Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 295
Filtrar
1.
Ophthalmology ; 127(2): 240-248, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31708274

RESUMO

PURPOSE: Cutaneous melanoma metastatic to the vitreous is very rare. This study investigated the clinical findings, treatment, and outcome of patients with metastatic cutaneous melanoma to the vitreous. Most patients received checkpoint inhibition for the treatment of systemic disease, and the significance of this was explored. DESIGN: Multicenter, retrospective cohort study. PARTICIPANTS: Fourteen eyes of 11 patients with metastatic cutaneous melanoma to the vitreous. METHODS: Clinical records, including fundus photography and ultrasound results, were reviewed retrospectively, and relevant data were recorded for each patient eye. MAIN OUTCOME MEASURES: Clinical features at presentation, ophthalmic and systemic treatments, and outcomes. RESULTS: The median age at presentation of ophthalmic disease was 66 years (range, 23-88 years), and the median follow-up from diagnosis of ophthalmic disease was 23 months. Ten of 11 patients were treated with immune checkpoint inhibition at some point in the treatment course. The median time from starting immunotherapy to ocular symptoms was 17 months (range, 4.5-38 months). Half of eyes demonstrated amelanotic vitreous debris. Five eyes demonstrated elevated intraocular pressure, and 4 eyes demonstrated a retinal detachment. Six patients showed metastatic disease in the central nervous system. Ophthalmic treatment included external beam radiation (30-40 Gy) in 6 eyes, intravitreous melphalan (10-20 µg) in 4 eyes, enucleation of 1 eye, and local observation while receiving systemic treatment in 2 eyes. Three eyes received intravitreous bevacizumab for neovascularization. The final Snellen visual acuity ranged from 20/20 to no light perception. CONCLUSIONS: The differential diagnosis of vitreous debris in the context of metastatic cutaneous melanoma includes intravitreal metastasis, and this seems to be particularly apparent during this era of treatment with checkpoint inhibition. External beam radiation, intravitreous melphalan, and systemic checkpoint inhibition can be used in the treatment of ophthalmic disease. Neovascular glaucoma and retinal detachments may occur, and most eyes show poor visual potential. Approximately one quarter of patients demonstrated ocular disease that preceded central nervous system metastasis. Patients with visual symptoms or vitreous debris in the context of metastatic cutaneous melanoma would benefit from evaluation by an ophthalmic oncologist.

2.
Nat Med ; 25(12): 1839-1842, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31768065

RESUMO

Histiocytoses are clonal hematopoietic disorders frequently driven by mutations mapping to the BRAF and MEK1 and MEK2 kinases. Currently, however, the developmental origins of histiocytoses in patients are not well understood, and clinically meaningful therapeutic targets outside of BRAF and MEK are undefined. In this study, we uncovered activating mutations in CSF1R and rearrangements in RET and ALK that conferred dramatic responses to selective inhibition of RET (selpercatinib) and crizotinib, respectively, in patients with histiocytosis.


Assuntos
Quinase do Linfoma Anaplásico/genética , Histiocitose/genética , Proteínas Proto-Oncogênicas c-ret/genética , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos/genética , Adolescente , Adulto , Aminopiridinas/farmacologia , Benzotiazóis/farmacologia , Criança , Pré-Escolar , Feminino , Genoma Humano , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/patologia , Histiocitose/tratamento farmacológico , Histiocitose/patologia , Humanos , Lactente , Masculino , Mutação , Ácidos Picolínicos/farmacologia , Inibidores de Proteínas Quinases/farmacologia , Pirazóis/farmacologia , Piridinas/farmacologia , Pirróis/farmacologia , Receptores Proteína Tirosina Quinases/genética , Gêmeos Monozigóticos , Sequenciamento Completo do Exoma , Adulto Jovem
3.
Palliat Support Care ; : 1-10, 2019 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-31699178

RESUMO

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. METHODS: Qualitative 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. RESULTS: Four 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 RESULTS: This 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.

