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1.
Ann Pathol ; 39(4): 286-291, 2019 Aug.
Artigo em Francês | MEDLINE | ID: mdl-30827691

RESUMO

OBJECTIVES: Describe the macroscopic and microscopic profiles of the retinoblastoma (RB) in Senegal and correlate histological criteria to evolution to emerge severity factors. METHODOLOGY: We realized a retrospective study on 10years (January 2005-December 2014). Only patients (n=67) who performed a histological analysis of the enucleation specimen (n=68) were followed until the end of the study. RESULTS: The tumor measured an average of 3.8cm (2/4.5). Endophytic tumor development was in 55 cases (80.8%), exophytic in 6 cases (8.9%) and mixed in 7 cases (10.3%). Extra-retinal form was present in 13 cases (19.2%). The RB was well differentiated in 11 cases (16.2%), moderately differentiated in 31 cases (45.6%) and undifferentiated in 26 cases. The invasion of optic nerve (ON) was present in 18 cases (26.5%). The tumor was extra-retinal in 37 cases (54.4%). The anterior chamber was invaded in 6 cases (8.8%). The global survival at 2years was 84% and 70% at 5years. In multivariate analysis, well tumor differentiation and stage pT1 were associated with remission. The other-laminar of ON invasion, the massive invasion of the choroid and pT3 stage were recurrence risk factors. The undifferentiating tumor, invasion of the ON section, tumor sclera crossing and pT4 stage were predictive death. CONCLUSION: The RB histological prognostic factors should be clearly identified in the pathological reports as they guide the after surgical period care and follow-up management strategy.


Assuntos
Neoplasias da Retina/patologia , Retinoblastoma/patologia , Feminino , Humanos , Masculino , Neoplasias da Retina/epidemiologia , Retinoblastoma/epidemiologia , Estudos Retrospectivos , Senegal/epidemiologia
2.
Tumour Biol ; 39(4): 1010428317695945, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28443472

RESUMO

MicroRNAs are non-coding short RNAs that target the 3' untranslated region of messenger RNAs (mRNAs) and lead to their degradation or to translational repression. Several microRNAs have been designated as oncomirs, owing to their regulating tumor suppressor genes. Interestingly, a few of them have been found to target multiple genes whose simultaneous suppression contributes to the development of a tumoral phenotype. Here, we have showed that miR-26a is overexpressed in colorectal cancer data obtained from TCGA Research Network and in human colon cancer pathological specimens; moreover, an orthotopic in vivo model of colon cancer showed overexpression of miR-26a, while Rb1 expression inversely correlated to miR-26a in TCGA Research Network data, pathological samples, and the in vivo model. Then, by means of luciferase assay, we demonstrated that miR-26a targets the 3' untranslated region of Rb1 mRNA directly. This is, to our knowledge, the first report of miR-26a targeting Rb1 in colon cancer. The results of this study suggested that miR-26a could serve as a progression biomarker in colorectal cancer. Further validation studies are still needed to confirm our findings.


Assuntos
Biomarcadores Tumorais/genética , Neoplasias Colorretais/genética , MicroRNAs/genética , Proteínas de Ligação a Retinoblastoma/biossíntese , Ubiquitina-Proteína Ligases/biossíntese , Regiões 3' não Traduzidas , Animais , Sítios de Ligação , Biomarcadores Tumorais/biossíntese , Linhagem Celular Tumoral , Neoplasias Colorretais/patologia , Regulação Neoplásica da Expressão Gênica , Células HCT116 , Humanos , Camundongos , MicroRNAs/biossíntese , RNA Mensageiro/biossíntese , RNA Mensageiro/genética , Proteínas de Ligação a Retinoblastoma/genética , Ubiquitina-Proteína Ligases/genética , Ensaios Antitumorais Modelo de Xenoenxerto
3.
J Fr Ophtalmol ; 47(9): 104258, 2024 Aug 29.
Artigo em Francês | MEDLINE | ID: mdl-39213923

