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Journal of AAPOS : the Official... Aug 2017Whereas most retinoblastomas are seen as intraocular tumors, the diffuse infiltrating type is distinguished by a horizontal growth along the retina with minimal vertical... (Review)
Review
Whereas most retinoblastomas are seen as intraocular tumors, the diffuse infiltrating type is distinguished by a horizontal growth along the retina with minimal vertical growth. These findings can resemble other entities and present as a diagnostic challenge. Fluorescein angiography (FA) features of retinoblastoma have been described in the literature; however, to our knowledge, little information is available on the diffuse type. We present FA findings and the clinicopathlogic correlation of 2 patients with diffuse retinoblastoma. Changes observed on FA correlate with the vascular changes observed on histopathology and can therefore be helpful in the diagnosis of atypical retinoblastomas when the clinical presentation is equivocal.
Topics: Adolescent; Calcinosis; Child, Preschool; Female; Fluorescein Angiography; Humans; Male; Retinal Neoplasms; Retinoblastoma
PubMed: 28694215
DOI: 10.1016/j.jaapos.2017.03.013 -
Archives of Ophthalmology (Chicago,... Jun 1995To apply modern techniques of molecular cell biology and to revisit the old question of the cell of origin for retinoblastoma in hopes of gaining a better understanding...
OBJECTIVES
To apply modern techniques of molecular cell biology and to revisit the old question of the cell of origin for retinoblastoma in hopes of gaining a better understanding of the retinoblastoma gene's antioncogenic mechanisms.
METHODS
Twenty-two consecutively accessed retinoblastomas were examined with immunocytochemical techniques for numerous retinal proteins. Both single and double labeling were used. Enzyme histochemistry for carbonic anhydrase was used as well.
RESULTS
Differentiated areas of the tumors contained abundant Müllerlike cells. Fleurettes stained mostly for red and green cone-specific antibodies while features of blue cones and rods predominated in areas with high cytoplasmic-to-nuclear ratios but no fleurettes. All of the differentiated neoplastic cells were either photoreceptors or Müller's cells. No other retinal cell types were found.
CONCLUSIONS
The cells of retinoblastoma are capable only of bipotential differentiation, ie, Müller's cells and photoreceptors. Given this and recent findings concerning retinal embryogenesis, we argue for the rod photoreceptor as the cell of origin. A possible role for the retinoblastoma gene product is discussed.
Topics: Cell Differentiation; Eye Neoplasms; Eye Proteins; Fluorescent Antibody Technique; Humans; Immunoenzyme Techniques; Nerve Tissue Proteins; Neuroglia; Photoreceptor Cells; Retina; Retinoblastoma
PubMed: 7786223
DOI: 10.1001/archopht.1995.01100060117046 -
Ophthalmic Paediatrics and Genetics Mar 1989Various studies which relate to the immunohistochemical identification of neuronal, glial, carbohydrate and nucleic acid associated antigens in retinoblastoma will be... (Review)
Review
Various studies which relate to the immunohistochemical identification of neuronal, glial, carbohydrate and nucleic acid associated antigens in retinoblastoma will be reviewed. The majority of these studies appear to support the concept that retinoblastomas arise, in situ, from neuron committed cells and in some cases specifically into photoreceptor-like cells. In selected cases, however, glial cell differentiation may be a feature of the tumor. In addition, the molecular biology of the retinoblastoma gene and the immunohistochemical characterization of its gene product will be discussed.
Topics: Antibodies, Monoclonal; Antigens, Neoplasm; Eye Neoplasms; Humans; Immunohistochemistry; Retinoblastoma
PubMed: 2662094
DOI: 10.3109/13816818909083770 -
La Revue Du Praticien Nov 1993Retinoblastoma is the most frequent malignant tumour of the eye in the child; the median age at diagnosis is two years. A model of carcinogenesis by two successive...
Retinoblastoma is the most frequent malignant tumour of the eye in the child; the median age at diagnosis is two years. A model of carcinogenesis by two successive mutations has been devised for this tumour and explains the two forms of the disease, hereditary and non-hereditary. It is most often manifested by a whitish pupillary reflection (leukocoria) or internal strabismus. Funduscopy using general anaesthesia is used to confirm diagnosis and assess endo-ocular extension. When the disease is confined to the retina, local treatment (enucleation, radiation therapy) leads to cure in a very large majority of cases. When diagnosis is made early, particularly as in familial cases, conservative treatment is possible. In intra- and extra-retinal disease, chemotherapy and radiation therapy can improve prognosis.
Topics: Child, Preschool; Eye Neoplasms; Humans; Infant; Retinoblastoma; Time Factors
PubMed: 8146537
DOI: No ID Found -
International Ophthalmology Clinics 1996In the pediatric population, retinoblastoma must be included in the differential diagnosis of intraocular or orbital inflammation, especially if the case is atypical of... (Review)
Review
In the pediatric population, retinoblastoma must be included in the differential diagnosis of intraocular or orbital inflammation, especially if the case is atypical of unresponsive to medical therapy. Diffuse infiltrating retinoblastoma, a rare subtype of retinoblastoma, often presents as unilateral intraocular inflammation, usually occurs in older children, and may contain insufficient calcification for detection by CT scan. Orbital inflammatory signs may also be associated with intraocular retinoblastoma. The clinician must perform a thorough ocular examination in these cases with the suspicion of retinoblastoma. He or she must also understand the subtleties of noninvasive imaging in such cases. The goal is to diagnose this tumor more accurately and expediently so that treatment and counseling can be provided without delay.
