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NCI CANCERLIT^® Search: Retinoblastoma - June 2002

National Cancer Institute^®
Last Modified: June 1, 2002

Impaired expression and promotor hypermethylation of O6-methylguanine-DNA methyltransferase in retinoblastoma tissues.
Induction of VEGF gene expression by retinoic acid through Sp1-binding sites in retinoblastoma Y79 cells.
Retinoic acid upregulates cone arrestin expression in retinoblastoma cells through a Cis element in the distal promoter region.
Epstein-Barr virus-associated lymphoma in a child undergoing an autologous stem cell rescue.
Pattern of ophthalmic lesions at two histopathology centres in Ethiopia.
Editorial: chemotherapy for retinoblastoma.
Combined chemotherapy and local treatment in the management of intraocular retinoblastoma.
Overview: chemotherapy for retinoblastoma: an expanding area of clinical research.
Brief report: conservative treatment in unilateral retinoblastoma: a preliminary report.
Letter to the editor: acute myeloid leukemia in a patient surviving retinoblastoma.
[New strategies in the management of retinoblastoma]
Chemothermotherapy in the management of retinoblastoma.
Association between telomerase activity and basic fibroblast growth factor up-regulation in retinoblastomas.
Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation.
Retinoblastoma metastatic to the ovary in a patient with Waardenburg syndrome.
Ocular preservation after 36 Gy external beam radiation therapy for retinoblastoma.
In vitro effects of radiation on human retinoblastoma cells.
(Neonatal) retinoblastoma in the first month of life.
Mushroom-shaped choroidal recurrence of retinoblastoma 25 years after therapy.
Cyclin D1 overexpression in thyroid carcinomas: relation with clinico-pathological parameters, retinoblastoma gene product, and Ki67
Evaluation of an indicator for lymph node metastasis of esophageal squamous cell carcinoma invading the submucosal layer.
Presenting signs of retinoblastoma in Congolese patients.
Osseous choristoma of the conjunctiva simulating extraocular extension of retinoblastoma.
[Results of combined chemotherapy and local ophtalmic therapy of children with intraocular retinoblastoma]

1
UI - 11980845
AU - Choy KW; Pang CP; To KF; Yu CB; Ng JS; Lam DS
TI - Impaired expression and promotor hypermethylation of O6-methylguanine-DNA methyltransferase in retinoblastoma tissues.
SO - Invest Ophthalmol Vis Sci 2002 May;43(5):1344-9
AD - Department of Ophthalmology and Visual Sciences, Hong Kong Eye Hospital, The Chinese University of Hong Kong, 3/F, 147K Argyle Street, Kowloon, Hong Kong, China.
PURPOSE: To investigate the role of epigenetic changes in the promoter region of tumor-suppressor genes in the retinoblastoma genome and to study the disruption of expression of O6-methylguanine-DNA Methyltransferase (MGMT) due to aberrant methylation and its association with retinoblastoma. METHODS: A series of 23 retinoblastoma tissue specimens and 2 retinoblastoma cell lines (Y79 and WERI-Rb1) were subjected to methylation-specific PCR (MSP) analysis of hypermethylated genes identified in human cancers, including p14(ARF), p15(INK4b), p16(INK4a), VHL, and MGMT. Further, the expression of MGMT was studied by immunohistochemistry and, when fresh tissue was available, by Western blot analysis and RT-PCR. RESULTS: Aberrant methylation of at least one MGMT locus was detected in 8 of the 23 tumors (35%), all of which (100%) had impaired or absent expression of MGMT. The remaining 15 tumor specimens were nonmethylated, and, among them, 7 (43%) showed defective expression. No methylation of tumor DNA was found on the p14(ARF), p15(INK4b), p16(INK4a), and VHL genes. Hypermethylation in the MGMT promoter was found to be prominently present in retinoblastoma with poor tissue differentiation, and was more frequently detected among patients with bilateral disease. Production of MGMT was consistent with expression of mRNA. No methylation of MGMT promoter was detected in the two retinoblastoma cell lines (Y79, WERI-Rb1). CONCLUSIONS: The data show a clear association between impaired production of MGMT and hypermethylation of the MGMT promoter, which appeared to relate to early onset and poor differentiation, suggesting that epigenetic silencing of MGMT by methylation of the promoter and reduced expression of MGMT may play an important role in the development and progression of retinoblastoma.