RESUMO

The objective of this work is to assess the current situation in the ophthalmology department of the tertiary care center for the conservative treatment of retinoblastoma in Niger. This was a retrospective study from January 2016 to October 2022 (6years and 10months). Retinoblastoma represents 43.27% of pediatric cancers, of which 10.92% of cases are bilateral. The mean age of our patients is 36months, with the range between 2-3years, with extremes ranging from 1month to 132months. The male sex represents 65.55% (n=78), with a sex ratio of 1.9. The distance traveled varies from 1355 kilometers in the furthest region (Diffa) from the capital to 113 kilometers in the nearest region (Tillabéry). Neighboring countries such as Mali and Benin represent 5.88% (n=5). It should be noted that 58% saw traditional healers before coming to the medical center. The most common clinical signs are leukocoria 39.5% (n=47), proptosis 34.45% (n=41) and exorbitism in 15.97% (n=19). An orbital/brain CT was ordered in 54.62% (n=65) of cases. We used the TNM classification; extraocular extension occurred in 47.90% (n=57) and intraocular cases 27.73% (n=33). Neoadjuvant chemotherapy was used in (63.03% n=74). Histology was carried out in (26.89% n=32), with the result obtained in 14days on average. The visit to traditional practitioners as well as the remoteness of certain regions contribute to the delay in diagnosis in our context. The continued implementation of the early diagnosis campaign program could reverse the trend.

4.
J Fr Ophtalmol ; 46(3): 216-222, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36642598

RESUMO

PURPOSE: The goal of this study is to describe the presence of secondary cataract in patients with retinoblastoma treated at the National Institute of Pediatrics of Mexico (INP) over the past 10 years. METHODS: This was a single center observational, retrospective and descriptive study. We included all eyes diagnosed with retinoblastoma and cataract between June 2011 and June 2021. RESULTS: In total, 833 records of patients diagnosed with Retinoblastoma at the National Institute of Pediatrics during the period between June 2011 and June 2021 were reviewed. Out of all of them, only 14 developed cataract (1.6%). The median age at retinoblastoma diagnosis was 10.5 months (Rank: 6-13 months), and the median age at cataract diagnosis was 51.5 months (Rank: 25-73 months). The majority (13, 92.9%) of the patients had bilateral involvement. 42% of the eyes were Stage D according to the international classification of retinoblastoma. Cryotherapy was applied in 57.1%, intravitreal chemotherapy in 85.7%, radiation therapy in 42.6%, and only 7.1% of cases were treated with intra-arterial chemotherapy. CONCLUSIONS: The presence of cataract in patients with retinoblastoma is a rare but important entity impacting the development of vision in children and detection of intraocular tumors. These probably occur late as a result of the multiple treatments to which the children have been subjected, without being able to determine in this study which is the risk factor most associated with the development of this pathology.


Assuntos
Catarata , Pediatria , Neoplasias da Retina , Retinoblastoma , Criança , Humanos , Lactente , Pré-Escolar , Retinoblastoma/complicações , Retinoblastoma/diagnóstico , Retinoblastoma/epidemiologia , Neoplasias da Retina/complicações , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/epidemiologia , Estudos Retrospectivos , México/epidemiologia , Resultado do Tratamento , Catarata/diagnóstico , Catarata/epidemiologia , Catarata/etiologia
5.
J Fr Ophtalmol ; 45(7): 762-766, 2022 Sep.
Artigo em Francês | MEDLINE | ID: mdl-35753853

RESUMO

INTRODUCTION: Retinoblastoma is a curable cancer when management is early and prompt. OBJECTIVE: To describe the epidemiological, clinical and therapeutic aspects of retinoblastoma. MATERIALS AND METHODS: A retrospective study was carried out from January 2014 to December 2018 (5 years) at the department of ophthalmology in Campus-University Teaching Hospital, Lomé, Togo. It focused on children with retinoblastoma. It included patients with diagnosis supported by ocular ultrasonography, orbito-cerebral computed tomography scan and/or histopathological examination. RESULTS: Among the 75 children with ocular cancer, 26 (34.7%) were diagnosed with retinoblastoma. The mean age at presentation was 31 months. The mean time from symptom onset to diagnosis was 15 months. A predominance of proptosis (65.4%), unilateral involvement (76.9%), extraocular tumor extension (65.4%) and endophytic form (96.15%) was found. Of the 32 eyes with retinoblastoma, 7 (21.9%) were enucleated after a mean time from diagnosis to enucleation of 6 months. Two cases of complete remission, 3 cases of tumor recurrence, and 2 cases lost to follow-up were noted. CONCLUSION: In our setting, retinoblastoma is a frequent childhood ocular cancer. It is important to improve the management of retinoblastoma as well as education of the parents.


