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PubMed1990

[Clinical and pathologic characteristics of malignant lymphoma of the retina].

Two cases of malignant non-Hodgkin lymphoma of the retina and choroid with cerebral involvement are reported. In both cases, early infiltrates appear to have originated in the capillary layer of the choroid. Subsequently, Bruch's membrane was penetrated and tumorous infiltrates were found beneath the retinal pigment epithelium. The resulting reactions created a distinctive ophthalmoscopic picture, for which the term "leopardskin pattern" was coined. It may be helpful in early diagnosis, which with suitable therapy can prolong life.

Aged↗
PubMed1988

Surface coil magnetic resonance imaging of the orbit at 1.5T.

MRI of the orbit at 1.5-Tesla was performed in 5 healthy volunteers and 30 patients with a variety of pathological intraocular or intraconal conditions. Major advantages of MRI over CT include higher spatial resolution, higher tissue contrast, possibility of direct multiplanar imaging, absence of bony and dental artifacts, and of ionising radiation. Specific indications of MRI in orbital pathology concern differentiation of uveal melanoma from subretinal haemorrhage, identification of lesions of the orbital apex, the orbital fissure or the optic canal, differentiation of inflammatory pseudotumour from malignant lesions, determination of the posterior extension of lesions of the optic nerve, and detection of abnormal flow in normal or hypertrophic intraorbital vessels. Limitations are due to motion artifacts on T2 weighted sequences, less accurate visualisation of calcification, poor specificity of some findings, and absence of signal from cortical bone.

Cranial Nerve Neoplasms↗
PubMed2005

Age-specific incidence of inherited versus sporadic cancers: a test of the multistage theory of carcinogenesis.

Knudson [Knudson, A. G. (1971) Proc. Natl. Acad. Sci. USA 68, 820-823] suggested that progression of retinoblastoma follows from two mutational events. Individuals who inherit one mutated gene copy should follow an age-onset pattern set by only a single rate-limiting step for transformation, whereas normal individuals should follow an age-onset pattern set by two rate-limiting events. Knudson's analysis of inherited and sporadic cases of retinoblastoma supported this prediction. However, retinoblastoma has a peculiar age-onset pattern concentrated in early life, because the retinal tissue completes most of its cell division by 5 years of age. Here, I compare age-specific incidences of inherited and sporadic forms of colon cancer, a much more typical form of human cancer. My simple mathematical analysis based on multistage theory explains the observed differences in age-onset patterns between inherited and sporadic cases. I also analyze recent retinoblastoma data and provide a mathematical analysis and interpretation. My analysis supports Knudson's two-hit theory but is much simpler and easier to understand than the original mathematical theory, which was based on a complicated model of cell division in the retina. My simpler theory for retinoblastoma makes clear the common basis for understanding multistage progression in tissues as different as the retina and colon.

Adult↗
PubMed1988

Some retinoblastomas, osteosarcomas, and soft tissue sarcomas may share a common etiology.

DNA and RNA were extracted from primary human osteosarcomas and soft tissue sarcomas obtained from patients without retinoblastoma and were analyzed by hybridization with a cDNA probe for RB mRNA; absence or alterations of the RB gene are associated with development of retinoblastoma. Most of the osteosarcomas or soft tissue sarcomas examined by us did not express detectable levels of RB mRNA, whereas normal cells and epithelial tumor cells did. One osteosarcoma expressed a 2.4-kilobase transcript in addition to a normal 4.7-kilobase species. Our data suggest that transcriptional inactivation or post-transcriptional down-regulation of the RB gene may be important in the etiology of some osteosarcomas and soft tissue sarcomas as well as retinoblastomas.

Bone Neoplasms↗
PubMed1993

Pigment epithelium-derived factor: neurotrophic activity and identification as a member of the serine protease inhibitor gene family.