4.
BMC Health Serv Res ; 19(1): 818, 2019 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-31703682

RESUMO

BACKGROUND: While Medicare is a federal health insurance program, managed Medicare limits access to healthcare services to networks within states or territories. However, if a natural disaster requires evacuation, displaced patients are at risk of losing coverage for their benefits. Previous literature has discussed the quality of managed Medicare plans within Puerto Rico but has not addressed the adequacy of this coverage if residents are displaced to the continental United States. We explore Hurricane Maria's impact on a resident of Puerto Rico with chronic health problems, and the challenges he faces seeking healthcare in New York. CASE PRESENTATION: A 59-year-old male with a history of diabetes mellitus type II, coronary artery disease, peripheral vascular disease status post right foot amputation, and end-stage kidney disease on hemodialysis was admitted in October of 2017 for chest pain and swelling of legs for 5 days. The patient had missed his last three dialysis sessions after Hurricane Maria forced him to leave Puerto Rico. In examining this patient's treatment, we observe the effect of Hurricane Maria on the medical management of Puerto Rican residents and identify challenges managed Medicare may pose to patients who cross state or territory lines. CONCLUSIONS: We employ this patient's narrative to frame a larger discussion of Puerto Rican managed Medicare and provide additional recommendations for healthcare providers. Moreover, we consider this case in the context of disaster-related continuity of care for patients with complex medical conditions or treatment regimens. To address the gaps in the care of these patients, this article proposes (1) developing system-based approaches for screening displaced patients, (2) increasing the awareness of Special Enrollment Periods related to Medicare among healthcare providers, and (3) creating policy solutions to assure access to care for patients with complex medical conditions.

5.
J Clin Oncol ; 37(35): 3436-3445, 2019 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-31622129

RESUMO

PURPOSE: Survivors of hereditary retinoblastoma have excellent survival but substantially increased risks of subsequent bone and soft-tissue sarcomas, particularly after radiotherapy. Comprehensive investigation of sarcoma risk patterns would inform clinical surveillance for survivors. PATIENTS AND METHODS: In a cohort of 952 irradiated survivors of hereditary retinoblastoma who were originally diagnosed during 1914 to 2006, we quantified sarcoma risk with standardized incidence ratios (SIRs) and cumulative incidence analyses. We conducted analyses separately for bone and soft-tissue sarcomas occurring in the head and neck (in/near the radiotherapy field) versus body and extremities (out of field). RESULTS: Of 105 bone and 124 soft-tissue sarcomas, more than one half occurred in the head and neck (bone, 53.3%; soft tissue, 51.6%), one quarter in the body and extremities (bone, 29.5%; soft tissue, 25.0%), and approximately one fifth in unknown/unspecified locations (bone, 17.1%; soft tissue, 23.4%). We noted substantially higher risks compared with the general population for head and neck versus body and extremity tumors for both bone (SIR, 2,213; 95% CI, 1,671 to 2,873 v SIR, 169; 95% CI, 115 to 239) and soft-tissue sarcomas (SIR, 542; 95% CI, 418 to 692 v SIR, 45.7; 95% CI, 31.1 to 64.9). Head and neck bone and soft-tissue sarcomas were diagnosed beginning in early childhood and continued well into adulthood, reaching a 60-year cumulative incidence of 6.8% (95% CI, 5.0% to 8.7%) and 9.3% (95% CI, 7.0% to 11.7%), respectively. In contrast, body and extremity bone sarcoma incidence flattened after adolescence (3.5%; 95% CI, 2.3% to 4.8%), whereas body and extremity soft-tissue sarcoma incidence was rare until age 30, when incidence rose steeply (60-year cumulative incidence, 6.6%; 95% CI, 4.1% to 9.2%), particularly for females (9.4%; 95% CI, 5.1% to 13.8%). CONCLUSION: Strikingly elevated bone and soft-tissue sarcoma risks differ by age, location, and sex, highlighting important contributions of both radiotherapy and genetic susceptibility. These data provide guidance for the development of a risk-based screening protocol that focuses on the highest sarcoma risks by age, location, and sex.