Assuntos
Neoplasias Oculares , Neoplasias da Retina , Retinoblastoma , Criança , Hospitais de Ensino , Humanos , Recidiva Local de Neoplasia , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/epidemiologia , Neoplasias da Retina/terapia , Retinoblastoma/diagnóstico , Retinoblastoma/epidemiologia , Retinoblastoma/terapia , Estudos Retrospectivos , Togo/epidemiologia , Universidades
6.
J Fr Ophtalmol ; 42(10): 1085-1089, 2019 Dec.
Artigo em Francês | MEDLINE | ID: mdl-31561918

RESUMO

INTRODUCTION: Retinoblastoma is a malignant tumor of neuroepithelial origin, developed from young retinal cells, occurring in infants and young children. The goal of the study was to assess the role of CT in the diagnosis of retinoblastoma at the Aristide le Dantec Hospital in Dakar. PATIENTS AND METHODS: This is an 11-year retrospective study of 160 patient records in the ophthalmology department and pediatric oncology unit of the same hospital. RESULTS: One hundred and sixty (160) patients were recruited. The male:female ratio was 1.05. The mean age was 32.19 months. Leukocoria was the most common clinical sign, occurring in 105 cases (65.62 %). The retinoblastoma was intraocular in 97 cases (60.62 %). We saw 122 (76.25 %) unilateral and 3 (1.87 %) trilateral presentations. CT was performed in 150 children with 110 cases (73.33 %) of calcifications ; 62 cases (41.33 %) of optic nerve invasion ; 24 cases (16 %) of extraocular muscle invasion and 18 cases (12 %) endocranial extension. Associated tumors were found on CT: 2 cases of suprasellar mass and 1 case of pineoblastoma. In patients whose specimens were analyzed, histology showed 48.15 % optic nerve invasion, consistent with the CT findings. DISCUSSION: CT has an important role in the diagnosis of retinoblastoma, despite its recent contraindication in bilateral and unilateral multinodular forms. CONCLUSION: CT is a good alternative to MRI in the diagnosis of retinoblastoma in developing countries with limited technological resources.


Assuntos
Técnicas de Diagnóstico Oftalmológico , Monitorização Fisiológica/métodos , Neoplasias da Retina/diagnóstico , Retinoblastoma/diagnóstico , Tomografia Computadorizada por Raios X , Criança , Pré-Escolar , Técnicas de Diagnóstico Oftalmológico/estatística & dados numéricos , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética/estatística & dados numéricos , Masculino , Monitorização Fisiológica/estatística & dados numéricos , Valor Preditivo dos Testes , Neoplasias da Retina/epidemiologia , Neoplasias da Retina/patologia , Retinoblastoma/epidemiologia , Retinoblastoma/patologia , Estudos Retrospectivos , Senegal/epidemiologia , Tomografia Computadorizada por Raios X/estatística & dados numéricos
7.
J Mycol Med ; 28(1): 215-217, 2018 Mar.
Artigo em Francês | MEDLINE | ID: mdl-29551441

RESUMO

Human cutaneous infections due to Rhodotorula species as an emerged pathogens yeasts are rarely reported, particularly in skin folds, with the ability to infect immunocompromised as well as immunocompetent patient. In present case, we describe a fungal cutaneous infection due to Rhodotorula mucilaginosa in immunocompromised child, the patient has been diagnosed with retinoblastoma; the diagnosis was established by the cytological and clinical examination. However, the cytological examination found fungal forms. The diagnosis relies on the microbiological analysis of clinical samples but the interpretation of a positive culture should be part of and over all analysis that integrate clinical symptoms and risk factors. Most of cases of infection due to Rhodotorula were associated with central venous catheter in patients who were receiving immunosuppressors and cyotoxic drugs. The antifungal susceptibility testing was done; a fluconazol and ketoconazol showed susceptibles. The patient was treated by combinate antifungal therapy and empirical antimicrobial regimen for prophylaxis.

