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PubMedDate not supplied

Spontaneous regression of retinoblastoma in a Taiwan series.

PURPOSE: To document the high occurrence of complete spontaneous regression of retinoblastoma in a Taiwan series. PATIENTS AND METHODS: Cases of retinoblastoma (133 eyes of 110 patients) at a single Taiwanese hospital from 1978 to 2002 were reviewed. RESULTS: Complete spontaneous tumor regression and phthisis bulbi were noted in 7 eyes of 7 patients. The regression rate was 5.3%, which is higher than previous reports of 1% to 2%. Tumor regression was associated with a common course of acute painful inflammation and ended with a shrunken, nonfunctioning eye. No residual vision was noted in any patient. CONCLUSION: Delayed diagnosis and advanced stage of the disease in this series of Taiwanese patients probably led to the massive intratumoral necrosis and resulted in phthisic change of the globe.

Child, Preschool↗
PubMed2000

[Ophthalmological tumors in children: diagnosis and therapeutic strategy].

Clinical aspects and diagnosis features of retinoblastoma are reported. The most important differential diagnosis are discussed. The diseases that can cause difficulties in diagnosis are uveitis and Coats disease. The principles of the treatment of retinoblastoma are exposed especially the side effects of radiotherapy that lead to a limitation of the use of external beam if possible. Treatments methods include external beam radiotherapy, chemotherapy, thermochemotherapy cryotherapy, curietherapy and photocoagulation. Results and indications of these treatments are given. We describe the diagnosis approach, for orbital tumors in children. The main orbital benign and malignant tumors and their treatment are listed: orbital cyst, neurofibroma; optic nerve gliomas; rhabdomyosarcomas, bone tumors and metastatic tumors.

Antineoplastic Agents↗
PubMed2001

Evaluation of chemoprophylaxis in patients with unilateral retinoblastoma with high-risk features on histopathologic examination.

OBJECTIVES: To identify risk factors for metastatic disease on histopathologic specimens of enucleated eyes from patients with unilateral retinoblastoma, and to evaluate the value of chemoprophylaxis in preventing disease dissemination. METHODS: Medical records from patients with unilateral retinoblastoma who underwent primary enucleation were reviewed at the University of California, San Francisco (1977-1998) and Bascom Palmer Eye Institute, University of Miami, Miami, Fla (1991-1998). All routine histopathologic specimens were reexamined. The extent of tumor invasion into the optic nerve or ocular coats and the prescribed chemoprophylactic regimen were recorded. RESULTS: This retrospective study included 129 patients followed for a median of 54 months. Three patients had tumor invading the sclera. The optic nerve was involved to some extent in 82 patients, 11 of whom had tumor extension beyond the lamina cribrosa. The surgical margin of the optic nerve was involved in an additional 4 patients. The choroid was involved in 43 patients, and was considered massively affected in 12 patients. Anterior segment involvement was observed in 10 patients. Postenucleation chemoprophylaxis was administered to 4 of 4 patients who had tumor cells at the surgical margin of the optic nerve and to 7 of 11 patients with postlaminar disease, all of whom had at least 1 mm of postlaminar tumor extension. External beam radiotherapy was administered to 3/4 and 1/11 of these patients, respectively. Chemoprophylaxis was not administered to patients with choroidal or anterior chamber involvement unless the optic nerve was also involved beyond the lamina cribrosa. One patient with tumor extending to the surgical margin of the optic nerve died of metastatic disease. CONCLUSIONS: Chemoprophylaxis is necessary for patients with tumor extending to the surgical margin of the optic nerve and is likely to be beneficial in preventing metastases in patients with tumor extending beyond the lamina cribrosa. We did not offer chemoprophylaxis to patients with prelaminar optic nerve disease or isolated choroidal involvement, and these patients remained free of disseminated disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

[Initial subretinal localization of acute myeloblastic leukemia (AML5) recurrence].