2
UI - 11980848
AU - Akiyama H; Tanaka T; Maeno T; Kanai H; Kimura Y; Kishi S; Kurabayashi M
TI - Induction of VEGF gene expression by retinoic acid through Sp1-binding sites in retinoblastoma Y79 cells.
SO - Invest Ophthalmol Vis Sci 2002 May;43(5):1367-74
AD - Department of Ophthalmology, Gunma University School of Medicine, 3-39-15 Showa-machi, Maebashi, Gunma 371-8511, Japan.
PURPOSE: Vascular endothelial growth factor (VEGF) is an angiogenic peptide that has been implicated in many retinopathies. Although all trans-retinoic acid (atRA) has long been known as an essential factor in the visual cycle, the role of atRA in the pathogenesis of retinal disease remains elusive. In this study, we investigated the effects of atRA on expression of the VEGF gene in retinoblastoma Y79 cells. METHODS: Total RNA prepared from Y79 cells, with or without atRA, was subjected to Northern blot analyses. Reporter constructs consisting of the VEGF promoter-luciferase gene were transfected into Y79 cells. Nuclear factors binding to the VEGF promoter were analyzed by electrophoretic mobility shift assays (EMSAs). RESULTS: The levels of VEGF transcripts were increased by atRA in a time- and dose-dependent manner. Progressive deletion and site-specific mutation analyses indicated that atRA increased VEGF promoter activity through a G+C-rich sequence that was shown to be an Sp1-binding site by supershift assays. EMSAs showed that Sp1 binding was increased by atRA stimulation. Although no measurable change was observed in Sp1 mRNA levels, Western blot analysis showed an increase in Sp1 protein levels in the atRA-treated cells. These data suggest that atRA increases Sp1 protein levels by posttranscriptional mechanisms, and elevated levels of Sp1 protein induce the expression of VEGF at the transcriptional level. CONCLUSIONS: atRA induced transcription of the VEGF gene through Sp1-binding sites in Y79 cells. Pharmacologic intervention that inhibits the signals elicited by atRA may be effective in treating VEGF-mediated retinopathies.

3
UI - 11980849
AU - Li A; Zhu X; Craft CM
TI - Retinoic acid upregulates cone arrestin expression in retinoblastoma cells through a Cis element in the distal promoter region.
SO - Invest Ophthalmol Vis Sci 2002 May;43(5):1375-83
AD - Mary D. Allen Laboratory for Vision Research, Doheny Eye Institute, University of Southern California, 1333 San Pablo Street, Los Angeles, CA 90089-9112, USA.
PURPOSE: This study was initiated to investigate the molecular mechanisms of activation of expression of the human cone arrestin (hCAR) gene by retinoic acid (RA), in an in vitro model of retinoblastoma cells. METHODS: Human retinoblastoma Weri-Rb-1 or Y79 cell lines were cultured in the absence or presence of RA analogues with transcription or translation inhibitors for various periods. The mRNAs encoding hCAR, retinoic acid receptor (RAR), and retinoid X receptor (RXR) subtypes were analyzed by Northern blot. Immunoblot analysis of hCAR protein was also performed. The hCAR promoter's activity and its responsiveness to RA treatment was evaluated by transient transfection of the hCAR promotor-luciferase reporter constructs, followed by promoter deletion analysis to map the specific regions responsible for the RA response. RESULTS: In our in vitro model, both all-trans RA and 9-cis RA induced hCAR mRNA in a time- and dose-dependent manner. RA's effect was blocked by either RNA or protein synthesis inhibitors; however, hCAR mRNA's stability was not affected by RA, as determined by RNA decay experiments. Although all RAR and RXR subtypes were detected, only RXRgamma and RARalpha increased dramatically after treatment with RA. An RXR-specific agonist, but not an RAR-specific agonist, also increased hCAR mRNA and protein expression in both Weri-Rb-1 and Y79 cells. RA stimulated hCAR promoter-luciferase activity in transient transfection studies. Subsequently, a region between -852 and -702 of the hCAR promoter, with RA-responsive elements (RAREs), was discovered to be responsible for the RA response. CONCLUSIONS: The hCAR gene is transcriptionally upregulated by RA acting through cis elements within -852 to -702 of the hCAR 5' flanking region. Based on the cumulative data, RXRgamma is most likely the RA receptor subtype involved in hCAR regulation by RA.