8.
J Fr Ophtalmol ; 39(9): 739-743, 2016 Nov.
Artigo em Francês | MEDLINE | ID: mdl-27765445

RESUMO

OBJECTIVES: Describe the macroscopic and microscopic profiles of retinoblastoma (RB) in Senegal and correlate histological criteria with progression to establish severity factors. METHODOLOGY: We realized a retrospective study over 10 years (January 2005-December 2014). Only patients (n=67) who underwent histological analysis of the enucleation specimen (n=68) were followed until the end of the study. RESULTS: The tumor measured an average of 3.8cm (2/4.5). Endophytic tumor development was observed in 55 cases (80.8%), exophytic in 6 cases (8.9%) and mixed in 7 cases (10.3%). Retinal detachment was present in 13 cases (19.2%). The RB was well differentiated in 11 cases (16.2%), moderately differentiated in 31 cases (45.6%) and undifferentiated in 26 cases. Optic nerve (ON) invasion was present in 18 cases (26.5%). The tumor was extraretinal in 37 cases (54.4%). The anterior chamber was invaded in 6 cases (8.8%). The global survival at 2 years was 84% and 70% at 5 years. On multivariate analysis, well differentiated tumors and stage pT1 were associated with remission. Retrolaminar ON invasion, massive choroidal invasion and stage pT3 were risk factors for recurrence. Poor tumor differentiation, invasion of the ON resection margin, tumor invasion through the sclera, and stage pT4 were predictive of death. CONCLUSION: The pathologist is a principal actor in the management of RB because his account guide the post-surgical management strategy.


Assuntos
Neoplasias da Retina/patologia , Retinoblastoma/patologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Invasividade Neoplásica , Recidiva Local de Neoplasia/epidemiologia , Neoplasias da Retina/epidemiologia , Retinoblastoma/epidemiologia , Estudos Retrospectivos , Senegal/epidemiologia , Índice de Gravidade de Doença , Análise de Sobrevida
9.
J Fr Ophtalmol ; 38(6): 535-41, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25976136

RESUMO

INTRODUCTION: To describe the results of retinoblastoma treatment from 1995-2009 in a single institution. MATERIAL AND METHODS: Retrospective review of the charts of patients treated for retinoblastoma. Clinical characteristics at diagnosis, treatments and outcomes in terms of survival and ocular preservation are described. RESULTS: During the study period 826 children were referred for retinoblastoma and 730 were managed in our institution. Four hundred and eleven children presented with unilateral retinoblastoma and 319 with bilateral retinoblastoma. Median follow-up is of 93 months. Global survival is 98.5% of children, 10 children presented with second tumors, 11 children died (6 of tumor-related causes). Of the 411 children with unilateral retinoblastoma enucleation was needed at diagnosis for 324 (78.8%). Conservative treatments were attempted for 87 patients (21.2%) and ocular preservation obtained for 65 patients (74% of eyes). Three hundred and nineteen patients presented with bilateral retinoblastoma. Three hundred and ten could be treated conservatively for at least one eye. Initial intravenous chemotherapy was necessary for 75% of them. Ocular preservation without external beam radiation was possible for 221 patients (70%). The use of EBR decreased significantly after 2004 (9.1% of eyes vs 25.1%: P<0.001). DISCUSSION: Management and treatment of retinoblastoma are complex, adapted to the extent of the disease. Survival is good. Enucleation is still required for extensive ocular disease, especially for unilateral patients. Intravenous chemotherapy allows good tumor control and eye preservation and decrease the need of EBR. CONCLUSIONS: Retinoblastoma treatment with intravenous chemotherapy and ocular adjuvant therapies is very effective on the local tumor control and eye preservation.


Assuntos
Neoplasias Primárias Múltiplas/terapia , Neoplasias da Retina/terapia , Retinoblastoma/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Quimioterapia Adjuvante , Criança , Pré-Escolar , Terapia Combinada , Enucleação Ocular , Feminino , Seguimentos , Predisposição Genética para Doença , Humanos , Hipertermia Induzida , Lactente , Recém-Nascido , Infusões Intravenosas , Masculino , Neoplasias Primárias Múltiplas/mortalidade , Neoplasias Primárias Múltiplas/patologia , Preservação de Órgãos , Radioterapia/métodos , Neoplasias da Retina/genética , Neoplasias da Retina/mortalidade , Neoplasias da Retina/patologia , Retinoblastoma/genética , Retinoblastoma/mortalidade , Retinoblastoma/patologia , Estudos Retrospectivos , Análise de Sobrevida
10.
J Fr Ophtalmol ; 38(6): 550-8, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25982424

