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The Lancet. Child & Adolescent Health Sep 2023
Topics: Humans; Retinoblastoma; Combined Modality Therapy; Retinal Neoplasms
PubMed: 37536350
DOI: 10.1016/S2352-4642(23)00165-7 -
AORN Journal Jan 2020Retinoblastoma is a rare malignancy of the eye affecting children, most commonly four years old and younger. Although chemotherapy and radiation treatment aim to spare... (Review)
Review
Retinoblastoma is a rare malignancy of the eye affecting children, most commonly four years old and younger. Although chemotherapy and radiation treatment aim to spare the eye, in some cases, enucleation (ie, removal of the eye) is required to prevent cancer metastases or recurrence. Enucleation procedures are primarily performed at specialty institutions and may involve the surgical placement of an implant in the orbit of the eye. Unique perioperative considerations are required because of the age of the child at the time of the diagnosis and procedure and the involvement of a parent or caregiver who will be caring for the child postoperatively. This article presents an overview of retinoblastoma and enucleation and discusses the care and management of the unique patient population undergoing enucleation.
Topics: Adolescent; Child; Child, Preschool; Eye Enucleation; Female; Humans; Infant; Male; Pediatrics; Retinoblastoma; Treatment Outcome
PubMed: 31886534
DOI: 10.1002/aorn.12896 -
Clinical Cancer Research : An Official... Jan 2022The retinoblastoma tumor suppressor protein (pRB) is a known regulator of cell-cycle control; however, recent studies identified critical functions for pRB in regulating... (Review)
Review
The retinoblastoma tumor suppressor protein (pRB) is a known regulator of cell-cycle control; however, recent studies identified critical functions for pRB in regulating cancer-associated gene networks that influence the DNA damage response, apoptosis, and cell metabolism. Understanding the impact of these pRB functions on cancer development and progression in the clinical setting will be essential, given the prevalence of pRB loss of function across disease types. Moreover, the current state of evidence supports the concept that pRB loss results in pleiotropic effects distinct from tumor proliferation. Here, the implications of pRB loss (and resultant pathway deregulation) on disease progression and therapeutic response will be reviewed, based on clinical observation. Developing a better understanding of the pRB-regulated pathways that underpin the aggressive features of pRB-deficient tumors will be essential for further developing pRB as a biomarker of disease progression and for stratifying pRB-deficient tumors into more effective treatment regimens.
Topics: Apoptosis; Humans; Retinal Neoplasms; Retinoblastoma; Retinoblastoma Protein
PubMed: 34407969
DOI: 10.1158/1078-0432.CCR-21-1565 -
JAMA Ophthalmology Jan 2022High-risk histopathologic features of retinoblastoma are useful to assess the risk of systemic metastasis. In this era of globe salvage treatments for retinoblastoma,...
IMPORTANCE
High-risk histopathologic features of retinoblastoma are useful to assess the risk of systemic metastasis. In this era of globe salvage treatments for retinoblastoma, the definition of high-risk retinoblastoma is evolving.
OBJECTIVE
To evaluate variations in the definition of high-risk histopathologic features for metastasis of retinoblastoma in different ocular oncology practices around the world.
DESIGN, SETTING, AND PARTICIPANTS
An electronic web-based, nonvalidated 10-question survey was sent in December 2020 to 52 oncologists and pathologists treating retinoblastoma at referral retinoblastoma centers.
INTERVENTION
Anonymized survey about the definition of high-risk histopathologic features for metastasis of retinoblastoma.
MAIN OUTCOMES AND MEASURES
High-risk histopathologic features that determine further treatment with adjuvant systemic chemotherapy to prevent metastasis.
