Retinoblastoma (Glioblastoma, Retinal)

A malignant tumor arising from the nuclear layer of the retina that is the most common primary tumor of the eye in children. The tumor tends to occur in early childhood or infancy and may be present at birth. The majority are sporadic, but the condition may be transmitted as an autosomal dominant trait. Histologic features include dense cellularity, small round polygonal cells, and areas of calcification and necrosis. An abnormal pupil reflex (leukokoria); NYSTAGMUS, PATHOLOGIC; STRABISMUS; and visual loss represent common clinical characteristics of this condition. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2104)

Also Known As:

Networked: 4726 relevant articles (144 outcomes, 422 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Neuroblastoma
3.	Neoplasm Metastasis (Metastasis)
4.	Retinal Neoplasms
5.	Retinoblastoma (Glioblastoma, Retinal)

Experts

1.	Abramson, David H: 81 articles (12/2015 - 01/2002)
2.	Shields, Carol L: 73 articles (01/2016 - 04/2002)
3.	Shields, Jerry A: 53 articles (01/2016 - 04/2002)
4.	Krishnakumar, Subramanian: 44 articles (12/2015 - 03/2004)
5.	Murray, Timothy G: 41 articles (06/2015 - 07/2003)
6.	Dunkel, Ira J: 35 articles (01/2014 - 10/2003)
7.	Marr, Brian P: 32 articles (12/2015 - 02/2007)
8.	Gallie, Brenda L: 31 articles (02/2015 - 02/2002)
9.	Brodie, Scott E: 30 articles (12/2015 - 08/2008)
10.	Yu, Young Suk: 27 articles (05/2015 - 06/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Retinoblastoma:

1.	Carboplatin (JM8)FDA LinkGeneric 08/01/2009 - "A single injection of subconjunctival nanoparticle carboplatin was effective in the treatment of transgenic murine retinoblastoma, with no associated toxic effects. " 10/01/1999 - "Subconjunctival carboplatin as used in this protocol is effective for intraocular retinoblastoma. " 11/01/2000 - "This study documents the efficacy of subconjunctival carboplatin in the treatment of an animal model of retinoblastoma. " 02/01/2013 - "Bilateral groups D and E retinoblastoma receive additional subtenon's carboplatin boost for improved local control. " 08/01/2009 - "To evaluate the efficacy of subconjunctival nanoparticle carboplatin in the treatment of transgenic murine retinoblastoma. "
2.	Melphalan (Alkeran)FDA LinkGeneric 10/01/2015 - "The 2-year results of this study suggest that standard lower-dose (20 µg) intravitreal melphalan is safe and highly effective for the management of viable VS from retinoblastoma. " 03/01/2015 - "SOAI of melphalan is an effective treatment for intraocular retinoblastoma, achieving high globe salvage in cases of advanced disease, but can be associated with significant ocular complications. " 09/01/2014 - "Intravitreal melphalan is emerging as an effective treatment for refractory vitreous seeds in retinoblastoma, but there is limited understanding regarding its toxicity. " 08/01/2014 - "Intra-ophthalmic artery melphalan is an effective treatment for advanced cases of retinoblastoma, with a reasonable level of success. " 01/01/2013 - "Superselective intra-arterial melphalan therapy is effective for less advanced eyes but further modifications to therapy are required to improve results in eyes with advanced retinoblastoma."
3.	Vincristine (Oncovin)FDA LinkGeneric 01/01/2012 - "To investigate the therapeutic effect of oncolytic adenovirus SG600 in combination with vincristine (VCR) on retinoblastoma in vitro, the cell viability, cell cycle effects and apoptotic activity of HXO-RB(44) cells treated with SG600, VCR or SG600 plus VCR were measured using a cell counting kit-8-based procedure and flow cytometry. " 01/01/2012 - "Combined treatment with an oncolytic adenovirus and antitumor activity of vincristine against retinoblastoma cells." 02/01/2010 - "Here we show that Akt activity is involved in vincristine-induced cytotoxicity in retinoblastoma SO-Rb50 cells. " 02/01/2010 - "But how Akt affects vincristine-induced cell death in retinoblastoma is unknown. " 02/01/2010 - "Constitutively active Akt contributes to vincristine resistance in human retinoblastoma cells."
4.	Etoposide (VP 16)FDA LinkGeneric 03/01/2004 - "The role of etoposide in the treatment of higher risk intraocular retinoblastoma deserves further study." 03/01/2004 - "A pilot trial of oral etoposide (50 mg/m2/ day for 21 days) in five children (6 eyes) with relapsed refractory intraocular retinoblastoma was performed. " 08/01/2012 - "Effect of miR-513a-5p on etoposide-stimulating B7-H1 expression in retinoblastoma cells." 03/01/2004 - "Lack of activity of oral etoposide for relapsed intraocular retinoblastoma." 01/01/1995 - "First, the ability of six retinoblastoma cell lines to undergo apoptosis was surveyed using etoposide (30 micrograms/ml, 20 h exposure). "
5.	Cyclin D1IBA 09/01/2015 - "The aim of the present study was to investigate cell cycle markers p-16, cyclin D1, CDK4, E2F, and retinoblastoma (Rb) in this context. " 07/01/2004 - "Further immunoprecipitation studies found that, in response to the treatment, the formation of cyclin D1/cdk 4 complex declined, preventing the phosphorylation of retinoblastoma (Rb) and the subsequent dissociation of Rb/E2F complex. " 05/01/2015 - "Following further examination of cell cycle-related factors, MCM2 knockdown inhibited protein retinoblastoma (Rb), cyclin D1 and CDK4 expression, but increased p21 and p53 expression, suggesting that siMCM2 indeed triggered cell cycle arrest. " 02/01/2014 - "PK properties were similar to prior publications, and immunohistochemical staining for cyclin D1 and phospho-retinoblastoma did not predict response. " 09/15/2012 - "Cell cycle was arrested at the G1 phase and the expression of CDK4, cyclin D1, and phosphorylated retinoblastoma (pRb) was decreased. "
6.	Retinoblastoma ProteinIBA 09/01/2004 - "To develop and use improved Semliki Forest vectors (SFVs) for functional and structural analyses of the retinoblastoma protein (RB) in developing retina and retinoblastoma cells. " 09/01/2004 - "Retinoblastoma protein purification and transduction of retina and retinoblastoma cells using improved alphavirus vectors." 03/05/2004 - "Previous studies have suggested that tTG may modulate retinoblastoma (Rb) protein, an important suppressor of apoptosis. " 01/01/1998 - "Two of the cell lines that express the retinoblastoma (Rb) protein had no sign of a deregulated Rb pathway but further studies at the protein level are necessary to demonstrate whether these two cell lines should have a normal Rb pathway or whether they will join the majority of cell lines with deregulated Rb pathway." 03/27/1995 - "Studies of the retinoblastoma (RB) gene product suggest that it may work as a fundamental regulator to coordinate pathways of cellular growth and differentiation. "
7.	Topotecan (Hycamtin)FDA LinkGeneric 09/01/2014 - "Systematic review of studies available at PubMed using the keywords retinoblastoma, topotecan, and camptothecins, including preclinical data such as cell lines and animal models, as well as clinical studies in patients with retinoblastoma. " 03/01/2013 - "Thus, we here study the pharmacokinetics of topotecan after 0.5 μg and the toxicity profile of intravitreal topotecan in the rabbit eye as a potential treatment of retinoblastoma. " 12/01/2012 - "More studies are needed to establish the safety of topotecan for retinoblastoma in children." 04/01/2009 - "Up to 2 mg of periocular topotecan could be given safely, but further studies are necessary to determine its effect on retinoblastoma (ClinicalTrials.gov number, NCT00460876)." 04/01/2009 - "A phase I study of periocular topotecan in children with intraocular retinoblastoma."
8.	Cyclin D3IBA 05/20/2010 - "Cell-cycle analysis showed that M33-Meis1 impeded the progression of cells from G(1)-to-S phase, which correlated with significant reduction of cyclin D3 levels and the inhibition of retinoblastoma (pRb) hyperphosphorylation. " 09/01/2003 - "The expressions of N-myc, cyclin D3, and Wnt10B were downregulated, whereas those of retinoblastoma (RB) and related genes (p107, RB2/p130, p300/CBP, E2F-1, DP-1) as well as others were upregulated. " 06/15/2007 - "This natural product was effective at nontoxic doses as a cancer-preventive agent, which exerted its actions, at least in part, through the inhibition of cyclin D3 and Cdk4 expression and retinoblastoma tumor suppressor protein (Rb) phosphorylation. " 10/01/2007 - "The expression of retinoblastoma (pRb) and cyclin D3 proteins is highly induced during the process of skeletal myoblast differentiation. " 10/15/2007 - "Although cyclin D3 up-regulation and retinoblastoma hypophosphorylation occurred normally in FADDdd T cells, cyclin E expression and cyclin-dependent kinase 2 activation were markedly impaired in FADDdd T cells. "