BACKGROUND: Reduced visual acuity in patients with acute leucemia can result from many causes including an ocular localization. CASE REPORT: A patient previously treated for acute myeloblastic leucemia-5 (AML5) developed bilateral vision impairment related to a subretinal localization of the leucemia. Meningeal and bone marrow relapse followed. The subretinal localization responded only to massive systemic steroid treatment. DISCUSSION: Although asymptomatic, ocular localizations are frequent in leucemia. Their prognostic impact depends on the ocular structure involved and on the chronology of onset--early or late in the leucemia course. The underlying pathophysiological mechanism of ocular involvement remains unexplained but hyperleucocytosis at presentation may be a risk factor and would justify at least systematic specialized examinations and discussion of prophylactic treatment.

Adult↗
PubMedDate not supplied

Second malignant neoplasms following treatment for primary cancer.

BACKGROUND: Long term survival following treatment for primary cancer has increased significantly in the past decade. With cure comes 'survivorship'. One of the factors clearly affecting quality and length of survival is morbidity associated with treatment and the development of subsequent malignancies. OBJECTIVE: This article aims to look at the risks associated with the development of second malignant neoplasms, give some meaningful figures, and provide suggestions for follow up. DISCUSSION: The balance of heightened awareness and healthy lifestyle versus relentless surveillance and ongoing patient concern is a difficult one. We hope to make our patients aware of the small but significant risk of a second malignancy, advise on any appropriate screening investigations and encourage a life long relationship with their chosen family physician to enable detection of any future malignancy.

Adult↗
PubMed2003

[Ultrasonography in ocular diagnosis].

Ultrasonography is a noninvazive and painless method, that can be performed to evaluate the ocular lesions. It is the most important imaging test that evaluate the posterior pole in eyes with opaque ocular media. Almost, it is helpful in the differentiation of intraocular lesions in the presence of clear ocular media. The paper presents the most common examination techniques and ecographyc aspects of varying intraocular lesions.

Biometry↗
PubMed2003

Eye-preservation treatment of retinoblastoma with vitreous seeding.

Retinoblastoma with vitreous seeding has been one of the most challenging conditions for eye-preservation therapy. Several modalities for treating vitreous seeding were reviewed in order to analyze the problems associated with them. External beam radiotherapy has been the most reliable method to treat vitreous seeding. However, recurrence after external beam radiotherapy needs other types of treatments to preserve the eyeballs. Due to the progress of investigations concerning retinoblastoma, chemotherapy has become the most promising method to cure not only recurrence but also primary tumors. Systemic chemotherapy can rarely cure vitreous seeding, but local chemotherapy using vitreous injections of melphalan can preserve about 50% of the eyeballs with vitreous seeding. Currently, animal experiments are being conducted to study the efficacy and safety of vitreous surgery combined with infusion of anticancer drugs for eradication of vitreous seeds and maintenance of visual function.

Animals↗
PubMed2004

[A new orthotopic retinoblastoma model expressing green fluorescent protein].

OBJECTIVE: In order to observe the growth and metastasis of the tumor directly, a new orthotopic retinoblastoma model was established with human RB cells expressing green fluorescent protein (GFP). METHODS: pEGFP-N(1), the eukaryotic expressive plasmid of GFP, was transferred into human RB cell line HXO-RB(44) by liposome Dosper. Then, the cell clones expressing GFP steadily were selected by means of neomycin, fluorescence microscope, and flow cytometer. Two microliters of RB cells (density at 4.5 x 10(8) - 5.5 x 10(8) cells per ml) were injected into the subretinal space of 30 nude mice (60 eyes) under binocular operating microscope. The growth of transplanted RB was observed in vivo using fluorescence stereomicroscope. The nude mice were killed at different times post-operatively to investigate the metastasis process of the tumor to the optic nerve, brain, and other organs including lung, liver and kidney. RESULTS: The spread process of the tumor in the subretinal space was successfully observed under stereomicroscope. Transplanted RB developed into extra-ocular stage phase at 34 - 37 days after the operation. And metastasis to the cranium along the optic nerve was observed, with green RB cells distributing along the optic nerve sheath and long posterior ciliary artery. The histopathological characteristics of the transplanted tumor were similar to the human RB. Immunohistochemical staining showed positive expression of GFP in the tumor cells. CONCLUSION: The established orthotopic RB model expressing GFP via injection of human RB cells into the subretinal space of nude mice provides a new approach to exploring the growth and metastasis processes of RB in natural situations.

