroma and blood supply in the choroid into a fluid growth phase resembling a tissue culture in the subretinal space is demonstrated in this case. This fluid state is represented by free-floating malignant melanoma cells living without their own stroma or direct blood supply in subretinal exudate. The transitional interface between the solid and fluid neoplastic states of this choroidal melanoma is demonstrated. The development of the fluid state indicates a potential of choroidal melanomas for isolation and survival of its cells without stroma and is related to the process of regional seeding and metastatic dispersion.

Choroid Neoplasms↗

[Ocular changes in patients with tuberous sclerosis].

The authors discussed ocular changes observed in 100 children with tuberous sclerosis. Retinal tumors, typical of this disease, were found in 19 patients (19%). This incidence was higher in older children. Three types of tumors were observed: a) flat or slightly elevated, indistinct, salmon-grey colour, b) whitish-yellow, mulberry-like, located in disc area, c) mixed or intermediate. The authors observed 4 stages of tumors' development: changes in distribution of pigment in retina, arising of semitranslucent, salmon-grey tumors, mineralization of tumors-so called intermediate type, mulberry-like type. Progress from one stage to the next one is slow.

Adolescent↗

[A case of von Hippel-Lindau disease with papillary capillary hemangioma treated by photodynamic therapy].

BACKGROUND: Laser photocoagulation of papillary hemangioma in von Hippel-Lindau disease often causes visual loss. Therefore, we applied photodynamic therapy for one patient. CASE: A 36-year-old male had 3.2 x 2.6 mm capillary hemangioma of the optic disc associated with a fibrovascular membrane and exudative retinal detachment involving the whole macular area in his left eye. His visual acuity was 0.08. TREATMENT: Two days after the intravenous injection of 2 mg/kg of hematoporphyrin derivatives, the eye was irradiated with a dye laser of 630 nm wavelength, with irradiance of 637 mW/cm2 and a radiant exposure of 150-250 J/cm2. The exudative retinal detachment and hemorrhage in the tumor increased from day 1, but the tumor and exudative changes began to resolve after 3 weeks. The fibrovascular membrane was removed by vitrectomy. Three years after the treatment, the tumor was completely resolved, but the patient's visual acuity was 0.1 due to atrophy of the optic nerve and pigment epithelium of the macula. CONCLUSION: Although further investigation on optimal radiance avoiding damage to the optic nerve is still needed, photodynamic therapy is a promising therapeutic option for papillary hemangioma.

Adult↗

Visual fields in metastatic choroidal carcinoma.

A large choroidal metastasis was diagnosed in the right eye of a 44-year-old man referred for admission to hospital because of 'retinal detachment.' At the same time in the second, symptomless, eye a very small metastasis was observed. Close follow-up during the next month showed an extremely rapid deterioration of visual acuity and visual field in this eye. This is thought to be characteristic of such metastatic tumours in contrast to the slower progress of choroidal malignant melanoma. The application of local radiotherapy to the same eye led to an impressive improvement in the visual acuity and visual field. The source of these bilateral choroidal metastases, which was found only after the patient's death, proved to a bronchial carcinoma.

Adult↗

A non-metastatic remote effect of lung carcinoma.

A 37-year-old man, who had an oat cell carcinoma of the left lung, lost in a few months a substantial part of both visual fields, on account of a destructive retinal process. This syndrome is known in the literature as a visual paraneoplastic syndrome, which clinically shows the very rapid development of a pseudoretinitis pigmentosa sine pigmento. For most of the patients described the visual problem was the presenting symptom. In contrast with most other described cases, our patient responded very well to systemic tumor treatment and his ocular process also came to a standstill after some delay. The nature of the relationship between the primary tumor and the photoreceptor destruction is not yet clear in detail. An auto-immune process probably forms the basis of the syndrome, but hormonal activity of the tumor could not be excluded in all cases.

Adult↗

Cadherin-10 is a novel blood-brain barrier adhesion molecule in human and mouse.

Maintenance of the specialised environment of the central nervous system requires barriers provided by the endothelium of brain microvessels (the blood-brain barrier (BBB)) or the epithelium lining the ventricles (CSF-brain barrier) or the choroid plexus (blood-CSF barrier). Inter-endothelial junctions are more extensive in the BBB than in other tissues, with elaborate tight junctions. However, few differences in the molecular composition of these junctions have been described. Here, we show, in both human and mouse brain, that the type II classical cadherin, cadherin-10, is expressed in BBB and retinal endothelia, but not in the leaky microvessels of brain circumventricular organs (CVO), or in those of non-CNS tissues. This expression pattern is distinct from, and reciprocal to, VE-cadherin, which is reduced or absent in tight cortical microvessels, but present in leaky CVO vessels. In CVO, the barrier function is switched from the microvasculature to the adjacent ventricular epithelium, which we also find to express cadherin-10. In the vessels of gliobastoma multiforme tumours, where BBB is lost, cadherin-10 is not detected. This demonstration of a distinctive expression pattern of cadherin-10 suggests that it has a pivotal role in the development and maintenance of brain barriers.