RESUMO

PURPOSE: To identify the socioeconomic and psychosocial impacts of clinical treatment decisions for advanced unilateral intraocular retinoblastoma. DESIGN: Retrospective observational case series. SETTING: institutional study at Alexandria Main University Hospital. STUDY POPULATION: records of 66 unilateral retinoblastoma cases treated from May 2005 to May 2013 were retrospectively reviewed. Sixty cases were eligible (International Intraocular Retinoblastoma Classification [IIRC] group C, D or E). PROCEDURES: two treatment groups were compared: enucleation vs. salvage treatment. Salvage treatment eyes were further subdivided based on IIRC group. Six socioeconomic parameters (financial burden, financial impact, psychological, social, medical and tumor impacts) were scored. Parameter scores ranged from 0 to 3, for overall score range 0 (no adverse impact) to 18 (severe adverse impact). MAIN OUTCOME MEASURES: derived Socioeconomic scores were correlated with treatment and outcomes. RESULTS: The enucleation group (28 eyes) had a median overall Socioeconomic score of 4/18, significantly lower than the salvage treatment group (32 eyes), median score 11/18 (P<0.01). Socioeconomic score varied with IIRC group. Attempted eye salvage failed in 25 children, due to uncontrolled tumor (44%) and socioeconomic impact of cumulative therapies (56%). Treatment duration and Socioeconomic score were higher for the 5 children in the salvage treatment group who developed metastatic disease compared to those without metastasis (P<0.01). CONCLUSIONS: The socioeconomic and psychosocial impacts of attempted ocular salvage for unilateral intraocular retinoblastoma are severe, in comparison to primary enucleation. Primary enucleation is a good treatment for unilateral retinoblastoma.


Assuntos
Adaptação Psicológica , Neoplasias da Retina/psicologia , Neoplasias da Retina/terapia , Retinoblastoma/psicologia , Retinoblastoma/terapia , Ajustamento Social , Criança , Pré-Escolar , Terapia Combinada/psicologia , Efeitos Psicossociais da Doença , Progressão da Doença , Egito , Enucleação Ocular/psicologia , Feminino , Hospitais Universitários , Humanos , Lactente , Masculino , Estadiamento de Neoplasias , Preservação de Órgãos/psicologia , Neoplasias da Retina/mortalidade , Neoplasias da Retina/patologia , Retinoblastoma/mortalidade , Retinoblastoma/patologia , Estudos Retrospectivos , Terapia de Salvação/psicologia , Fatores Socioeconômicos , Taxa de Sobrevida
11.
J Fr Ophtalmol ; 38(6): 542-9, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25982423

RESUMO

PURPOSE: To determine the efficacy of rescue intra-arterial chemotherapy (IAC) for retinoblastoma recurrence following failed initial IAC. METHODS: Retrospective, non-comparative, interventional case series of 12 eyes in 12 patients. INTERVENTION: Rescue IAC employed chemotherapy agents of melphalan (5mg, 7.5mg) alone or with additional topotecan (1mg). MAIN OUTCOME MEASURE: Tumor control and globe salvage. RESULTS: The median patient age at initial presentation was 16 months. At initial examination, the International Classification of Retinoblastoma grouping was group B (n=1), group D (n=7), or group E (n=4). The initial IAC was primary in 5 cases (42%) and secondary following failure of intravenous chemotherapy in 7 (58%). In all cases, initial IAC was delivered using melphalan 3mg (n=3), melphalan 5mg (n=7), or combination melphalan 5mg/topotecan 1mg (n=2) for a median of 3 cycles. The mean interval from initial IAC to recurrence necessitating rescue IAC was 5 months (median 4, range 2-10 months). Of the 12 patients, 3 (25%) had undergone previous enucleation of the opposite eye and the rescue IAC was planned for the only remaining eye. Rescue IAC was delivered for recurrent solid tumor (n=1), recurrent subretinal seeds (n=7), recurrent vitreous seeds (n=1), or combination recurrent subretinal/vitreous seeds (n=3). IAC was technically successful through the ophthalmic artery in 9 cases (75%) or the middle meningeal artery in 3 (25%). Rescue IAC involved median 3 cycles (mean 3, range 2-4 cycles) of higher dose melphalan in 4 cases (33%) or combination melphalan/topotecan in 8 (67%). At mean follow-up of 20 months (median 14 months, range 7-36 months), complete tumor control was achieved in 9 eyes (75%) and globe salvage in 8 eyes (67%). Of the 3 failure eyes, all were initially groups D or E, previously treated with initial IAC, and 2 had previous intravenous chemotherapy. There were 4 eyes that came to enucleation for persistent subretinal/vitreous seeds (n=3) or neovascular glaucoma without viable tumor (n=1). There was no case of cerebrovascular stroke, systemic metastasis, or death. CONCLUSION: Rescue IAC following retinoblastoma recurrence after initial IAC provided tumor control in 75% of cases and globe salvage in 67%. Rescue IAC can be considered in children who fail initial IAC, especially if the opposite eye has been enucleated.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Recidiva Local de Neoplasia/tratamento farmacológico , Neoplasias da Retina/tratamento farmacológico , Retinoblastoma/tratamento farmacológico , Terapia de Salvação , Quimioterapia Adjuvante , Pré-Escolar , Enucleação Ocular , Feminino , Seguimentos , Humanos , Lactente , Infusões Intra-Arteriais , Masculino , Melfalan/administração & dosagem , Recidiva Local de Neoplasia/diagnóstico , Neoplasias da Retina/diagnóstico , Retinoblastoma/diagnóstico , Retinoscopia , Retratamento , Topotecan/administração & dosagem , Falha de Tratamento , Resultado do Tratamento
12.
Cancer Radiother ; 19(8): 762-74, 2015 Dec.
Artigo em Francês | MEDLINE | ID: mdl-26508321