4
UI - 11998794
AU - Heath JA; Broxson EH Jr; Dole MG; Filippa DA; George D; Lyden D; Dunkel
TI - IJ Epstein-Barr virus-associated lymphoma in a child undergoing an autologous stem cell rescue.
SO - J Pediatr Hematol Oncol 2002 Feb;24(2):160-3
AD - Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
Epstein-Barr virus-associated lymphoproliferative disease (EBV-LPD) is a serious disorder seen in various states of immunodeficiency, often with a fatal outcome. In this article, a patient with EBV-lymphoma after autologous stem cell rescue for treatment of a nonhematologic solid tumor is described. The child, a 4-year-old boy, had unilateral retinoblastoma with metastatic spread to the central nervous system. He had previously received both local tumor bed and craniospinal radiation therapy together with intensive myeloablative alkylator chemotherapy before autologous stem cell rescue. Histologically confirmed lymphoma with evidence of active EBV proliferation developed within cervical lymph nodes 3 weeks after his first autologous stem cell rescue. A complete clinical remission of the lymphadenopathy was obtained after infusions of rituximab (an anti-CD20 monoclonal antibody), acyclovir, and high-titer anticytomegalovirus immunoglobulin. The patient died approximately 6 months later of persistent and progressive retinoblastoma without any clinical evidence of lymphoma. It is concluded that EBV-LPD should be included in the differential diagnosis in patients in whom lymphadenopathy develops after autologous stem cell rescue.

5
UI - 12002085
AU - Assegid A
TI - Pattern of ophthalmic lesions at two histopathology centres in Ethiopia.
SO - East Afr Med J 2001 May;78(5):250-4
AD - Department of Ophthalmology, Addis Ababa University, Ethiopia.
OBJECTIVE: To describe the distribution of ocular, orbital and eyelid lesions that required histopathologic analysis in Ethiopian children and adults. DESIGN: A retrospective study. SETTING: Tikur Anbessa and Menelik II Teaching hospitals, Addis Ababa, Ethiopia, during 1995 and 1999 period. RESULTS: Two hundred and ninety ophthalmic specimens were examined, 20% of which came from children. Half of the lesions had epithelial origin, about 30% were malignant, 22.6% were benign and 16.4% were potentially malignant. Squamous cell carcinoma was the leading conjunctival (26%), eyelid (33%), orbital (33%) and ocular (20%) lesion among adults and elderly people whereas only 6% of eyelid lesion were basal cell carcinomas. In children the most frequent intra-ocular as well as orbital tumour was retinoblastoma, 39%, followed by miscellaneous benign lesions (24%). More than half of the request forms were incomplete. CONCLUSIONS: In Addis Ababa, squamous cell carcinoma and retinoblastoma should be considered when evaluating ophthalmic lesions in adults and children, respectively. Clinicians and pathologists should improve their communication by filling in request forms, providing clear reports and making dialogue.

6
UI - 11984796
AU - Shields CL; Shields JA
TI - Editorial: chemotherapy for retinoblastoma.
SO - Med Pediatr Oncol 2002 Jun;38(6):377-8

7
UI - 11984802
AU - Brichard B; De Bruycker JJ; De Potter P; Neven B; Vermylen C; Cornu G
TI - Combined chemotherapy and local treatment in the management of intraocular retinoblastoma.
SO - Med Pediatr Oncol 2002 Jun;38(6):411-5
AD - Department of Pediatric Hematology and Oncology, Cliniques Universitaires Saint Luc, Universite Catholique de Louvain, Brussels, Belgium. Brichard@pedi.ucl.ac.be
BACKGROUND: To assess the efficacy of chemotherapy (chemoreduction) plus local treatments as an alternative to external beam and enucleation for intraocular retinoblastoma. MATERIALS AND METHODS: A prospective study was performed on 21 patients with retinoblastoma treated in our outcome of those 33 eyes. RESULTS: There were 9 unilateral and 12 bilateral retinoblastoma cases. There were 12 eyes with Reese-Ellsworth group I-IV and 21 eyes with group V. Among 33 eyes, nine eyes (27%) were initially managed by enucleation. The remaining 24 eyes (73%) were initially treated with chemoreduction (maximum of six cycles of carboplatin, vincristine, etoposide) or chemothermotherapy. Among those 24 eyes, 20 were successfully treated with local treatments (thermotherapy plus cryotherapy in 16 eyes and thermotherapy plus cryotherapy plus (125)I plaque radiotherapy in 4 eyes), enucleation eventually underwent in two eyes and was proposed but refused in one child with bilateral group V retinoblastoma. With a median follow-up of 21 months, conservative management without external beam radiation was successful in all 12 eyes with group I-IV and in a total of 20/33 eyes (60%). Among the nine cases of unilateral retinoblastoma, eight were enucleated but among the 24 eyes with bilateral retinoblastoma, 19 (79%) were successfully treated with conservative therapy. CONCLUSIONS: It may be possible to eradicate viable tumor in all eyes with Reese-Ellsworth group I-IV retinoblastoma by chemoreduction followed by local treatments. Although 8 out of 21 eyes (38%) with group V retinoblastoma may be salvaged after chemoreduction and local therapies, enucleation remained the treatment of choice in those eyes with total retinal detachment and diffuse vitreous seeding. Copyright 2002 Wiley-Liss, Inc.