9.	Proteins (Proteins, Gene)IBA 01/01/2007 - "The in silico and functional data presented in this work highlight considerable differences between dicot and monocot species in the retinoblastoma regulatory pathways and permit an improved classification of RBR proteins in higher plants." 08/01/2015 - "The objective of this study is to investigate the role of PLK1 and PLK3 proteins in human retinoblastoma tissues. " 04/01/2015 - "The aim of the study is to elucidate the role of CDC25A and CDC25B proteins in retinoblastoma and their association with the clinical and histopathological parameters. " 01/01/2015 - "This study aimed to examine the expression of Sonic hedgehog (SHH) signalling proteins in retinoblastoma and to evaluate its clinical significance. " 01/01/2012 - "This study represents a preliminary step towards a more accurate two dimensional electrophoresis (2DE) pattern, which will be combined with mass spectrometry analysis to clarify the potential role of specific proteins in tumour development and progression; although these results suggest that aqueous humour protein pattern in retinoblastoma is characteristic, several aspects of the study are still under investigation."
10.	Retinaldehyde (Retinal)IBA 09/01/2004 - "The efficacy of SFV as a retinal delivery system was tested on mouse explants and cultured human retinoblastoma cells. " 10/30/2014 - "Rb is critical for normal retinal development and germline mutations lead to retinoblastoma making retinae an attractive system to study Rb family signaling. " 03/01/2014 - "Our study demonstrates the downregulation of microRNA-183 (miR-183) in retinoblastoma (RB) tissues and RB cell lines compared with normal retinal tissues. " 08/01/2012 - "Further studies are required to assess IViC retinal toxicity and to better delineate its role in the management of retinoblastoma." 03/15/2008 - "Our recent studies on the role of the Rb family of genes in retinal development and retinoblastoma have led to a new hypothesis that explains this paradox. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 07/01/2009 - "Salvage EBRT is highly effective in preserving eyes with useful vision in bilateral retinoblastoma after failed chemotherapy and focal treatments. " 12/01/2014 - "Intra-arterial chemotherapy (IAC) has proved to be an effective treatment for retinoblastoma, but can be very expensive in developing countries. " 09/01/2008 - "This study aimed to evaluate the efficacy of tandem high-dose chemotherapy and autologous stem cell rescue (HDCT/ASCR) for treatment, instead of EBRT, in children with bilateral advanced retinoblastoma. " 09/01/2014 - "Our results suggest that improvements in overall survival are attributable to improved chemotherapy regimens and early detection of pineal trilateral retinoblastoma. " 05/01/2013 - "Intra-arterial chemotherapy is more effective than sequential periocular and intravenous chemotherapy as salvage treatment for relapsed retinoblastoma."
2.	Radiotherapy 03/01/2012 - "To determine the best treatment technique for patients with retinoblastoma requiring radiotherapy to the whole eye. " 08/01/2012 - "This technique is potentially safe and effective at a low cost and may play a promising role, especially in the treatment of recurrent and/or resistant vitreous disease in retinoblastoma, as an alternative to enucleation and/or external beam radiotherapy. " 01/01/2009 - "The proportion of cases treated with radiotherapy first increased from 20.5% in 1975-9 to 34.6% in 1985-9 and then sharply decreased to 6.5% in 2000-4. Over the last 30 years, there has been a gradual improvement in 5-year survival of children with retinoblastoma in the USA." 08/01/1987 - "Improved external beam radiotherapy for the treatment of retinoblastoma." 01/01/1953 - "[Case of bilateral retinal glioma cured by radiotherapy]."
3.	Cryotherapy (Therapy, Cold) 07/03/1976 - "Efficacy of cryotherapy in retinoblastoma." 02/01/2013 - "Discrete retinoblastoma (RB), too large for cryotherapy or thermolaser, or recurrent after these modalities, can be treated with plaque therapy, I-125, or Ru-106. " 01/01/2000 - "Presumed rhegmatogenous RD may occur after external beam radiation or cryotherapy for retinoblastoma. " 01/01/1993 - "Cryotherapy for retinoblastoma." 01/01/1990 - "The authors define their current indications and contraindications for cryotherapy of retinoblastoma."
4.	Heterologous Transplantation (Xenotransplantation) 06/01/1989 - "A xenograft model of human retinoblastoma heterotransplanted directly into the anterior chamber of the nude mouse eye has been adapted for evaluation of therapeutic efficacy. " 11/01/2014 - "The efficacy of the SYK antagonist R406 delivered locally in a human orthotopic xenograft mouse model of retinoblastoma was tested. " 01/01/2015 - "Studies of preclinical models of retinoblastoma in the form of transgenic mice and xenograft animal models have significantly contributed to the development of effective therapies for this disease. " 12/01/2009 - "Those changes correlated with the ability of the human retinoblastoma cell lines to invade the optic nerve and metastasize in orthotopic xenograft studies. " 10/01/2002 - "Preclinical studies were performed in a rabbit xenograft model of retinoblastoma. "
5.	Transplants (Transplant) 08/01/2010 - "To assess complete remission before subjecting nongermline metastatic retinoblastoma patients to an autologous peripheral stem cell transplant, we tested for patient-specific retinoblastoma tumor suppressor gene (RB1) mutant alleles in cerebrospinal fluid (CSF) and bone marrow. " 03/01/2015 - "A retrospective chart review of 14 consecutive pediatric patients who underwent dermis fat graft implantation after unilateral enucleation for retinoblastoma by 1 surgeon (E.A.S.) was performed to evaluate graft efficacy with regard to orbital volume growth and any associated morbidity. " 03/01/2015 - "To evaluate the efficacy of dermis fat graft (DFG) as a primary implant technique in pediatric patients requiring unilateral enucleation due to retinoblastoma. " 01/01/2004 - "Patients with extraocular retinoblastoma who achieve remission may benefit from consolidation of their therapy with autologous bone marrow transplant." 03/01/2015 - "In pediatric patients below 4 years of age with unilateral retinoblastoma treated with enucleation and primary dermis fat graft implantation, there was no statistically significant difference in bony orbital volume between the surgical and nonsurgical orbits during the follow-up period."