RESUMO

Malignant tumours of the eye are not common, barely representing 1 % of all cancers. This article aims to summarise, for each of the main eye malignant diseases, aspects of epidemiology, diagnostic methods and treatments, with a focus on radiation therapy techniques. The studied tumours are: eye metastasis, intraocular and ocular adnexal lymphomas, uveal melanomas, malignant tumours of the conjunctive, of the lids, and retinoblastomas. The last chapter outlines ocular complications of radiation therapy and their management.


Assuntos
Neoplasias Oculares , Carcinoma/diagnóstico , Carcinoma/epidemiologia , Carcinoma/radioterapia , Árvores de Decisões , Neoplasias Oculares/diagnóstico , Neoplasias Oculares/epidemiologia , Neoplasias Oculares/radioterapia , Neoplasias Oculares/secundário , Neoplasias Palpebrais/diagnóstico , Neoplasias Palpebrais/epidemiologia , Neoplasias Palpebrais/radioterapia , Humanos , Linfoma/diagnóstico , Linfoma/epidemiologia , Linfoma/radioterapia , Melanoma/diagnóstico , Melanoma/epidemiologia , Melanoma/radioterapia , Retinoblastoma/diagnóstico , Retinoblastoma/epidemiologia , Retinoblastoma/radioterapia , Neoplasias Uveais/diagnóstico , Neoplasias Uveais/epidemiologia , Neoplasias Uveais/radioterapia
13.
J Fr Ophtalmol ; 37(5): 381-7, 2014 May.
Artigo em Francês | MEDLINE | ID: mdl-24679452

RESUMO

INTRODUCTION: Retinoblastoma is a malignant tumor arising from retinal stem cells or retinoblasts. Its management has been well determined. The goal of this study is to report preliminary results obtained with combined chemotherapy and surgical treatment. PATIENTS AND METHODS: Our transverse prospective study includes retinoblastomas diagnosed and treated on our service from January 2006 to December 2010. Fundus examination under general anesthesia and radiological examination, orbital/brain CT or if unavailable, ultrasound, were systematically performed. MRI was able to be obtained in one case. The patients received 6 courses of chemotherapy (vincristin-carboplatin-etoposide or vincristin-cyclophosphamide), including 2 preoperative courses and 4 postoperative courses. A surgical procedure (enucleation or exenteration) supplemented the treatment, followed by histologic analysis of the specimen. External radiation therapy and conservative management were not available. RESULTS: Fifty-nine patients were treated. Median age at time of diagnosis was 9months (range 2months to 7years); the gender ratio was 1.18. Tumors were bilateral in 12 cases and unilateral in 47 cases, intraocular in 34 cases and extraocular in 25 cases. Optic nerve involvement was confirmed by histology in 12 cases. Twenty-one patients died from recurrence and/or metastatic disease, or during chemotherapy. Seven were lost to follow-up, and 31 have survived uneventfully. DISCUSSION: Improvement of mortality is still our major concern. Radiation therapy is essential in the presence of optic nerve involvement. CONCLUSION: Long-term survival will determine the efficacy of this form of management. The contribution of new therapeutic means will improve these results.