6.
J Thorac Oncol ; 14(11): 1989-1994, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31323388

RESUMO

INTRODUCTION: Inherited mutations are easily detected factors that influence the disease courses and optimal treatment strategies of some cancers. Germline mutations in BRCA1 associated protein 1 (BAP1) are associated with unique disease profiles in mesothelioma, atypical spitz nevi, and uveal melanoma, but the patient characteristics of an unselected population of BAP1 carriers identified by an ascertainment prevalence study are unknown. METHODS: We collected blood samples, cancer histories, and occupational exposures from 183 unselected patients with BAP1-related diseases. Clinical information for each patient was obtained from medical records. Germline DNA was extracted from blood samples and sequenced using a next-generation sequencing assay. We tested screening criteria developed to identify patients with a possible germline BAP1 mutation. RESULTS: Pathogenic or likely pathogenic germline BAP1 mutations were observed in 5 of 180 sequenced specimens and were exclusively found in patients identified by our screening criteria. Several patients with characteristics suspicious for a heritable deleterious mutation did not have a germline BAP1 mutation. The prevalence of pathogenic germline BAP1 mutations in patients with mesothelioma was 4.4% (95% confidence interval 1.1-11.1). CONCLUSIONS: Results from the first unselected prevalence ascertainment study of germline BAP1 alterations suggest that the frequency of this mutation is low among patients with mesothelioma. The proposed screening criteria successfully identified all patients with germline BAP1-mutant mesothelioma. These screening guidelines may assist physicians in selecting patients who would benefit from genetic testing. Future efforts should validate and refine these criteria and search for other germline mutations associated with mesothelioma and related diseases.

7.
Risk Anal ; 39(12): 2683-2693, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31290166

RESUMO

Perceptions of infectious diseases are important predictors of whether people engage in disease-specific preventive behaviors. Having accurate beliefs about a given infectious disease has been found to be a necessary condition for engaging in appropriate preventive behaviors during an infectious disease outbreak, while endorsing conspiracy beliefs can inhibit preventive behaviors. Despite their seemingly opposing natures, knowledge and conspiracy beliefs may share some of the same psychological motivations, including a relationship with perceived risk and self-efficacy (i.e., control). The 2015-2016 Zika epidemic provided an opportunity to explore this. The current research provides some exploratory tests of this topic derived from two studies with similar measures, but different primary outcomes: one study that included knowledge of Zika as a key outcome and one that included conspiracy beliefs about Zika as a key outcome. Both studies involved cross-sectional data collections that occurred during the same two periods of the Zika outbreak: one data collection prior to the first cases of local Zika transmission in the United States (March-May 2016) and one just after the first cases of local transmission (July-August). Using ordinal logistic and linear regression analyses of data from two time points in both studies, the authors show an increase in relationship strength between greater perceived risk and self-efficacy with both increased knowledge and increased conspiracy beliefs after local Zika transmission in the United States. Although these results highlight that similar psychological motivations may lead to Zika knowledge and conspiracy beliefs, there was a divergence in demographic association.

8.
Artigo em Inglês | MEDLINE | ID: mdl-31259824

RESUMO

We attempted to investigate the potential role for apparent diffusion coefficient (ADC) to diagnose trilateral retinoblastoma (TRb) by retrospectively reviewing brain magnetic resonance images of retinoblastoma patients. Observations: The median ADC measured 620.95 for TRb (n=6) and 1238.5 for normal pineal gland in bilateral retinoblastoma (n=8). Monitoring ADC trends aided in establishing the appropriate diagnoses in 3 patients (2 TRb, 1 benign pineal cyst). Conclusions: Our results provide baseline reference data and describe the importance of downward trending ADC which should prompt consideration of TRb. Unchanged high/nonrestricted values (>1000) may distinguish those with benign pineal tissue and obviate invasive neurosurgical procedures.