Cultured pigment epithelial cells of the fetal human retina secrete a protein, pigment epithelium-derived factor (PEDF), that induces a neuronal phenotype in cultured human retinoblastoma cells. Morphological changes include the induction of an extensive neurite meshwork and the establishment of corona-like cellular aggregates surrounding a central lumen. The differentiated cells also show increases in the expression of neuron-specific enolase and the 200-kDa neurofilament subunit. Amino acid and DNA sequence data demonstrate that PEDF belongs to the serine protease inhibitor (serpin) family. The PEDF gene contains a typical signal-peptide sequence, initiator methionine codon, and polyadenylylation signal and matches the size of other members of the serpin superfamily (e.g., alpha 1-antitrypsin). It lacks homology, however, at the putative serpin reactive center. Thus, PEDF could exert a paracrine effect in the embryonic retina, influencing neuronal differentiation by a mechanism that does not involve classic inhibition of serine protease activity.

Amino Acid Sequence↗
PubMed2004

HLA expression in choroidal melanomas: correlation with clinicopathological features.

PURPOSE: In this retrospective study, we analyzed the relevance of human leukocyte antigen (HLA) expression to clinical behavior of uveal melanoma and correlated it with conventional light microscopic parameters. METHODS: HLA class I antigen and Beta 2 microglobulin expression were analyzed in 45 primary choroidal melanoma lesions by immunoperoxidase staining with monoclonal antibodies to HLA class I antigen and beta 2 microglobulin and correlated with the known clinicopathological parameters. Immunoanalysis was done by a semi quantitative method. The tumors were divided into 3 groups. Group A: Tumors with no extrascleral extension/no liver metastases, group B: tumors with only extrascleral extension and group C: tumors with liver metastases. For statistical analysis we analyzed the negative, weak (heterogeneous) and the positive expression of HLA and beta 2 microglobulin with known clinicopathological parameters. RESULTS: In-group A (n = 35) tumors with no extrascleral extension and no liver metastases HLA class I antigen and beta 2 microglobulin are negative (absent staining) in 30 choroidal melanomas. In-group B (n = 4) they are weak (heterogeneous) in 3 tumors. In-group C (n = 6) all the 6 tumors have a positive (bright staining) immunoexpression. No statistical significance was obtained when HLA and beta 2 microglobulin immunoreactivity was compared against largest tumor diameter (LTD), tumor infiltrating lymphocytes (TIL), mitosis and nuclear grade. The difference of HLA class I and beta 2-microglobulin imunoreactivity among the various cell types spindle, mixed and epithelioid was statistically significant (p = 0.003), (p = 0.004). The difference in immunoreactivity between tumors with no liver metastases and tumors with liver metastases was statistically significant (p < 0.001). CONCLUSIONS: HLA class I antigen and beta 2 microglobulin are negative in melanomas with no extrascleral extension and liver metastases and weak in melanomas with extrascleral extension and are positive in melanomas with liver metastases. HLA expression is independent of the conventional markers in uveal melanoma.

Adolescent↗
PubMed1987

Samarium-145: a new brachytherapy source.

A new radiation source has been produced for brachytherapy, with radiation energies slightly above those of 125I, and a T1/2 of 340 d. This source, 145Sm, is produced by neutron irradiation of 144Sm (96.5% enriched). Decay is by electron capture with 140 K x-rays per 100 disintegrations in the energy region between 38-45 keV, plus 13 gamma-rays at 61 keV. These sources are encapsulated in Ti tubes, approximately 0.8 mm X 4.5 mm, and have been developed for temporary implantation in brain and ocular tumours. The 38-61 keV photons should make such sources easy to shield, while providing a dose distribution from source arrays somewhat more homogeneous than that from 125I. In addition, the 340 d half life of 145Sm permits its use for times significantly longer than that of 60 d 125I. While the 145Sm sources have been designed primarily for implantation in a brain tumour, they should be useful for almost any conventional brachytherapy application.

Brachytherapy↗
PubMed1993

Nucleolar organizer regions in optic gliomas.

The biological nature of optic gliomas is controversial, with some considering them benign hamartomatous lesions, and others believing them to be true neoplasms. We evaluated the use of colloid silver impregnation of nucleolar organizer region-associated proteins (AgNORs) in making this distinction. Thirty-one optic gliomas, 14 optic nerve meningiomas, and a single case of giant cell glioblastoma multiforme (monstrocellular glioma) of the optic chiasm were stained for AgNORs and counted in a masked fashion. The optic gliomas contained 2.01 +/- 0.09 AgNORs per nucleus, similar to that of optic nerve meningiomas (2.15 +/- 0.15) and our previously reported counts for diffuse fibrillary astrocytoma (2.22 +/- 0.10), and significantly more than that of reactive astrocytosis (1.18 +/- 0.02). Six of the seven optic gliomas examined had compound AgNORs, a feature associated with malignancy in other tumour types. AgNOR counts did not correlate with clinical features, including those seen during the post-operation course. These data suggest that optic gliomas may be true neoplasms, and not benign hamartomas.