8
UI - 11984806
AU - Schouten-Van Meeteren AY; Moll AC; Imhof SM; Veerman AJ
TI - Overview: chemotherapy for retinoblastoma: an expanding area of clinical research.
SO - Med Pediatr Oncol 2002 Jun;38(6):428-38
AD - Department of Pediatrics, Division of Hemato-Oncology, Vrije Universiteit Medical Center, VUMC, Amsterdam. n.schouten@azvu.nl

9
UI - 11984807
AU - Hadjistilianou T; Mastrangelo D; De Francesco S; Mazzotta C
TI - Brief report: conservative treatment in unilateral retinoblastoma: a preliminary report.
SO - Med Pediatr Oncol 2002 Jun;38(6):439-41
AD - Centro per la Ricerca Interdipartimentale per lo Studio delle Affezioni Tumorali dell'Occhio, Department of Ophthalmology, University of Siena, Siena, Italy.

10
UI - 11984811
AU - Gallegos-Castorena S; Medina-Sanson A; Gonzalez-Montalvo P;
TI - Martinez-Avalos A; Zafra de la Rosa GZ Letter to the editor: acute myeloid leukemia in a patient surviving retinoblastoma.
SO - Med Pediatr Oncol 2002 Jun;38(6):450

11
UI - 11941242
AU - Balmer A; Munier F; Zografos L
TI - [New strategies in the management of retinoblastoma]
SO - J Fr Ophtalmol 2002 Feb;25(2):187-93
AD - Hopital Ophtalmique Jules Gonin, Service Universitaire d'Ophtalmologie, 15, Avenue de France, CH 1004 Lausanne, France. aubin.balmer@bluewin.ch
It was rare that a child survived retinoblastoma at the beginning of the twentieth century. Today the survival rate is in the order of 95% in reference centers, with new strategies improving prognosis step by step. Systematic enucleation used to be the starting point of any true and structured management, until the advent of radiotherapy made it possible not only to save lives but also to retain some useful vision. Early diagnosis has enabled focal therapies such as photocoagulation, cryocoagulation, and radioactive applicators to open up a new era of targeted tumor treatment. However, the onset of nonocular tumors secondary to radiotherapy, the resistance of certain tumors to irradiation, and unsightly cosmetic consequences all justify research into alternative therapeutic strategies. New types of chemotherapy have shown spectacular results and are currently under study: chemoreduction to make large tumors more manageable and enable less aggressive treatment of tumors located in delicate sites, thermochemotherapy using the effect of heat on plasma membrane permeability to antimitotics, and chemotherapy associated with cyclosporine to reduce the multidrug resistance of certain tumors. The aim is to avoid primary enucleation and external beam radiation as far as possible. The future may lie in local chemotherapy, hyperthermia, and dynamic phototherapy, accelerated proton beam radiotherapy also has promising prospects.

12
UI - 12045055
AU - Lumbroso L; Doz F; Urbieta M; Levy C; Bours D; Asselain B; Vedrenne J;
TI - Zucker JM; Desjardins L Chemothermotherapy in the management of retinoblastoma.
SO - Ophthalmology 2002 Jun;109(6):1130-6
AD - Department of Ophthalmology, Institut Curie, Paris, France.
OBJECTIVE: To evaluate the results of chemothermotherapy for the treatment of retinoblastoma. DESIGN: Non-comparative interventional case series. PATIENTS: Fifty-one children (65 eyes and 103 tumors) were treated with chemothermotherapy in a single institution from January of transpupillary thermotherapy delivered shortly after intravenous (IV) injection of carboplatin (560 mg/m(2)). Each tumor is treated separately with a diode laser using a microscope. Laser intensity, spot size, and duration are adapted to the size of each tumor and to the clinical response. After 8 days, thermotherapy alone is repeated. This cycle is performed from one to six times, every 28 days. The treatment data and outcome are analyzed separately. MAIN OUTCOME MEASURES: Assessment of local tumor control. RESULTS: One hundred three tumors were treated in 65 eyes of 51 children. Age at diagnosis was 0 to 60 months (median, 7 months). Median tumor diameter at the time of treatment was 3.5 mm (range, 1.5-12 mm). Laser modalities were as follows: median intensity, 450 mW (range, 150-1000 mW); median spot size, 1.2 mm (range, 0.3-2.0 mm); and median number of cycles required to obtain tumor control, three. Tumor regression was obtained for 99 tumors (96.1%) after a median follow-up of 30 months (17-61 months). Seven tumors relapsed after initial control (6.8%). Salvage treatment (external beam radiation, iodine plaques, or enucleation) was necessary for a total of 11 tumors (10.7%). The only risk factor for relapse was the initial diameter of the lesion greater than 3.5 mm, whereas the other tumor characteristics or treatment variables were not significantly correlated with relapse. Ninety-seven percent of treated eyes were able to be preserved, and 92% of cases were treated without external beam radiation. CONCLUSIONS: Chemothermotherapy is an effective technique to treat small- to medium-sized retinoblastomas in children, avoiding external beam irradiation.

13
UI - 11911245
AU - Kondo Y; Tanaka Y; Shields JA; Kondo S
TI - Association between telomerase activity and basic fibroblast growth factor up-regulation in retinoblastomas.
SO - Anticancer Res 2001 Nov-Dec;21(6A):3765-72
AD - Center for Surgery Research, The Cleveland Clinic Foundation, OH 44195, USA. Yasuko.Kondo@mssm.edu
BACKGROUND: Telomerase is an enzyme associated with cellular immortality and malignancy in many cell types. On the other hand, growth factors such as basic fibroblast growth factor (bFGF) or platelet-derived growth factor (PDGF) promote tumor growth, whereas the association between telomerase and these growth factors remains unclear. MATERIALS AND METHODS: Telomerase activity and expression of bFGF and PDGF were assayed in 9 retinoblastoma tissues and 2 cell lines (WERI-Rb-1 and Y79). The association of telomerase activity with bFGF or PDGF was investigated. RESULTS: Telomerase activity was detected in three out of nine tissues and both cell lines. Two telomerase highly-positive tissues and WERI-Rb-1 and Y79 cells expressed bFGF. As for the expression of PDGF, only one retinoblastoma tissue with high telomerase was positive. To further determine whether telomerase activity and bFGF are closely associated, we inhibited each expression. Inhibition of telomerase in WERI-Rb-1 cells using the anti-sense treatment suppressed the expression of bFGF and subsequently induced apoptosis after 25 to 30 doublings. When bFGF expression was suppressed by the neutralizing antibody, telomerase activity was not affected nor was apoptosis detected. CONCLUSION: Telomerase may up-regulate the expression of bFGF and protect retinoblastoma cells from cell death, indicating, the possibility that inhibition of telomerase is a promising approach for the treatment of telomerase-positive tumors.

15
UI - 11992879
AU - Moshfeghi DM; Wilson MW; Haik BG; Hill DA; Rodriguez-Galindo C; Pratt CB
TI - Retinoblastoma metastatic to the ovary in a patient with Waardenburg syndrome.
SO - Am J Ophthalmol 2002 May;133(5):716-8
AD - Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, Tennessee 38163, USA.
PURPOSE: To report a child with retinoblastoma and Waardenburg syndrome who developed ovarian metastases. DESIGN: Interventional case report. METHODS: Unilateral retinoblastoma was diagnosed in a 3-year-old girl with Waardenburg syndrome and leukocoria in the right eye. The patient had a Reese-Ellsworth Group Va tumor and underwent enucleation. Two years later, she developed metastatic disease involving the bone marrow, right humerus, both supraorbital bones, and both tibias. She was treated with chemotherapy, orbital irradiation, and bone marrow transplant but returned 7 months later with back pain and urinary retention. RESULTS: Exploratory laparotomy revealed a right ovarian mass, and the excised ovary showed metastatic retinoblastoma. The child underwent chemotherapy and remained asymptomatic for 9 months, when brain metastases were diagnosed. She died within 2 days of admission. CONCLUSION: We believe that this is the first description of a patient with retinoblastoma and Waardenburg syndrome and of an ovarian metastasis from retinoblastoma.

16
UI - 11972090
AU - Merchant TE; Gould CJ; Hilton NE; Kun LE; Rodriguez-Galindo C; Pratt CB;
TI - Wilson MW; Haik B Ocular preservation after 36 Gy external beam radiation therapy for retinoblastoma.
SO - J Pediatr Hematol Oncol 2002 May;24(4):246-9
AD - Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA. thomas.merchant@stjude.org
PURPOSE: The purpose of this study was to document the ocular preservation rate after 36 Gy external beam radiation therapy (EBRT) for retinoblastoma. PATIENTS AND METHODS: Forty-nine eyes of 38 patients were treated with a median dose of 36 Gy EBRT. The patient population included 7 unilateral and 31 bilateral presentations, with a median age at diagnosis of 4 months. Eyes enucleated at the time of diagnosis or treated with other measures were not included in the analysis of ocular preservation. The median age at EBRT was 8 months. Patients were monitored for progression of disease after EBRT and second malignant neoplasms. RESULTS: The median follow-up was 88.6 months, with an estimated ocular preservation rate of 82.0% +/- 5% at 10 years. There was a difference in the ocular preservation rates for patients with advanced disease (Reese-Ellsworth group III-V) compared with early disease. Metastatic disease developed in two patients, and a second malignant neoplasm developed in three. Patients treated with en face electrons experienced a lower 5-year estimate of ocular preservation than those treated with photons, although patients treated with electrons were more likely to have advanced disease. CONCLUSIONS: The use of low-dose EBRT (36 Gy) results in ocular preservation rates that are comparable to those of high-dose EBRT. The use of electrons requires careful treatment planning and computerized dosimetry.

17
UI - 11992382
AU - Zhang M; Stevens G; Madigan MC
TI - In vitro effects of radiation on human retinoblastoma cells.
SO - Int J Cancer 2001;96 Suppl():7-14
AD - Department of Radiation Oncology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
Retinoblastoma (Rb) is the most common intraocular tumor of childhood and has been demonstrated clinically to be very sensitive to external beam radiation (EBR). The purpose of this study was to determine the survival of Rb cell lines at different endpoints following irradiation. We also studied Rb-reconstituted cell lines postirradiation to gain insight into the role of Rb following DNA damage. Suspension cultures were exposed to single doses of 1, 2, 5, and 10 Gy. Following irradiation, cell viability and cell death was assessed for up to 72 hr using Trypan blue exclusion and acridine orange/ethidium bromide (AO/EB) staining, respectively. Morphological features were examined with light and electron microscopy (LM and EM). Clonogenic survival was assessed 2 weeks postirradiation. Cell cycle distribution was analyzed by flow cytometry. A time- and dose-dependent decrease in cell viability was induced in Y79 and WERI-Rb1 cells following irradiation, although not below approximately 45% for the times and doses used. A similar but much-reduced effect on viability was observed in Rb-reconstituted cells. AO/EB staining, LM, and EM revealed features characteristic of apoptosis following irradiation and a time- and dose-dependent increase in apoptosis was observed. For Y79 and WERI-Rb1 cells, 30-40% apoptosis was observed 72 hr after 10 Gy irradiation; however, apoptosis was much reduced in Rb-reconstituted cells (approximately 8% Y79LxRb28; approximately 18% WERILxRb8). Rb cell lines were extremely sensitive to radiation, although less so in Rb-reconstituted lines, with clonogenic survivals after 2 Gy (SF2) of 0.14 for Y79, 0.06 for WERI-Rb1, 0.36 for Y79LxRb28, and 0.19 for WERILxRb8. Postirradiation, a sub-G1 population was observed, consistent with radiation-induced apoptosis, and Rb-reconstituted cells displayed a prolonged G2 phase. The clonogenic survival parameters of Rb cell lines are consistent with clinical observations, where extreme sensitivity to irradiation has been reported. Additionally, Rb protein protected cells from DNA damage and may also play a role in radiation-induced G2 delay. This in vitro approach provides a useful model for further radiobiological studies of Rb. Copyright 2002 Wiley-Liss, Inc.

18
UI - 12049578
AU - Abramson DH; Du TT; Beaverson KL
TI - (Neonatal) retinoblastoma in the first month of life.
SO - Arch Ophthalmol 2002 Jun;120(6):738-42
AD - Robert M. Ellsworth Ophthalmic Oncology Center, New York Presbyterian Hospital-Weill Cornell Medical College, NY, USA. ICancerMD@aol.com
OBJECTIVES: To identify patients with retinoblastoma whose conditions were diagnosed at the age of 1 month or younger and to describe their clinical features (including ocular and patient survival) and the development of second nonocular tumors. MATERIALS AND METHODS: A retrospective study of 1831 patients. The cumulative incidence of second cancer development was analyzed using the Kaplan-Meier method. RESULTS: Forty-six patients were identified as having a diagnosis of retinoblastoma at the age of 1 month or younger (mean age, 18.5 days). Family history (31 patients [67%]) exceeded leukocoria (6 patients [13%]) as the most common reason for detection. Twenty-six (56%) of the 46 patients were seen with unilateral retinoblastoma, with 22 ultimately developing cancer in the fellow eye. At the initial diagnosis, 81 (85%) of the 95 tumors were detected in zones 1 and 2. Eighty-two (93%) of the 88 subsequent tumors were located in zones 2 and 3. In the 26 patients who had unilateral retinoblastoma, 16 of the initially affected eyes and 21 of the fellow eyes were salvaged. In the 19 (44%) of 20 patients who were seen initially with bilateral retinoblastomas, 31 (82%) of the 38 eyes were salvaged. The mean follow-up was 10.9 years. The incidence of second nonocular cancers reached 54% by 23.7 years for the patients who received radiation therapy, while the incidence was 0% for the patients who did not. Four (8.7%) of the 46 patients developed metastatic disease and died; 3 of these patients had documented metastases in the first month of life (one at birth). CONCLUSIONS: The most common manifesting sign of children diagnosed as having retinoblastoma in the first month of life is family history. Eyes with Reese-Ellsworth group I retinoblastomas were the most common. In patients with bilateral and unilateral retinoblastoma, new (subsequent) ocular tumors developed in a centrifugal pattern. Despite an early diagnosis, patients' eyes came to enucleation, and metastatic disease and death occurred from ocular metastases. In patients who received radiation therapy, the probability of developing second nonocular cancer is 54% by 23.7 years; no second cancers developed in patients who did not receive radiation therapy.

19
UI - 12049595
AU - Shields CL; Piccone MR; Shields JA; Eagle RC Jr; Singer M
TI - Mushroom-shaped choroidal recurrence of retinoblastoma 25 years after therapy.
SO - Arch Ophthalmol 2002 Jun;120(6):844-6
AD - Ocular Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107, USA.

20
UI - 11041450
AU - Basolo F; Caligo MA; Pinchera A; Fedeli F; Baldanzi A; Miccoli P;
TI - Iacconi P; Fontanini G; Pacini F Cyclin D1 overexpression in thyroid carcinomas: relation with clinico-pathological parameters, retinoblastoma gene product, and Ki67 labeling index.
SO - Thyroid 2000 Sep;10(9):741-6
AD - Department of Oncology, University of Pisa, Italy. f.basolo@do.mcd.unipi.it
Cyclin D1 is a G1 cyclin participating in the control of cell cycle progression through interaction with the retinoblastoma gene product (pRB). The overexpression of positive regulators (such as cyclin D1) has been reported in a variety of neoplasms, but their role in thyroid tumorigenesis is yet to be established. In our series of 54 thyroid carcinomas, cyclin D1 overexpression (detected by both immunohistochemistry and by Northern blotting) was correlated with prognostic variables, proliferative activity and pRB. Cyclin D1 overexpression was observed in 35% of thyroid carcinomas with a significantly higher expression of this cyclin in neoplastic tissues than in matched normal parenchyma. In well-differentiated carcinomas, the cyclin D1 mRNA overexpression was inversely correlated with nodal status (p = 0.03), while the protein product was higher in tumors from patients less than 40 than patients over 40 years of age. Inversely, there was no significant correlation with gender and tumor status, pRB and with proliferative activity.

21
UI - 11927013
AU - Nakajima Y; Nagai K; Miyake S; Ohashi K; Kawano T; Iwai T
TI - Evaluation of an indicator for lymph node metastasis of esophageal squamous cell carcinoma invading the submucosal layer.
SO - Jpn J Cancer Res 2002 Mar;93(3):305-12
AD - Department of Surgery, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo 113-8519, Japan. yasu.nakajima.srg1@tmd.ac.jp
Lymph node metastasis is a major prognostic factor for esophageal squamous cell carcinoma (ESCC). In recent years, endoscopic mucosal resection (EMR) has been developed with excellent results for the treatment of the superficial ESCC. To make the EMR treatment successful, it is important to establish a good indicator to identify ESCC patients at a high risk of lymph node metastasis. In this study, we examined clinicopathological and immunohistochemical factors to investigate the factors involved in lymph node metastasis of ESCC invading to the submucosal layer (sm-ESCC). Surgical specimens from 84 sm-ESCC patients were examined. Among 84 sm-ESCC patients, 33 (39.3%) had lymph node metastases. Clinicopathologically, tumor depth, lymphatic invasion and blood vessel invasion showed significant correlations with lymph node metastasis by univariate analysis. Tumor depth and lymphatic invasion showed significant correlations by multivariate analysis of these factors. Immunohistochemically, P53 accumulation was observed in 45 cases (53.6%), cyclin D1 overexpression in 25 (29.8%), and pRB in 65 (77.4%). P53 accumulation, cyclin D1 overexpression and MIB-1 Labeling Index were significantly associated with lymph node metastasis by univariate analysis, and P53 accumulation showed a significant correlation with lymph node metastasis by multivariate analysis. Among tumor depth, lymphatic invasion and P53 accumulation, tumor depth and lymphatic invasion were significantly correlated with lymph node metastasis (P = 0.0023 and P = 0.0092, respectively) by multivariate analysis. These data suggest that tumor depth and lymphatic invasion can be considered as good indicators for lymph node metastasis among patients with sm-ESCC. In addition, P53 accumulation could be helpful to identify the patients who need additional treatment after EMR.

22
UI - 12058485
AU - Kaimbo WK; Mvitu MM; Missotten L
TI - Presenting signs of retinoblastoma in Congolese patients.
SO - Bull Soc Belge Ophtalmol 2002;(283):37-41
AD - Department of Ophthalmology, University of Kinshasa, D R Congo.
OBJECTIVE: To report the relative frequency of the signs of presentation in Congolese children with retinoblastoma. METHODS: A retrospective study was undertaken of all (29) patients with retinoblastoma examined boys and 10 (34%) girls. For all cases, mean age at diagnosis was 2.94 years +/- 1.6 (range, four months to six years). For bilateral cases, it was 1.12 year +/- 1.4 (range, two months to three years) whereas for unilateral cases it was 3.23 years +/- 1.5 (range, four months to six years) (P = 0.016). More than seven distinct signs were identified. Leukocoria was the most common presenting sign in 49% of diagnosed cases followed by proptosis (28%). Other signs were strabismus, red eye, anterior scleral staphyloma, hyphema and buphthalmia. CONCLUSION: Strabismus seemed to be uncommon whereas proptosis is important in our small series when compared to signs reported in the developed world.

23
UI - 12036677
AU - Marback EF; Stout TJ; Rao NA
TI - Osseous choristoma of the conjunctiva simulating extraocular extension of retinoblastoma.
SO - Am J Ophthalmol 2002 Jun;133(6):825-7
AD - Doheny Eye Institute and the Department of Ophthalmology, Keck School of Medicine of the University of Southern California, Los Angeles, California, USA.
PURPOSE: To report the clinicopathologic features of an epibulbar osseous choristoma simulating extraocular extension of retinoblastoma. DESIGN: Interventional case report. METHODS: A 7-month-old male presented with a unilateral intraocular mass localized to the temporal retina and a white pedunculated lesion extending from the conjunctiva, anterior to the insertion of the lateral rectus muscle. Clinical examination and computed tomography scan findings led to the diagnosis of retinoblastoma with extraocular extension. The child underwent excision of the epibulbar lesion and enucleation of the globe. RESULTS: The histopathologic examination of the epibulbar lesion revealed an osseous choristoma. The intraocular contents displayed features typical of retinoblastoma without extraocular extension. CONCLUSION: An epibulbar osseous choristoma can simulate extraocular extension of retinoblastoma in an eye harboring the intraocular malignancy.

24
UI - 12004148
AU - Balwierz W; Kobylarz J; Starzycka M; Dluzniewska A
TI - [Results of combined chemotherapy and local ophtalmic therapy of children with intraocular retinoblastoma]
SO - Med Wieku Rozwoj 2001 Jul-Sep;5(3 Suppl 1):15-23
AD - Klinika Hematologii i Onkologii Dzieciecej, Polsko-Amerykanski Instytut Pediatrii Collegium Medicum, Uniwersytet Jagielonski, Wielicka 265, 30-663 Krakow, Poland.
Until recently chemotherapy in retinoblastoma was used as adjuvant therapy after enucleation in cases with extraretinal spread of the disease (weal extension, orbital extension, neoplastic infiltrates of the optic nerve at resection line, intracranial metastasis and generalized disease). Recent experience has proved that use of chemotherapy for intraocular retinoblastoma before local treatment (so called "chemoreduction") has allowed not only to decrease the number of enucleations and indications for external beam irradiation or limit the extension of local therapy; but also to increase the chances for vision preservation and to decrease the risk of severe complications. Twenty children aged 0,5-96 months (with lesions in 29 eyes) in whom 2000, were the subject of this study. Among 29 children, in 15 (52%) stage V according to Reese-Ellsworth was established. Enucleation before chemotherapy was necessary in 9 cases, and in two more children the eye had to be removed after 1-2 courses of chemotherapy. In 11 remaining children (with 18 involved eyes) VEC chemotherapy combined with delayed local therapy (cryotherapy, photocoagulation, brachytherapy) was employed. Out of 18 treated cases enucleation could be avoided in 13 (72%), including 12 qualified as Reese-Ellsworth stage II or I. No eye with stage V could be saved. First-line chemotherapy combined with local treatment should be standard treatment for intraocular retinoblastoma groups I and II. More effective therapy is required for advanced cases particularly for Reese-Ellsworth eye group V.

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