Topics: Diagnosis, Differential; Endophthalmitis; Eye Neoplasms; Humans; Orbital Diseases; Prognosis; Retinoblastoma
PubMed: 8778062
DOI: 10.1097/00004397-199603610-00016 -
Ophthalmology Jan 1997The authors report the clinical, cytologic, and histopathologic findings of a unilateral retinoblastoma occurring in a 26-year-old woman. This tumor usually affects... (Review)
Review
PURPOSE
The authors report the clinical, cytologic, and histopathologic findings of a unilateral retinoblastoma occurring in a 26-year-old woman. This tumor usually affects young children; the mean age at the time of diagnosis usually ranges from 10 to 25 months.
METHODS
Histopathologic examination of the enucleated right eye included using standard techniques, as well as immunohistochemical stains of formalin-fixed, paraffin-embedded tissues.
RESULTS
Histologic examination of sections of the eye showed a retinal tumor that was centered in the equatorial region and had the typical features of a poorly differentiated retinoblastoma. Focal choroidal invasion and seeding of the anterior and posterior chambers were observed. Immunoreactivity of the tumor cells for neuron-specific enolase confirmed that the tumor is a neuronal neoplasm consistent with retinoblastoma.
CONCLUSION
Retinoblastoma occurring in adults is a rare finding. In most large series of retinoblastomas, no adults are included. Only eight patients 20 years of age or older with retinoblastomas have been documented previously. In the current case, the patient had no evidence of orbital recurrence or metastasis 6 years after enucleation of the eye. It may be important for clinicians to consider this diagnosis when confronted with a retinal mass of unknown etiology in adults.
Topics: Adult; Eye Enucleation; Eye Neoplasms; Female; Fundus Oculi; Glial Fibrillary Acidic Protein; Humans; Immunoenzyme Techniques; Phosphopyruvate Hydratase; Retinoblastoma; Ultrasonography; Visual Acuity
PubMed: 9022103
DOI: 10.1016/s0161-6420(97)30363-7 -
Development, Growth & Differentiation Jun 2014Once neurons enter the post-mitotic G0 phase during central nervous system (CNS) development, they lose their proliferative potential. When neurons re-enter the cell... (Review)
Review
Once neurons enter the post-mitotic G0 phase during central nervous system (CNS) development, they lose their proliferative potential. When neurons re-enter the cell cycle during pathological situations such as neurodegeneration, they undergo cell death after S phase progression. Thus, the regulatory networks that drive cell proliferation and maintain neuronal differentiation are highly coordinated. In this review, the coordination of cell cycle control and neuronal differentiation during development are discussed, focusing on regulation by the Rb family of tumor suppressors (including p107 and p130), and the Cip/Kip family of cyclin dependent kinase (Cdk) inhibitors. Based on recent findings suggesting roles for these families in regulating neurogenesis and neuronal differentiation, I propose that the Rb family is essential for daughter cells of neuronal progenitors to enter the post-mitotic G0 phase without affecting the initiation of neuronal differentiation in most cases, while the Cip/Kip family regulates the timing of neuronal progenitor cell cycle exit and the initiation of neuronal differentiation at least in the progenitor cells of the cerebral cortex and the retina. Rb's lack of involvement in regulating the initiation of neuronal differentiation may explain why Rb family-deficient retinoblastomas characteristically exhibit neuronal features.
Topics: Animals; Cell Cycle; Cell Differentiation; Cell Proliferation; Humans; Neurons; Resting Phase, Cell Cycle; Retinoblastoma; Retinoblastoma Protein
PubMed: 24697649
DOI: 10.1111/dgd.12127 -
Clinical & Translational Oncology :... Sep 2008
Topics: Humans; Retinal Neoplasms; Retinoblastoma
PubMed: 18796367
DOI: 10.1007/s12094-008-0245-8 -
Current Opinion in Ophthalmology Jun 1996The management of retinoblastoma has evolved tremendously for the past century, and recently there is a trend toward focal conservative treatments. This trend is due... (Review)
Review
The management of retinoblastoma has evolved tremendously for the past century, and recently there is a trend toward focal conservative treatments. This trend is due primarily to the earlier detection of the disease when the tumors are in a smaller stage of development as well as to advanced, more focused treatment modalities. Enucleation is still used for advanced retinoblastoma, especially when there is a concern that there may be invasion of the optic nerve, choroid, or orbit. The hydroxyapatite implant has provided improved cosmetic rehabilitation of the socket after enucleation. External-beam radiotherapy continues to be an important method of treating advanced retinoblastoma, especially when there is diffuse vitreous seeding. Plaque radiotherapy is a useful tool for controlling medium or small retinoblastomas, especially tumors with focal vitreous seeding or those that recur after other methods have failed. Cryotherapy and photocoagulation provide excellent control of small tumors, and advanced laser delivery systems have improved the visualization and ease of treatment of retinoblastoma. Thermotherapy and chemothermotherapy are the newest focal methods that are showing promising results for small to medium-sized retinoblastoma. Recent developments with chemotherapy regimens have allowed dramatic control of intraocular retinoblastoma, and they play an important role in the initial management of many cases.
Topics: Combined Modality Therapy; Eye Neoplasms; Humans; Retinoblastoma
PubMed: 10163455
DOI: 10.1097/00055735-199606000-00004 -
Indian Pediatrics Nov 1984
Topics: Child; Child, Preschool; Cobalt Radioisotopes; Combined Modality Therapy; Eye Neoplasms; Female; Humans; Infant; Male; Neoplasm Staging; Retinoblastoma
PubMed: 6534882
DOI: No ID Found