9.
Ocul Oncol Pathol ; 5(3): 186-189, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31049326

RESUMO

Background/Aims: The treatment of orbital melanoma poses a management challenge. This case explores the delivery of high-dose melphalan to an orbital recurrence of uveal melanoma via intra-arterial delivery of melphalan to the orbit. A 62-year-old man developed recurrent orbital disease 7 months after enucleation for a large uveal melanoma. He received 6 monthly intra-arterial infusions of melphalan to the orbit, ranging in dose from 20 to 30 mg per infusion. Following the last infusion, mild temporary erythema was noted on the forehead along the distribution of the supratrochlear artery. The orbital recurrence was reduced in size by 66% in the longest dimension as measured by magnetic resonance imaging (MRI). However, 9 months following intra-arterial melphalan, tumor regrowth was detected on MRI, and additional treatment options were pursued. Conclusion: This case demonstrates that intra-arterial melphalan can result in nonsustained tumor regression of recurrent orbital uveal melanoma. It suggests that local delivery of high-dose melphalan may be helpful as a neoadjuvant treatment for uveal melanoma, and future studies will be useful to confirm the value of this approach in additional cases of recurrent and possibly in primary uveal melanoma.

10.
Ophthalmic Surg Lasers Imaging Retina ; 50(4): 201-207, 2019 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-30998240

RESUMO

BACKGROUND AND OBJECTIVE: Aggressive posterior vitreoretinopathy (APVR) manifests with a broad area of retinal avascularity, progressive neovascularization, and/or tractional retinal detachment during the neonatal period. PATIENTS AND METHODS: A multicenter, retrospective, observational, consecutive case series study was performed to evaluate the retinal findings and structural retinal outcomes in patients treated for APVR within the first 3 months of life. RESULTS: Three premature neonates with a non-retinopathy of prematurity (ROP) APVR identified during routine ROP screening exams exhibited relatively severe, rapidly progressive retinal vascular abnormalities. Immediate laser photocoagulation of the avascular retina and vitrectomy for traction retinal detachment within several days to weeks improved or stabilized the retinal anatomy in all cases. CONCLUSIONS: This series describes clinical features in APVR in premature infants and suggests that early diagnosis and intervention may mitigate the typical aggressive course and poor prognosis of this condition. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:201-207.].


Assuntos
Inibidores da Angiogênese/administração & dosagem , Diagnóstico Precoce , Angiofluoresceinografia/métodos , Recém-Nascido Prematuro , Terapia a Laser/métodos , Vitrectomia/métodos , Vitreorretinopatia Proliferativa/diagnóstico , Gerenciamento Clínico , Feminino , Fundo de Olho , Idade Gestacional , Humanos , Recém-Nascido , Injeções Intravítreas , Masculino , Prognóstico , Estudos Retrospectivos , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores , Acuidade Visual , Vitreorretinopatia Proliferativa/tratamento farmacológico , Vitreorretinopatia Proliferativa/cirurgia
11.
Methods Mol Biol ; 1958: 123-131, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30945216

RESUMO

We recently developed an unsupervised Bayesian inference methodology to automatically infer a dictionary of protein supersecondary structures (Subramanian et al., IEEE data compression conference proceedings (DCC), 340-349, 2017). Specifically, this methodology uses the information-theoretic framework of minimum message length (MML) criterion for hypothesis selection (Wallace, Statistical and inductive inference by minimum message length, Springer Science & Business Media, New York, 2005). The best dictionary of supersecondary structures is the one that yields the most (lossless) compression on the source collection of folding patterns represented as tableaux (matrix representations that capture the essence of protein folding patterns (Lesk, J Mol Graph. 13:159-164, 1995). This book chapter outlines our MML methodology for inferring the supersecondary structure dictionary. The inferred dictionary is available at http://lcb.infotech.monash.edu.au/proteinConcepts/scop100/dictionary.html .