RESULTS
Among the 52 survey recipients, the results are based on the responses from 27 individuals (52%) from 24 different retinoblastoma practices across 16 countries in 6 continents. The following were considered to be high-risk features: postlaminar optic nerve infiltration (27 [100%]), involvement of optic nerve transection (27 [100%]), extrascleral tissue infiltration (27 [100%]), massive (≥3 mm) choroidal invasion (25 [93%]), microscopic scleral infiltration (23 [85%]), ciliary body infiltration (20 [74%]), trabecular meshwork invasion (18 [67%]), iris infiltration (17 [63%]), anterior chamber seeds (14 [52%]), laminar optic nerve infiltration (13 [48%]), combination of prelaminar and laminar optic nerve infiltration and minor choroidal invasion (11 [41%]), minor (<3 mm) choroidal invasion (5 [19%]), and prelaminar optic nerve infiltration (2 [7%]). The other histopathologic features considered high risk included Schlemm canal invasion (4 [15%]) and severe anaplasia (1 [4%]). Four respondents (15%) said that the presence of more than 1 high-risk feature, especially a combination of massive peripapillary choroidal invasion and postlaminar optic nerve infiltration, should be considered very high risk for metastasis.
CONCLUSIONS AND RELEVANCE
Responses to this nonvalidated survey conducted in 2020-2021 showed little uniformity in the definition of high-risk retinoblastoma. Postlaminar optic nerve infiltration, involvement of optic nerve transection, and extrascleral tumor extension were the only features uniformly considered as high risk for metastasis across all oncology practices. These findings suggest that the relevance about their value in the current scenario with advanced disease being treated conservatively needs further evaluation; there is also a need to arrive at consensus definitions and conduct prospective multicenter studies to understand their relevance.
Topics: Eye Enucleation; Humans; Infant; Neoplasm Invasiveness; Optic Nerve Injuries; Prospective Studies; Retinal Neoplasms; Retinoblastoma; Retrospective Studies; Risk Factors; Surveys and Questionnaires
PubMed: 34762098
DOI: 10.1001/jamaophthalmol.2021.4732 -
Biomaterials Science Sep 2022Retinoblastoma is the most common primary intraocular malignancy in children. Although traditional chemotherapy has shown some success in retinoblastoma management,... (Review)
Review
Retinoblastoma is the most common primary intraocular malignancy in children. Although traditional chemotherapy has shown some success in retinoblastoma management, there are several shortcomings to this approach, including inadequate pharmacokinetic parameters, multidrug resistance, low therapeutic efficiency, nonspecific targeting, and the need for adjuvant therapy, among others. The revolutionary developments in biomaterials for drug delivery have enabled breakthroughs in cancer management. Today, biomaterials are playing a crucial role in developing more efficacious retinoblastoma treatments. The key goal in the evolution of drug delivery biomaterials for retinoblastoma therapy is to resolve delivery-associated obstacles and lower nonlocal exposure while ameliorating certain adverse effects. In this review, we will first delve into the historical perspective of retinoblastoma with a focus on the classical treatments currently used in clinics to enhance patients' quality of life and survival rate. As we move along, we will discuss biomaterials for drug delivery applications. Various aspects of biomaterials for drug delivery will be dissected, including their features and recent advances. In accordance with the current advances in biomaterials, we will deliver a synopsis on the novel chemotherapeutic drug delivery strategies and evaluate these approaches to gain new insights into retinoblastoma treatment.
Topics: Biocompatible Materials; Child; Combined Modality Therapy; Humans; Quality of Life; Retinal Neoplasms; Retinoblastoma
PubMed: 35959730
DOI: 10.1039/d2bm01005d -
Genes Nov 2019The gene is the first described human tumor suppressor gene and plays an integral role in the development of retinoblastoma, a pediatric malignancy of the eye. Since... (Review)
Review
The gene is the first described human tumor suppressor gene and plays an integral role in the development of retinoblastoma, a pediatric malignancy of the eye. Since its discovery, the stepwise characterization and cloning of have laid the foundation for numerous advances in the understanding of tumor suppressor genes, retinoblastoma tumorigenesis, and inheritance. Knowledge of led to a paradigm shift in the field of cancer genetics, including widespread acceptance of the concept of tumor suppressor genes, and has provided crucial diagnostic and prognostic information through genetic testing for patients affected by retinoblastoma. This article reviews the long history of gene research, characterization, and cloning, and also discusses recent advances in retinoblastoma genetics that have grown out of this foundational work.
Topics: Biomarkers, Tumor; Cloning, Molecular; Genes, Tumor Suppressor; Humans; Retinoblastoma; Retinoblastoma Binding Proteins; Ubiquitin-Protein Ligases
PubMed: 31683923
DOI: 10.3390/genes10110879 -
Journal of Pediatric Ophthalmology and... 2024
Topics: Humans; Retinoblastoma; Penetrance; Retinal Neoplasms; Pedigree; Mutation
PubMed: 37489625
DOI: 10.3928/01913913-20230427-03 -
Nepalese Journal of Ophthalmology : a... Jul 2021We read with keen interest the article by Shah et al "Pathologic Risk Factor in Retinoblastoma: An Institutional Experience Based on Analysis of Enucleated Eyes"(Shah A...
We read with keen interest the article by Shah et al "Pathologic Risk Factor in Retinoblastoma: An Institutional Experience Based on Analysis of Enucleated Eyes"(Shah A et al, 2012). They have highlighted high-risk histopathological features in Retinoblastoma with retrolaminar optic nerve invasion being the most common and also showed statistically significant correlation of high risk histological features with tumor size and AJCC stage of tumor. Their aim to assess the frequency and spectrum of high risk histopathological features in enucleated specimens of retinoblastoma was successful which may guide the clinician in timely planning for subsequent neoadjuvant therapy and prevent further ocular morbidity and mortality in children. We would like to congratulate authors for this article and with invoked interest it led us to go in more depth and we wish to discuss several aspects of the study that can potentially influence the results.
Topics: Child; Eye Enucleation; Humans; Infant; Neoplasm Invasiveness; Retinal Neoplasms; Retinoblastoma; Retrospective Studies
PubMed: 35996791
DOI: 10.3126/nepjoph.v13i2.39752 -
Health Expectations : An International... Jun 2020We launched a patient engagement strategy to facilitate research involvement of the retinoblastoma (childhood eye cancer) community in Canada. To inform our strategy, we...
BACKGROUND
We launched a patient engagement strategy to facilitate research involvement of the retinoblastoma (childhood eye cancer) community in Canada. To inform our strategy, we aimed to uncover the experiences with retinoblastoma, knowledge of retinoblastoma and research engagement among retinoblastoma survivors and parents.
METHODS
Focus groups were held in Toronto and Calgary, including both in-person and remote participants (via videoconference). Discussions centred on experience with retinoblastoma, knowledge of the disease and engagement with research. Focus group transcripts were evaluated by inductive thematic analysis.
RESULTS
Four focus groups (3 in Toronto, 1 in Calgary) were held with a collective total of 34 participants. Retinoblastoma had a substantial impact on the life of participants, but overall, patients reported being able to adapt and persevere. Experiential knowledge of retinoblastoma was identified as distinct from the theoretical knowledge held by their clinicians. Participants indicated they often acted as a knowledge broker, communicating information about the cancer to their social networks. Participants were willing to engage in research as partners, but recognized barriers such as time and appropriate training.
CONCLUSIONS
Patients view their experiential knowledge of retinoblastoma as valuable to improving care and directing research. There is a unique role for research engagement in meeting the educational needs of patients.
Topics: Child; Humans; Parents; Patient Participation; Qualitative Research; Retinal Neoplasms; Retinoblastoma
PubMed: 32113195
DOI: 10.1111/hex.13043 -
Pediatric Blood & Cancer May 2021The role of surgery, chemotherapy, and radiation therapy for retinoblastoma has evolved considerably over the years with the efficacy of intraarterial chemotherapy and... (Review)
Review
The role of surgery, chemotherapy, and radiation therapy for retinoblastoma has evolved considerably over the years with the efficacy of intraarterial chemotherapy and the high incidence of secondary malignant neoplasms following radiation therapy. The use of spot scanning intensity-modulated proton therapy may reduce the risk of secondary malignancies. For pediatric nasopharyngeal carcinoma, the current standard of care is induction chemotherapy followed by chemoradiation therapy. For adrenocortical carcinoma, the mainstay of treatment is surgery and chemotherapy. The role of radiation therapy remains to be defined.
Topics: Adrenal Cortex Neoplasms; Child; Combined Modality Therapy; Humans; Nasopharyngeal Neoplasms; Prognosis; Rare Diseases; Retinal Neoplasms; Retinoblastoma; Survival Rate
PubMed: 33818883
DOI: 10.1002/pbc.28253