Animals↗
PubMed2006

[Cancer in childhood and inheritance].

Five percent of childhood cancers reflect well-known hereditary cancer disposition syndromes. Hereditary cancer disposition syndromes should be considered when the cancer in question frequently is hereditary, when several related cancers occur in the same family, or in the presence of congenital malformations. Genetic counselling requires specialist training and should focus on the risk of developing cancer among family members, the risk of a second cancer in the patient, the possibility of prenatal diagnosis of the cancer disposition syndrome, and the advantages of prophylaxis and of early cancer diagnosis through screening.

Child↗
PubMed1996

A molecular pathologic study on apoptosis in retinoblastoma and the mechanism of spontaneous regression in retinoblastoma.

OBJECTIVE: The present study was designed to prove the existence of apoptosis in retinoblastoma (Rb) and to determine the pathogenic mechanism of spontaneous regression of Rb as well as the relationship between them. METHODS: Qualitative morphological study on Rb was performed by means of light microscope, electron microscope and TdT mediated biotin-dUTP nick-end labeling (TUNEL). Quantitative study was performed by automatic image analysis technology (AIAT) stained with Feulgen reaction. RESULTS: The characteristic regressed area occurred in all 47 cases of Rb. Morphological changes observed within Rb closely resembled the apoptotic cell described by Kerr et al in 1972. Under electron microscope, details of apoptosis were observed in 7 cases of Rb: the morphological sequence of events occurred in and around the cell nucleus. The morphology of the TUNEL labeling Rb cell was various. TUNEL labeling showed more positive cells in regressed area, while fewer in advanced area. AIAT revealed that apoptosis index (AI) in regressed areas was higher than that in other areas of Rb, DNA average ploidy (DP) consisted with the histology grade of Rb, the degree of hyperdiploid (DH) in metastatic area was distinctly higher than that in other areas (P < 0.05). CONCLUSIONS 1. Morphologic evidence proved the existence of apoptosis, especially more in regressed area of Rb. 2. Apoptosis may participate in the spontaneous regression of Rb. Apoptosis contributed to the spontaneous regression of Rb. 3. Tumor growth parameters of Rb (AI, DP, DH) obtained by AIAT may be used as quantitative index for pathologic classification, the selection of clinical treatment and the prognostic evaluation.

Apoptosis↗
PubMed1996

[Observation of the invasion of retinoblastoma cells to the corneal epithelial cells and keratocytes in culture].

OBJECTIVE: To study the invasive ability of retinoblastoma cells into the corneal epithelial cells and keratocytes in culture. METHODS: SO-Rb50 cells of retinoblastoma, corneal epithelial cells and keratocytes were co-cultured. The morphological changes of SO-Rb50 cells adherent to the corneal epithelial cells and keratocytes were observed. The distribution of proteoglycans in the SO-Rb50 cells, corneal cells and keratocytes was analyzed by histochemical method and electron microscopy. RESULTS: SO-Rb50 cells could adhere to the keratocytes, but did not adhere to the corneal epithelial cells. There are common hyaluronidase-resistant and chondroitinase-resistant proteoglycans on the surface of SO-Rb50 cells and corneal epithelial cells. Chondroitin is present on the surface of keratocytes. CONCLUSION: It is considered that the cell surface proteoglycans may play an important role in mediating SO-Rb50 tumor cell adhesion and non-adhesion to normal cells.

Adult↗