Assuntos
Motivos de Aminoácidos , Biologia Computacional/métodos , Proteínas/química , Algoritmos , Teorema de Bayes , Compressão de Dados , Humanos , Modelos Moleculares , Dobramento de Proteína
14.
Int J Mol Sci ; 20(5)2019 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-30832308

RESUMO

A preclinical model could aid in understanding retinoblastoma vitreous seeds behavior, drug penetration, and response to chemotherapy to optimize patient treatment. Our aim was to develop a tridimensional in vitro model of retinoblastoma vitreous seeds to assess chemotherapy penetration by means of live-cell imaging. Cell cultures from patients with retinoblastoma who underwent upfront enucleation were established and thoroughly characterized for authentication of human tumor origin. The correlation of the in vitro tridimensional structures resembling human spheres and dusts vitreous seeds was established. Confocal microscopy was used to quantify real-time fluorescence of topotecan as a measure of its penetration into different sizes of spheres. Cell viability was determined after chemotherapy penetration. The in vitro spheres and dusts models were able to recapitulate the morphology, phenotype, and genotype of patient vitreous seeds. The larger the size of the spheres, the longer the time required for the drug to fully penetrate into the core (p < 0.05). Importantly, topotecan penetration correlated with its cytotoxic activity. Therefore, the studied tridimensional cell model recapitulated several characteristics of vitreous seeds observed in patients with retinoblastoma and were successfully used to assess live-cell imaging of chemotherapy penetration for drug distribution studies.


Assuntos
Antineoplásicos/farmacologia , Organoides/efeitos dos fármacos , Retinoblastoma/tratamento farmacológico , Topotecan/farmacologia , Antineoplásicos/uso terapêutico , Linhagem Celular Tumoral , Células Cultivadas , Ensaios de Seleção de Medicamentos Antitumorais/métodos , Humanos , Imagem Tridimensional/métodos , Organoides/diagnóstico por imagem , Cultura Primária de Células/métodos , Topotecan/uso terapêutico
15.
Retina ; 2019 Jan 09.
Artigo em Inglês | MEDLINE | ID: mdl-30633085

RESUMO

PURPOSE: To evaluate the subfoveal choroidal thickness (SFCT) and vascular architecture in the fellow eyes of patients with circumscribed choroidal hemangioma (CCH). METHODS: In this retrospective observational study, patients were selected from outpatient ophthalmology clinics at the Memorial Sloan Kettering Cancer Center and Vitreous Retina Macula Consultants of New York. Subfoveal choroidal thickness was measured using enhanced depth imaging spectral domain optical coherence tomography from the outer portion of Bruch membrane to the choroidal-scleral interface. Choroidal vascular architecture was qualitatively examined. The main outcome measure was SFCT in fellow eyes of patients with CCH, which was compared with an age- and gender-matched control group. RESULTS: Thirty-one fellow eyes (15 right eyes and 16 left eyes) of patients with CCH (23 males and 8 females) were examined. The fellow eye had a mean SFCT of 361.2 ± 99.9 µm compared with 252.0 ± 77.6 µm in the control group (P < 0.0001). Vascular architecture was disorganized in 13 (42%) fellow eyes and 1 (3%) control eye (P < 0.0001), with no apparent gradient of vessel sizes or discrete choroidal layers. The normal association between older age and a thinner choroid existed in control eyes but not in fellow eyes. Hemangioma thickness measured by ultrasound and the presence of subfoveal fluid in the CCH eye did not correlate with the fellow-eye SFCT. CONCLUSION: In patients with CCH, fellow eyes had thicker SFCT when compared with age- and gender-matched control eyes. Choroidal architecture was often irregular, without segmented vascular layers. These findings suggest that inherent choroidal changes may exist in patients with CCH.

