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Impact of enucleation on adult retinoblastoma survivors’ quality of life: A qualitative study of survivors' perspectives
- Smita C. Banerjee, Elaine Pottenger, Mary Petriccione, Joanne F. Chou, Jennifer S. Ford, Charles A. Sklar, Leslie L. Robison, Ruth A. Kleinerman, Kevin C. Oeffinger, Jasmine H. Francis, David H. Abramson, Ira J. Dunkel, Danielle Novetsky Friedman
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- Journal:
- Palliative & Supportive Care / Volume 18 / Issue 3 / June 2020
- Published online by Cambridge University Press:
- 08 November 2019, pp. 322-331
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- Article
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Objectives
Retinoblastoma is the most common primary intraocular tumor of childhood with >95% survival rates in the US. Traditional therapy for retinoblastoma often included enucleation (removal of the eye). While much is known about the visual, physical, and cognitive ramifications of enucleation, data are lacking about survivors' perception of how this treatment impacts overall quality of life.
MethodsQualitative analysis of an open-ended response describing how much the removal of an eye had affected retinoblastoma survivors' lives and in what ways in free text, narrative form.
ResultsFour hundred and four retinoblastoma survivors who had undergone enucleation (bilateral disease = 214; 52% female; mean age = 44, SD = 11) completed the survey. Survivors reported physical problems (n = 205, 50.7%), intrapersonal problems (n = 77, 19.1%), social and relational problems (n = 98, 24.3%), and affective problems (n = 34, 8.4%) at a mean of 42 years after diagnosis. Three key themes emerged from survivors' responses; specifically, they (1) continue to report physical and intrapersonal struggles with appearance and related self-consciousness due to appearance; (2) have multiple social and relational problems, with teasing and bullying being prominent problems; and (3) reported utilization of active coping strategies, including developing more acceptance and learning compensatory skills around activities of daily living.
Significance of resultsThis study suggests that adult retinoblastoma survivors treated with enucleation continue to struggle with a unique set of psychosocial problems. Future interventions can be designed to teach survivors more active coping skills (e.g., for appearance-related issues, vision-related issues, and teasing/bullying) to optimize survivors' long-term quality of life.
26 - Retinoblastoma
- from PART II - CLINICAL RESEARCH
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- By Ira J. Dunkel, Memorial Sloan-Kettering Cancer Center, United States, David H. Abramson, Memorial Sloan-Kettering Cancer Center, United States
- Edited by David Lyden, Danny R. Welch, Bethan Psaila
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- Book:
- Cancer Metastasis
- Published online:
- 05 June 2012
- Print publication:
- 25 April 2011, pp 278-281
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- Chapter
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Summary
PATTERNS OF METASTATIC SPREAD, ORGAN SPECIFICITY, TIMING OF RECURRENT DISEASE, AND COMPLICATIONS CONFRONTED WITH METASTASIS
Retinoblastoma is the most common type of primary intraocular pediatric tumor. The incidence of retinoblastoma is approximately 1 in 20,000 live births, and it is estimated that there are about 300 to 350 new cases each year in the United States.
There are two forms of retinoblastoma. The most common form of retinoblastoma, known as unilateral disease, presents with a single tumor focus at a median age of about twenty-four months. Patients may also present with multiple tumor foci, usually in both eyes (bilateral disease), at a median age of about twelve months. The recognition of the earlier presentation of bilateral disease led to the classic two-hit hypothesis of retinoblastoma etiology [1]; in 1986 the “hit” was discovered to be a mutation in the RB1 gene on chromosome 13q14 [2]. It is currently believed that all patients with bilateral disease have a germline mutation in RB1, whereas most patients (about 85%) with unilateral disease have a sporadic mutation in the tumor cells only [3]. DNA sequencing to identify the mutation can be performed at specialized laboratories and is clinically useful to identify survivors of unilateral disease who may be at risk for other cancers later in life (secondary malignancy) and/or transmission of the disease to their offspring, to determine which relatives or offspring of survivors inherit the mutation and need to be carefully clinically screened, and to allow survivors with a germline mutation the option of considering preimplantation genetic diagnosis and in vitro fertilization to avoid the risk of transmitting the mutated RB1 to their offspring [4].