Animals↗

Mice lacking p27(Kip1) display increased body size, multiple organ hyperplasia, retinal dysplasia, and pituitary tumors.

SUMMARY: Mice lacking p27(Kip1) have been created by gene targeting in embryonic stem cells. These mice are larger than the control animals, with thymus, pituitary, and adrenal glands and gonadal organs exhibiting striking enlargement. CDK2 activity is elevated about 10-fold in p27(-/-) thymocytes. Development of ovarian follicles seems to be impaired, resulting in female sterility. Similar to mice with the Rb mutation, the p27(-/-) mice often develop pituitary tumors spontaneously. The retinas of the mutant mice show a disturbed organization of the normal cellular layer pattern. These findings indicate that p27(Kip1) acts to regulate the growth of a variety of cells. Unexpectedly, the cell cycle arrest mediated by TGFbeta, rapamycin, or contact inhibition remained intact in p27(-/-) cells, suggesting that p27(Kip1) is not required in these pathways.

Animals↗

Review of optical coherence tomography for intraocular tumors.

PURPOSE OF REVIEW: Optical coherence tomography has assumed an important role in the management of numerous ocular conditions. With regard to ocular oncology, optical coherence tomography can illustrate retinal changes overlying choroidal tumors. Some of these features include photoreceptor loss, intraretinal edema, and retinal thinning overlying choroidal nevus; fresh subretinal fluid with preservation of photoreceptors overlying choroidal melanoma; and intraretinal edema, retinoschisis, and retinal thinning overlying irradiated choroidal melanoma. RECENT FINDINGS: The optical coherence tomography features of tumors of the retinal pigment epithelium include typical findings of peaked vitreoretinal traction and retinal disorganization with combined hamartoma of the retina and retinal pigment epithelium, full-thickness retinal shadowing with congenital simple hamartoma, and photoreceptor loss and retinal thinning overlying congenital hypertrophy of the retinal pigment epithelium. SUMMARY: Optical coherence tomography of retinal tumors, such as retinoblastoma and astrocytic hamartoma, reveals full-thickness replacement of the retinal anatomic layers with the tumor and shadowing corresponding to the intralesional calcification. For all intraocular tumors, optical coherence tomography provides valuable information regarding the status of the retina and the retinal pigment epithelium and can be useful in ascertaining reasons for visual loss.

Choroid Neoplasms↗

Cytogenetic abnormalities in tumors of patients with von Hippel-Lindau disease.

Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that causes the development of benign and malignant tumors in several organ systems. Tumors causing significant morbidity include retinal angioma, cerebellar hemangioblastoma (CH), renal cell carcinoma (RCC), and pheochromocytoma (Pheo). Cytogenetic studies of tumors in VHL patients are rare. Cytogenetic findings in tumors from 12 patients with VHL disease, including four RCCs, three CHs, and five Pheos are presented. Three of the four RCC cases were abnormal. Monosomy 3 or a deletion of 3p was present in all three abnormal cases. Complete or partial trisomy of chromosome 5 was present in two cases. A deletion of 14q, trisomy 7, and a missing Y were each observed in one case. These findings indicate that a deletion of 3p may be a primary cytogenetic change in RCCs associated with VHL disease in addition to playing a role in sporadic RCC. Duplications of 5q and deletions of 14q may be important secondary changes in the progression of the malignant phenotype. No visible cytogenetic abnormalities were observed in the three CHs, or in four of the Pheos. One of the five Pheos was found to exhibit mosaic trisomy 7; its significance is unclear at the present time.

Adrenal Gland Neoplasms↗

Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium.

An ultrastructural study of a postmortem donor eye from a 24-year-old male patient with sex-linked retinitis pigmentosa showed abnormalities in all remaining cones and rods. Central foveal cones were reduced in number by about 50% and had shortened and severely distorted outer segments. Cones from the parafovea through the midperiphery gradually decreased in density and had no organized outer segments. In the far periphery, cones and rods had only slightly shortened outer segments. Photoreceptors equidistant from the fovea in all quadrants showed similar changes. The virtual absence of organized cone outer segments from the parafovea through the midperiphery was conspicuous in that this patient had full visual fields with large test lights 3 weeks prior to death. The pigment epithelium contained abnormally large numbers of melanolysosomes and few free melanin granules from the fovea through the midperiphery and few melanolysosomes and many free melanin granules in the far periphery. Whether or not these observations in the pigment epithelial cells represent a primary defect in this disease or reflect changes secondary to a defect in the photoreceptor cells remains to be defined.

Adolescent↗