16.
J Natl Cancer Inst ; 111(9): 961-969, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-30698734

RESUMO

BACKGROUND: Previous studies of hereditary retinoblastoma survivors have reported elevated mortality, particularly for sarcomas, compared with the general population. However, cause-specific mortality patterns for long-term hereditary and nonhereditary retinoblastoma survivors are poorly understood. METHODS: Among 2053 retinoblastoma patients diagnosed during 1914-2006 at two major US treatment centers and followed to 2016, we estimated cumulative mortality, standardized mortality ratios (SMRs), and absolute excess risks (AERs) compared with the US general population. RESULTS: Most deaths occurred in 1129 hereditary retinoblastoma patients (n = 518 deaths, cumulative mortality 70 years after retinoblastoma = 75.8%, 95% CI = 69.0% to 82.6%; SMR = 8.5, 95% CI = 7.7 to 9.2). Of these, 267 were due to subsequent cancers (SMR = 27.4, 95% CI = 24.2 to 30.9; AER = 72.3 deaths/10 000 person-years), for which SMRs were highest 15-29 years after diagnosis (n = 69, SMR = 89.9, 95% CI = 70.0 to 113.8) but remained statistically significantly elevated at 60 and more years (n = 14, SMR = 6.7, 95% CI = 3.6 to 11.2), whereas AERs increased with time (AER<15years = 38.0; AER60+years = 327.5). Increased risk of death due to cancers of pancreas, large intestines, and kidney were noted for the first time. Overall risk of subsequent cancers was greater for those treated with radiotherapy and chemotherapy compared to radiotherapy alone, although patterns varied by organ site. For 924 patients with nonhereditary retinoblastoma, we noted a modestly increased risk of death for subsequent cancers (n = 27, SMR = 1.8, 95% CI = 1.2 to 2.6) possibly due to treatment or misclassification of hereditary status. Risks of noncancer causes of death were not elevated for hereditary or nonhereditary patients. CONCLUSION: Hereditary retinoblastoma survivors died mainly from an excess risk of subsequent cancers up to six decades later, highlighting the need to develop long-term clinical management guidelines for hereditary retinoblastoma survivors treated in the past.

17.
PLoS One ; 14(1): e0210647, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30650131

RESUMO

IMPORTANCE: Predictions of visual outcomes are useful in clinical and family decisions regarding treatment for retinoblastoma. Very little has been published on the association of post-treatment visual acuity with pre-treatment electroretinography (ERG), which can be performed on infants too young to reliably quantify visual acuity. OBJECTIVE: To report associations of pre-treatment ERG with post-treatment visual acuity in eyes with advanced retinoblastoma treated with ophthalmic artery chemosurgery (OAC). DESIGN: Retrospective case-control study of eyes treated from 2006 through 2017, with mean follow-up of 51 months (range 2.3-150 months). SETTING: Single large academic center. PARTICIPANTS: Group D and E eyes treated with OAC at Memorial Sloan Kettering Cancer Center with recorded visual acuity and ERG (30Hz flicker). MAIN OUTCOME AND MEASURE: Snellen visual acuity (uncorrected) compared to initial 30Hz flicker ERG. RESULTS: This study included 157 Group D and E eyes. Results of the Jonckheere-Terpstra test for trend were statistically significant and indicated that eyes with lower pre-treatment ERG readings tended to have more visual impairment post-treatment. Among eyes with initial ERG 75+ µV, 11 of 32 eyes (34%) had visual acuity 20/40 or better. Among eyes with ERG 0 µV, 44 of 46 (96%) had visual acuity of 20/200 or worse. CONCLUSIONS AND RELEVANCE: Eyes with advanced intraocular retinoblastoma treated with OAC can achieve excellent visual acuity, but poor ERG at initial visit is associated with poor visual acuity after treatment in the majority of eyes. Expectations regarding visual potential may influence decisions about treatment.


Assuntos
Eletrorretinografia/métodos , Neoplasias da Retina/diagnóstico por imagem , Neoplasias da Retina/cirurgia , Retinoblastoma/diagnóstico por imagem , Retinoblastoma/cirurgia , Carboplatina/uso terapêutico , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Melfalan/uso terapêutico , Neoplasias da Retina/tratamento farmacológico , Retinoblastoma/tratamento farmacológico , Estudos Retrospectivos , Topotecan/uso terapêutico , Acuidade Visual/fisiologia
18.
Br J Ophthalmol ; 103(9): 1272-1277, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30361279