Adolescent↗
PubMed1989

Pediatric oncology: model tumors of unparalleled import.

Molecular studies of tumors arising during childhood have provided insights important for our understanding of the genetic and cellular events that now seem likely to mediate the development of many different malignancies. Of particular interest have been recent studies using recombinant DNA technology to study the pressure genetic alterations now thought to be central features of oncogenesis. Oncogenes and recessive cancer genes, first recognized to be of clinical importance during the study of Burkitt's lymphoma and retinoblastoma, are now thought to play a role in the development of most, if not all, tumors. Studies to identify more effective approaches to cancer prevention, detection, staging, and treatment are now seeking to build upon an understanding of those genetic alterations. It can be expected that pediatric oncology will once again play a pivotal role as these studies mature into clinical trials.

Child↗
PubMed1995

Pathology of ocular melanomas.

Primary ocular melanomas usually arise in the uvea, in the choroid and ciliary body. They metastasize primarily and initially exclusively, to the liver. Metastasis and survival is determined by the maximum tumour dimension, the number of epithelioid cells present within the tumour, vascular patterns within the tumour and nucleolar size and activity. Ganglioside and integrin profiles differ from cutaneous melanomas. Iris melanocytic lesions tend not to metastasize, most being naevi of varying degrees of aggressiveness which may cause glaucoma and corneal decompensation. Conjunctival melanoma is a rare unilateral tumour arising either in primary acquired melanosis or de novo rather than within a naevus. Survival of the patient depends on the location of the tumour and the histological subtype. Tumours not arising in the bulbar or limbal conjunctiva have a much poorer prognosis as do eyelid (cutaneous) melanomas if they involve the lid margin.

Conjunctival Neoplasms↗
PubMed1988

Eye socket and eyelid reconstruction using the combined island frontal flap and retroauricular island flap: a preliminary report.

One-stage eye socket and eyelid reconstruction was performed, with relatively satisfactory results, on a patient with a broad forehead. This was done after the complete exenteration of the orbit, using an island frontal flap and a retroauricular island flap with the common superficial temporal vessels. A Foley urethral catheter and a continuous suction drainage tube were effective for good wound healing between the bony orbit and a skin bag, made with a frontal flap, which was hidden in the orbital cavity. On the eighth postoperative day, the skin incision producing the false palpebral fissure was made, and it was enlarged slightly thereafter. A nylon implant was inserted into the orbital apex and cartilage grafts from the auricle to the false upper and lower eyelids were performed secondarily. The fundamental consideration of the eye socket and facial coverage corresponding to the eyelids and the possibility of modification of the author's method are briefly discussed.

Aged↗
PubMed1989

Eye socket reconstruction with free radial forearm flap.

Deformity of the orbital region and contraction of the eye socket were encountered in 3 patients who in their infancy underwent exenteration of the orbit and postoperative irradiation for the treatment of retinoblastoma. These major problems were attributed to the less-vascularized cicatricial conjunctiva left in place. To solve these disadvantages, a microvascular technique using a free radial forearm flap was adopted. A large permanent eye socket was achieved and depression deformity of the orbital region was corrected in a one-stage operation. This is, to our knowledge, the first report on the use of a free vascularized skin flap for "malignant contracture" of an anophthalmic eye socket. The excellent cosmetic results of this method are demonstrated.

Adolescent↗
PubMed1989

Fibrous capsule from extruded implant used as graft material in cul-de-sac reconstruction.

A 74-year-old woman underwent radiation therapy for ocular malignant melanoma. The eye was subsequently enucleated. Three years later, implant migration and conjunctival contracture necessitated a socket reconstruction. The fibrous capsule that formed around the migrated implant proved to be an easily harvested and effective tissue for expanding the contracted socket.

Aged↗