Assuntos
Neoplasias da Retina/terapia , Retinoblastoma/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Progressão da Doença , Feminino , Humanos , Lactente , Estudos Longitudinais , Masculino , Neoplasias da Retina/complicações , Neoplasias da Retina/epidemiologia , Neoplasias da Retina/patologia , Retinoblastoma/complicações , Retinoblastoma/epidemiologia , Retinoblastoma/patologia , Estudos Retrospectivos , Senegal/epidemiologia
14.
J Fr Ophtalmol ; 37(2): 99-106, 2014 Feb.
Artigo em Francês | MEDLINE | ID: mdl-24210936

RESUMO

PURPOSE: Retrospective study of local tolerability of a natural hydroxyapatite orbital implant wrapped with Vicryl(®) (polyglactin) mesh in patients undergoing enucleation. METHOD: Complications were classified into four types according to their management: type 1 if no reoperation was required, type 2 if additional surgery without grafting was required, type 3 if an oral mucosal graft was performed (major dehiscence) and type 4 if the complication required removal of the implant. RESULTS: Seven hundred and four patients with a median follow-up of 44 months. Five hundred and three patients were enucleated as a primary procedure and 201 after failure of conservative management. The overall complication rate was 12.07% (85 patients) with 68 type 1 complications, nine type 2 complications, three type 3 complications and five type 4 complications (0.71%). A total of 17 patients (2.42%) required additional surgery. The use of chemotherapy or radiotherapy before or after surgery did not influence the results. In univariate analysis, the tolerability was better in children than in adults. With multivariate analysis, only the diameter of the implant was an independent risk factor for complications (P=0.001). CONCLUSION: Use of a Vicryl(®) mesh-wrapped natural hydroxyapatite orbital implant after enucleation is particularly well tolerated including the pediatric population. A compromise should be sought with an implant large enough for good cosmetic results but small enough to avoid complications.


Assuntos
Durapatita/química , Enucleação Ocular , Implantes Orbitários , Poliglactina 910/química , Adolescente , Adulto , Criança , Pré-Escolar , Durapatita/efeitos adversos , Enucleação Ocular/estatística & dados numéricos , Neoplasias Oculares/epidemiologia , Neoplasias Oculares/cirurgia , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Implantes Orbitários/efeitos adversos , Poliglactina 910/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Implantação de Prótese/efeitos adversos , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Telas Cirúrgicas/efeitos adversos , Deiscência da Ferida Operatória/epidemiologia , Deiscência da Ferida Operatória/etiologia
15.
J Fr Ophtalmol ; 37(2): 155-65, 2014 Feb.
Artigo em Francês | MEDLINE | ID: mdl-24503203

RESUMO

Since the discovery of the structure of DNA in 1953 by Watson and Crick, our understanding of the genetic causes and the regulations involved in tumor development have hugely increased. The important amount of research developed since then has led to the development of gene therapy, which specifically targets and treats cancer cells by interacting with, and correcting their genetic material. This study is a review of the most accomplished research using gene therapy aimed at treating malignant ophthalmologic diseases, and focuses more specifically on uveal melanoma and retinoblastoma. Such approaches are remarkable regarding the efficiency and the cellular targeting specificity. However, gene therapy-based treatments are so recent that many long-term interrogations subsist. The majority of the reviewed studies are conducted in vitro or in murine models, thereby requiring several years before the resulting therapies become part of the daily ophthalmologists' arsenal. However, the recent spectacular developments based on advanced scientific knowledge justify an up-to-date review that would benefit the ophthalmologist community.


Assuntos
Neoplasias Oculares/terapia , Terapia Genética/métodos , Procedimentos Cirúrgicos Oftalmológicos/métodos , Procedimentos Cirúrgicos Oftalmológicos/tendências , Animais , Neoplasias Oculares/genética , Técnicas de Transferência de Genes , Vetores Genéticos/administração & dosagem , Humanos , Imunoterapia/métodos , Imunoterapia/tendências , Lipossomos , Melanoma/terapia , Neoplasias da Retina/terapia , Retinoblastoma/terapia , Neoplasias Uveais/terapia
16.
J Fr Ophtalmol ; 37(6): 442-8, 2014 Jun.
Artigo em Francês | MEDLINE | ID: mdl-24792399

RESUMO

PURPOSE: To report the relative frequency of presenting signs in Tunisian children with retinoblastoma and to evaluate their prognostic impact. METHODS: A retrospective study was undertaken of 200 patients with retinoblastoma examined and treated between January 1993 and June 2009. RESULTS: There were 123 boys and 77 girls. For all cases, mean age at diagnosis was 29.8 months (range, 1 month to 9 years). There were 138 (69%) unilateral cases and 62 (31%) bilateral cases. Nineteen children (9.5%) had a family history of retinoblastoma. The most common signs were leukocoria (80%) and strabismus (28%) followed by proptosis. Orbital inflammation, hyphemia and phthisis are rare presenting findings in retinoblastoma. In our series, 26 children (13%) presented with extraocular retinoblastoma. Leukocoria and proptosis are significantly associated with a worse prognosis. CONCLUSION: The ability to recognize the presenting signs and symptoms of retinoblastoma can lead to earlier diagnosis and better prognosis.


Assuntos
Neoplasias da Retina/diagnóstico , Neoplasias da Retina/mortalidade , Retinoblastoma/diagnóstico , Retinoblastoma/mortalidade , Criança , Pré-Escolar , Diagnóstico Tardio , Exoftalmia/etiologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Distúrbios Pupilares/etiologia , Neoplasias da Retina/genética , Neoplasias da Retina/terapia , Retinoblastoma/genética , Retinoblastoma/terapia , Estudos Retrospectivos , Estrabismo/etiologia
17.
J Fr Ophtalmol ; 37(2): 115-24, 2014 Feb.
Artigo em Francês | MEDLINE | ID: mdl-24507074

RESUMO

Retinoblastoma is the most frequent intraocular cancer, affecting almost exclusively children. We report prospective study results assessing the national protocol for retinoblastoma treatment in Morocco. Treatment included, depending on stage and laterality, primary chemotherapy either to facilitate enucleation or to make conservative treatment possible, postoperative chemotherapy, enucleation and conservative treatments such as transpupillary thermotherapy, thermochemotherapy and cryotherapy. Radiation was used in a few cases. Close supervision was performed until the age of 5. The incidence of retinoblastoma within the study period was 18 new cases per year in our department. Observations of 32 children were included in the study: 18 unilateral retinoblastomas (56%) and 14 bilateral retinoblastomas (44%), for a total of 46 eyes. Leucocoria was the most frequent presenting symptom (69%). Buphthalmia or proptosis were present in 47% of cases. The stage of retinoblastoma was V/D or E (Reese-Elsworth/ABC) in 69.5% of cases. Enucleation was necessary for 28 eyes. Transpupillary thermotherapy or thermochemotherapy were used for 13 eyes (11 children) and cryotherapy for 13 eyes (10 children). After an average follow-up period of 52 months, among 32 children, 4 died and 2 abandoned treatment. Ocular salvage rate was 85.7% (12 eyes out of 14, among which 11 without radiation). Retinoblastoma is a genetic tumor, which occurs in two forms: sporadic, always unilateral, and hereditary, often bilateral. The latter is the most challenging case. Current treatment protocols rely primarily on chemotherapy and local treatments. The future is oriented toward purely local treatments such as intra-arterial chemotherapy and intraocular chemotherapy.


Assuntos
Neoplasias da Retina/terapia , Retinoblastoma/terapia , Centros Médicos Acadêmicos , Criança , Pré-Escolar , Protocolos Clínicos/normas , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Marrocos/epidemiologia , Programas Nacionais de Saúde/normas , Tratamentos com Preservação do Órgão/normas , Tratamentos com Preservação do Órgão/estatística & dados numéricos , Projetos Piloto , Neoplasias da Retina/epidemiologia , Retinoblastoma/epidemiologia
18.
J Fr Ophtalmol ; 36(8): 704-9, 2013 Oct.
Artigo em Francês | MEDLINE | ID: mdl-23911063

RESUMO

The first 2 years of life are essential to visual development. The ophthalmological examination of a baby is different from that of an adult, in terms of both methodology and clinical signs. The specifics of examination at this age require a rigorous history taking as a first step: personal and family medical history, first sign of the disease and its progression. Cycloplegic refraction and fundus exam are both critical regardless of the reason for consultation. In addition, fundus exam must be performed at the first visit in cases of strabismus, nystagmus, or abnormal visual function, in order to rule out underlying retinoblastoma. Gross inspection of the patient can offer much information: malformations, visual behavior, oculomotor abnormalities. Quantification of vision and refraction is age-specific. It cannot be based solely on visual behavior, it cannot be just an approximation, and it often needs to be repeated. Lastly, examination of the anterior and posterior segments may require specialized equipment, special techniques, and may reveal pathology specific to the infant. Throughout the examination, patience and gentleness help greatly to insure a reliable diagnosis.


Assuntos
Técnicas de Diagnóstico Oftalmológico , Oftalmopatias/diagnóstico , Exame Físico/métodos , Adulto , Pré-Escolar , Anormalidades do Olho/diagnóstico , Anormalidades do Olho/terapia , Oftalmopatias/congênito , Oftalmopatias/terapia , Humanos , Lactente , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/terapia , Testes Visuais
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