RESUMO

BACKGROUND/AIMS: Enucleation for retinoblastoma is performed less often in the past decade due to increasingly widespread alternative therapies, but enucleation remains an important option. There is a paucity of reports on the current incidence of metastases and metastatic deaths in unilateral retinoblastoma from US centres. METHODS: Retrospective chart review at five tertiary retinoblastoma centres in the USA for unilateral retinoblastoma patients treated with primary enucleation, 2007-2017, with >1 year of follow-up or treatment failure. RESULTS: Among 228 patients (228 eyes), there were nine metastases (3.9%) and four deaths (1.7%). The Kaplan-Meier estimate at 5 years for metastasis-free survival was 96% (95% CI, 94% to 99 %), and for overall survival was 98% (95% CI 96% to 100%). All metastases were evident within 12 months. Histopathology revealed higher risk pathology (postlaminar optic nerve and/or massive choroidal invasion) in 62 of 228 eyes (27%). Of these higher risk eyes, 39 received adjuvant chemotherapy. There were four subsequent metastases in this higher risk pathology with adjuvant chemotherapy group, with three deaths. Of the nine overall with metastases, seven (78%) showed higher risk pathology. All metastatic patients were classified as Reese-Ellsworth V and International Classification of Retinoblastoma Groups D or E. Initial metastases presented as orbital invasion in seven of nine cases. CONCLUSIONS: Primary enucleation for unilateral retinoblastoma results in a low rate of metastatic death, but is still associated with a 3.9% chance of metastases within a year of enucleation. Most but not all patients who developed metastases had higher risk histopathological findings.

19.
Br J Ophthalmol ; 103(9): 1266-1271, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30381389

RESUMO

BACKGROUND: Metastases to the liver are often the first finding in patients with uveal melanoma with extraocular disease, but little has been published on the utility of staging MRI at initial diagnosis. We aimed to evaluate the proportion of abnormal hepatic findings on baseline MRI and accuracy of MRI in patients with newly diagnosed uveal melanoma. METHODS: This is a single-centre, retrospective, institutional review board-approved study of 145 consecutive patients diagnosed with uveal melanoma, at Memorial Sloan Kettering Cancer Center between 2004 and 2016, who had staging MRI within 1 month of diagnosis. Scans were classified as normal or abnormal, and further distinguished as abnormal non-metastatic, uncharacterisable lesions and suspicious for metastasis. Where available, follow-up MRI (at ~1 year) or biopsies were reviewed. RESULTS: MRI in 145 patients revealed 62% (90) with abnormal hepatic findings; out of these 87% (78) had non-metastatic benign findings, 6.7% (6) had unclassifiable lesions and 6.7% (6) were suspicious for metastasis (6). Abnormal non-metastatic findings included 72 focal (36 solitary and 36 multiple) and 12 diffuse lesions. Lesions suspicious for metastases were found in 6 of 145 patients (4%), despite normal liver function tests. Of these, five had confirmed liver metastases and one patient had a stable, presumed non-metastatic lesion on follow-up. In this study, the sensitivity and specificity of staging MRI for all findings were 83.3% (95% CI 35.9 to 99.6) and 99.0% (95% CI 94.3 to 99.9), respectively. CONCLUSION: Staging MRI of patients with newly diagnosed uveal melanoma accurately identified early metastases. Furthermore, imaging revealed hepatic abnormalities in the majority of patients, although as expected few of these represented metastatic disease.

20.
J Prosthet Dent ; 121(3): 535-537, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30554822

RESUMO

This article reviews the prosthodontic and laboratory steps in the fabrication of a custom brachytherapy appliance. This technique is described through the treatment of a patient with recurrent orbital rhabdomyosarcoma. After a non-eyelid-sparing orbital exenteration of a 7-year-old boy, an impression of the orbital defect was captured to fabricate a custom brachytherapy appliance with mock catheters. One week later, the prosthesis was placed in the orbit, and the device was loaded with radiation catheters to deliver targeted radiation. The prosthesis was secured with Velcro straps fitted into slots made in the prosthesis and wrapped around the back of the patient's head. Brachytherapy appliances have been used to provide effective therapy for patients receiving care at Memorial Sloan Kettering Cancer Center.


Assuntos
Braquiterapia , Neoplasias Orbitárias , Criança , Humanos , Masculino , Recidiva Local de Neoplasia , Órbita , Exenteração Orbitária
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA