RESUMEN
INTRODUCTION: The treatment of conjunctival melanoma is most often conservative, but exenteration is sometimes necessary in order to achieve local control of the disease. It can be performed as a primary procedure in cases of locally advanced disease or as a secondary procedure after one or more recurrences. No benefit to secondary exenteration on patient survival has been demonstrated to date for conjunctival melanoma, and it is generally considered a palliative procedure. PATIENTS AND METHODS: Single-center retrospective study performed in the ocular oncology department of the Institut Curie (Paris, France). We included all patients who underwent secondary orbital exenteration for conjunctival melanoma between January 2008 and January 2016. RESULTS: Twenty-five patients underwent secondary exenteration for conjunctival melanoma. The maximum number of local recurrences prior to exenteration was six. Metastases occurred in 11 patients after exenteration and were more common when there was a greater tumor thickness on histology, if the tumor had not been treated initially in an ocular oncology center, or if there had been a greater number of local recurrences before the secondary exenteration was performed. Seventy-five percent of patients developed metastases when the exenteration was performed after 5 or 6 local recurrences. CONCLUSION: This study suggests that early secondary exenteration (i.e. after a number of local recurrences less than or equal to 4) may reduce the occurrence of metastases (and therefore improve patient survival) in conjunctival melanoma. Thus, secondary exenteration might be a curative surgery in some patients with recurrent disease.
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Neoplasias de la Conjuntiva , Melanoma , Neoplasias de la Conjuntiva/cirugía , Humanos , Melanoma/cirugía , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/cirugía , Evisceración Orbitaria , Estudios RetrospectivosRESUMEN
PURPOSE: Our objective was to assess the results of surgical management of palpebral basal cell carcinomas (BCC) followed by a second line treatment discussed during a Multidisciplinary Team Meeting (MTM). MATERIALS AND METHODS: This retrospective single-centred study includes all surgically-treated basal cell carcinomas of the eyelids between January 2005 and January 2015. After initial surgery, the cases were systematically discussed during a multidisciplinary team meeting in order to assess the need for additional treatment. Data relative to the patient, tumor and management were pulled from the medical record. RESULTS: A total of 171 patients were included, with a mean age of 74 years. Among the patients, 151 underwent pentagonal resection of the tumor, and 20 patients had a superficial excision. After surgical management, 120 patients (70.2%) were considered to have sufficient free margins. The other 51 patients (29.8%) had insufficient margins due to remaining tumor cells (38 patients) or free margins less than 1mm. Among these 51 patients with insufficient margins, 19 received a second surgical treatment, 17 patients received adjuvant radiotherapy, and 15 were followed closely with an intensive biannual follow-up program. No patients were lost to follow-up. With a mean follow-up of 42 months (min. 6 months-max. 128 months), 7 out of 171 patients (4.1%) developed a local recurrence. The mean time between surgical management and recurrence was 24 months. The recurrence rate was higher for the group of patients with a recurrent tumor (11.6%) than for the group of patients referred for initial management (2.8%). Incomplete resection was also associated with a higher recurrence rate (3 recurrences out of 51 patients). DISCUSSION: The management of basal cell carcinomas of the eyelid is first and foremost surgical with the goal of complete resection confirmed by histopathological analysis. The histological analyses (Mohs micrographic surgery, frozen section technic, paraffin fixation) and recommended sizes of the margins can vary in the literature, with recurrence rates from 1.8% to 9.5%. CONCLUSION: In our experience, multidisciplinary management of BCC of the eyelid, including initial macroscopic surgery, histopathological analysis stating the histological type and size of the margins, along with additional treatment discussed in a MTM, allows for a recurrence rate of 4.1%.
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Carcinoma Basocelular/cirugía , Neoplasias de los Párpados/cirugía , Procedimientos Quirúrgicos Oftalmológicos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma Basocelular/patología , Quimioterapia Adyuvante/estadística & datos numéricos , Neoplasias de los Párpados/epidemiología , Neoplasias de los Párpados/patología , Femenino , Francia/epidemiología , Humanos , Comunicación Interdisciplinaria , Masculino , Persona de Mediana Edad , Terapia Neoadyuvante , Procedimientos Quirúrgicos Oftalmológicos/métodos , Procedimientos Quirúrgicos Oftalmológicos/estadística & datos numéricos , Grupo de Atención al Paciente/organización & administración , Radioterapia Adyuvante/estadística & datos numéricos , Procedimientos de Cirugía Plástica/métodos , Procedimientos de Cirugía Plástica/estadística & datos numéricos , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenAsunto(s)
Neoplasias de la Coroides/patología , Neoplasias de la Coroides/cirugía , Melanoma/patología , Melanoma/cirugía , Herida Quirúrgica/patología , Vitrectomía/efectos adversos , Anciano de 80 o más Años , Femenino , Humanos , Neoplasias Hepáticas/secundario , Invasividad Neoplásica , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/patología , Esclerótica/patología , Esclerótica/cirugíaRESUMEN
PURPOSE: Evisceration can be performed for blind, painful eyes. This surgery can promote the dissemination of tumor cells within the orbit if an ocular tumor has been missed preoperatively. METHODS: We reviewed the medical records of patients who were eviscerated for blind, painful eyes between 2009 and 2014 and who were referred after the surgery to the Institut Curie or the Rothschild Foundation in Paris. We included the patients with a histological diagnosis of ocular tumor or orbital recurrence. Cytogenetic analysis was performed whenever possible. RESULTS: Four patients turned out to have an ocular tumor after evisceration (two choroidal melanomas, a rhabdoid tumor and an adenocarcinoma of the retinal pigment epithelium); two had a history of prior ocular trauma. The tumors were diagnosed either on histological analysis of the intraocular contents (2 patients) or biopsy of orbital recurrence (2 patients). Prior to evisceration, fundus examination was not performed in 3 patients. One had preoperative imaging but no intraocular tumor was suspected. At the time of this study, 3 patients had had an orbital recurrence and died. We also found 2 patients who had an evisceration despite a past history of choroidal melanoma treated with proton beam therapy. CONCLUSION: We showed that evisceration of eyes with unsuspected ocular malignancies was associated with a poor prognosis due to orbital recurrence and metastasis. The evisceration specimen should therefore always be sent for histological analysis in order to perform prompt adjuvant orbital radiotherapy if an ocular tumor is found.
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Adenocarcinoma/patología , Adenocarcinoma/cirugía , Evisceración del Ojo , Neoplasias del Ojo/patología , Neoplasias del Ojo/cirugía , Adulto , Anciano de 80 o más Años , Neoplasias de la Coroides/patología , Neoplasias de la Coroides/cirugía , Enucleación del Ojo , Femenino , Humanos , Masculino , Melanoma/patología , Melanoma/cirugía , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Neoplasias de la Úvea/patología , Neoplasias de la Úvea/cirugíaRESUMEN
PurposeIntraocular retinoblastoma treatments often combine chemotherapy and focal treatments. A first prospective protocol of conservative treatments in our institution showed the efficacy of the use of two courses of chemoreduction with etoposide and carboplatin, followed by chemothermotherapy using carboplatin as a single agent and diode laser. In order to decrease the possible long-term toxicity of chemotherapy due to etoposide, a randomized neoadjuvant phase II protocol was conducted using vincristine-carboplatin vs etoposide-carboplatin.Patients and methodsThe study was proposed when initial tumor characteristics did not allow front-line local treatments. Patients included in this phase II noncomparative randomized study of neoadjuvant chemotherapy received vincristin-carboplatin (new arm) vs etoposide-carboplatin (our reference arm). They were subsequently treated by local treatments and chemothermotherapy. Primary end point was the need for secondary enucleation or external beam radiotherapy (EBRT) not exceeding 40% at 2 years.ResultsA total of 65 eyes in 55 children were included in the study (May 2004 to August 2009). Of these, 32 eyes (27 children) were treated in the arm etoposide-carboplatin and 33 eyes (28 children) in the arm vincristin-carboplatin. At 2 years after treatment, 23/33 (69.7%) eyes were treated and salvaged without EBRT or enucleation in the arm vincristin-carboplatin and 26/32 (81.2%) in the arm etoposide-carboplatin.ConclusionEven if the two treatment arms could be considered as sufficiently active according to the study decision rules, neoadjuvant chemotherapy by two cycles of vincristine-carboplatin followed by chemothermotherapy appear to offer less optimal local control than the etoposide-carboplatin combination.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Hipertermia Inducida , Terapia Neoadyuvante , Neoplasias de la Retina/terapia , Retinoblastoma/terapia , Carboplatino/administración & dosificación , Etopósido/administración & dosificación , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Neoplasias de la Retina/clasificación , Neoplasias de la Retina/patología , Retinoblastoma/clasificación , Retinoblastoma/patología , Vincristina/administración & dosificaciónRESUMEN
Retinoblastoma is the most common intraocular malignancy of infancy with an incidence of 1/15,000 births. Sixty percent of retinoblastomas are unilateral, with a median age at diagnosis of 2 years, and in most cases they are not hereditary. Retinoblastoma is bilateral in 40% of cases, with an earlier median age at diagnosis of 1 year. All bilateral and multifocal unilateral forms are hereditary and are part of a genetic cancer predisposition syndrome. All children with a bilateral or familial form, and 10-15% of children with a unilateral form, constitutionally carry an RB1 gene mutation. The two most frequent symptoms at diagnosis are leukocoria and strabismus. Diagnosis is made by fundoscopy, with ultrasound and magnetic resonance imaging (MRI) contributing both to diagnosis and assessment of the extension of the disease. Treatment of patients with retinoblastoma must take into account the various aspects of the disease (unilateral/bilateral, size, location), the risks for vision, and the possible hereditary nature of the disease. The main prognostic aspects are still early detection and adapted coverage by a multidisciplinary, highly specialized team. Enucleation is still often necessary in unilateral disease; the decision for adjuvant treatment is made according to the histological risk factors. The most important recent therapeutic advances concern conservative treatment, which is proposed for at least one of the two eyes in most bilateral cases: laser alone or in combination with chemotherapy, cryotherapy, or brachytherapy. Recently, the development of new conservative techniques of treatment, such as intra-arterial selective chemotherapy perfusion and intravitreal injections, aims at preserving visual function in these children and decreasing the number of enucleations and the need for external beam radiotherapy. The vital prognosis related to retinoblastoma is now excellent in industrialized countries, but long-term survival is still related to the development of secondary tumors, mainly secondary sarcoma. Retinoblastoma requires multidisciplinary care as well as a long-term specialized follow-up. Early counseling of patients and their family concerning the risk of transmission of the disease and the risk of development of secondary tumors is necessary.
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Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/terapia , Retinoblastoma/diagnóstico , Retinoblastoma/terapia , Niño , Humanos , Incidencia , Pronóstico , Reflejo Pupilar , Neoplasias de la Retina/epidemiología , Neoplasias de la Retina/genética , Retinoblastoma/epidemiología , Retinoblastoma/genética , Proteína de Retinoblastoma/genética , Estrabismo/etiologíaRESUMEN
Despite advances in the local treatment of UM, half of patients develop metastases typically to the liver with poor survival. Microscopic complete surgical resection (R0) of liver metastases improves survival in high selected patients. Early identification of high-risk patients might allow detection of asymptomatic metastases, and increase R0 liver surgery rate. From October 2006 to December 2009, we conducted a prospective study to detect early minimal lesions with 6-monthly liver function tests (LFTs) and liver MRI in 100 high-risk patients. High risk was defined by primary tumor clinical or genomic criteria: thickness>8mm or diameter>15 mm, or extra-scleral extension, or monosomy 3 by FISH or aCGH. With a median follow-up of 49 months, the 5-year metastasis-free survival and overall survival were 47 and 33%, respectively. Of the 60 patients who became metastatic, 50 (83%) had exclusive liver metastasis. LFTs screening had no sufficient accurary, but biannual MRI showed high predictive value to detect metastasis and select patients eligible for curative surgery: 25/50 underwent laparotomy and among them, 8/25 (32%) had a R0 surgery. Median survival after metastasis was 14 months, mean survival reached 40 months in the R0 resected population. Six-monthly liver MRI screening is recommended in patients with large tumors or genomic high risk in order to detect early patient candidates to complete resection of liver metastases.
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Diagnóstico Precoz , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/secundario , Melanoma/diagnóstico , Melanoma/secundario , Neoplasias de la Úvea/diagnóstico , Neoplasias de la Úvea/secundario , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Supervivencia sin Enfermedad , Intervención Médica Temprana , Femenino , Predisposición Genética a la Enfermedad/genética , Humanos , Pruebas de Función Hepática , Neoplasias Hepáticas/mortalidad , Neoplasias Hepáticas/cirugía , Imagen por Resonancia Magnética , Masculino , Melanoma/genética , Melanoma/mortalidad , Melanoma/cirugía , Metastasectomía/métodos , Microcirugia/métodos , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Factores de Riesgo , Neoplasias de la Úvea/genética , Neoplasias de la Úvea/mortalidad , Neoplasias de la Úvea/cirugía , Adulto JovenRESUMEN
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.
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Neoplasias Primarias Múltiples/terapia , Neoplasias de la Retina/terapia , Retinoblastoma/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Quimioterapia Adyuvante , Niño , Preescolar , Terapia Combinada , Enucleación del Ojo , Femenino , Estudios de Seguimiento , Predisposición Genética a la Enfermedad , Humanos , Hipertermia Inducida , Lactante , Recién Nacido , Infusiones Intravenosas , Masculino , Neoplasias Primarias Múltiples/mortalidad , Neoplasias Primarias Múltiples/patología , Preservación de Órganos , Radioterapia/métodos , Neoplasias de la Retina/genética , Neoplasias de la Retina/mortalidad , Neoplasias de la Retina/patología , Retinoblastoma/genética , Retinoblastoma/mortalidad , Retinoblastoma/patología , Estudios Retrospectivos , Análisis de SupervivenciaRESUMEN
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.
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Durapatita/química , Enucleación del Ojo , Implantes Orbitales , Poliglactina 910/química , Adolescente , Adulto , Niño , Preescolar , Durapatita/efectos adversos , Enucleación del Ojo/estadística & datos numéricos , Neoplasias del Ojo/epidemiología , Neoplasias del Ojo/cirugía , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Implantes Orbitales/efectos adversos , Poliglactina 910/efectos adversos , Complicaciones Posoperatorias/epidemiología , Implantación de Prótesis/efectos adversos , Reoperación/estadística & datos numéricos , Estudios Retrospectivos , Mallas Quirúrgicas/efectos adversos , Dehiscencia de la Herida Operatoria/epidemiología , Dehiscencia de la Herida Operatoria/etiologíaRESUMEN
BACKGROUND: Retinoblastoma (RB) is the most frequent eye tumour in children, with an incidence of 1 in 15-20,000 births. It accounts for 11% of all cancers in the first year of life. Except for the hereditary forms, its causes are not well-known. Studies have recently suggested an increased risk of RB among children born after IVF, but the relevant literature is sparse. We assessed the association between infertility treatment, subfertility and RB. METHODS: We included all children living in France diagnosed with RB between 1 January 2000 and 31 December 2006 at the Institut Curie, the national reference centre for RB diagnosis and treatment. We used multiple logistic regression to compare them with a national sample of births in France in 1998 and 2003 (n = 28 170). RESULTS: The study included 244 non-familial RB cases. The risk of RB increased with maternal age [adjusted odds ratio (adj OR) = 2.07, 95% confidence interval (CI) 1.33-3.22 at 35-39 years compared with younger than 25 years and adj OR = 2.42, 95% CI 1.22-4.81 at 40 years or older], but the associations with IVF (adj OR = 1.37, 95% CI 0.64-2.95) and ovarian stimulation or intrauterine insemination (adj OR = 1.35, 95% CI 0.77-2.38) were not statistically significant after adjustment for maternal age and tobacco use. Among women who had no infertility treatment, the risk of RB was significantly increased when time to pregnancy exceeded 24 months (adj OR = 2.02, 95% CI 1.17-3.48) compared with time to pregnancy ≤ 24 months. CONCLUSIONS: Our study did not observe a significantly increased risk of RB associated with infertility treatment, in particular with IVF. But we did find an increased risk for women for whom time to pregnancy exceeded 24 months.
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Infertilidad/terapia , Técnicas Reproductivas Asistidas/efectos adversos , Retinoblastoma/diagnóstico , Retinoblastoma/etiología , Preescolar , Femenino , Fertilización In Vitro/efectos adversos , Francia , Humanos , Lactante , Recién Nacido , Masculino , Oportunidad Relativa , Inducción de la Ovulación/efectos adversos , Embarazo , Análisis de Regresión , RiesgoRESUMEN
Intra-arterial infusion of chemotherapy into the ophthalmic artery for treatment of retinoblastoma has been realized after catheterization of the internal carotid and temporary balloon occlusion beyond the orifice of the ophthalmic artery, or more recently after superselective canulation of the ophthalmic artery by a microcatheter. The superselective catheterization of the ophthalmic artery could be cumbersome because of the implantation of the ostium on the carotid siphon or because of the tortuosity of the carotid siphon. We report our experience of using a retrograde approach through the posterior communicating artery that allows a more direct angle of access to the origin of the ophthalmic artery.
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Antineoplásicos Alquilantes/administración & dosificación , Melfalán/administración & dosificación , Arteria Oftálmica , Neoplasias de la Retina/tratamiento farmacológico , Retinoblastoma/tratamiento farmacológico , Femenino , Fluoroscopía , Humanos , Lactante , Infusiones IntraarterialesRESUMEN
AIMS: To assess if systematic fundus screening according to an 'intensive' schedule alters ocular outcome and to propose fundus screening schedule guidelines for children related to a retinoblastoma patient. METHODS: For children with a positive family history of retinoblastoma, we perform fundus exams shortly after birth under general anaesthesia and then at regular intervals according to schedules based on the risk. Familial retinoblastoma cases seen at our institution from January 1995 to December 2004 were retrospectively classified as 'screened' or 'non-screened' (NS) and, among the 'screened' patients, as 'intensively screened' (IS) if screening matched our recommendations or 'non-intensively screened' (S). Groups were compared by Fisher exact test for categorical variables and Kruskal-Wallis test for continuous variables. RESULTS: Among the 547 retinoblastoma patients managed at our institution during this period, 59 were familial cases. In all, 20 were in the NS group, 23 in the S group, and 16 in the IS group. The number of children enucleated was, respectively, 13, 2, and 0 (P<10(-4)); external beam radiation (EBRT) was required for, respectively, 6, 0, and 2 children (P<0.009). Chemotherapy burden and visual acuity were not significantly different between groups. CONCLUSION: An 'intensive' fundus screening schedule decreased the need for enucleation and EBRT. Therefore, despite the heavy burden of the screening schedule, we recommend physicians and health-care professionals to better inform and refer children with a family history of retinoblastoma for genetic counselling and proper fundus screening in specialized centres.
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Fondo de Ojo , Tamizaje Masivo/métodos , Guías de Práctica Clínica como Asunto , Neoplasias de la Retina/diagnóstico , Retinoblastoma/diagnóstico , Adolescente , Niño , Preescolar , Enucleación del Ojo/estadística & datos numéricos , Femenino , Humanos , Lactante , Masculino , Estadificación de Neoplasias , Neoplasias de la Retina/genética , Neoplasias de la Retina/patología , Neoplasias de la Retina/terapia , Retinoblastoma/genética , Retinoblastoma/patología , Retinoblastoma/terapia , Estudios Retrospectivos , Estadísticas no Paramétricas , Agudeza VisualRESUMEN
Uveal melanoma arises from melanocytes of the uveal tract (iris, ciliary body and choroid) and represents the most common intraocular malignancy in adults. Some rare clinical situations (young age at diagnosis, bilateral or multifocal forms, association with cutaneous malignant melanoma and/or familial aggregations of melanomas) are suggestive of genetic susceptibility. The aim of this study was to evaluate the contribution of CDKN2A/P16INK4A, P14ARF and CDK4 gene germline mutations in a series of patients with uveal melanoma recruited in a single institution with a clinical presentation indicative of genetic predisposition. Molecular analyses were proposed to 36 patients and were performed in 25 cases. The contribution of BRCA1/2 gene germline mutations in patients with uveal melanoma and a personal and/or family history of breast/ovarian cancers was also evaluated. Molecular analysis of BRCA1/2 genes was proposed to 35 patients and was performed in 25 patients. No deleterious germline mutation was identified in either group of patients. These results indicate that the CDKN2A/P16INK4A, P14ARF, CDK4 genes are not responsible for the vast majority of genetic susceptibility to uveal melanoma. They also suggest that one case of uveal melanoma in a family with a history of breast cancer is not sufficient to justify BRCA1/2 genetic testing when the classical criteria for molecular analysis are not present. International studies are ongoing in melanoma-prone families in an attempt to identify uveal melanoma susceptibility loci and genes.
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Quinasa 4 Dependiente de la Ciclina/genética , Inhibidor p16 de la Quinasa Dependiente de Ciclina/genética , Predisposición Genética a la Enfermedad , Mutación de Línea Germinal/genética , Melanoma/genética , Proteína p14ARF Supresora de Tumor/genética , Neoplasias de la Úvea/genética , Adolescente , Adulto , Anciano , Proteína BRCA1/genética , Proteína BRCA2/genética , Neoplasias de la Mama/genética , Neoplasias de la Mama/patología , ADN de Neoplasias/genética , Femenino , Pruebas Genéticas , Humanos , Masculino , Melanoma/patología , Neoplasias Ováricas/genética , Neoplasias Ováricas/patología , Linaje , Reacción en Cadena de la Polimerasa , Pronóstico , Neoplasias de la Úvea/patología , Adulto JovenRESUMEN
BACKGROUND: Microscopically complete (R0) resection of metastases from uveal melanoma prolongs median overall survival compared to incomplete surgery. The aim of this study was to compare the sensitivity of dynamic-enhanced magnetic resonance imaging (MRI) with fluorodeoxyglucose-positron emission tomography (FDG-PET) in the preoperative diagnosis of liver metastases from uveal melanoma. PATIENTS AND METHODS: Fifteen consecutive patients (mean age: 56 years) underwent FDG-PET and liver MRI. Extrahepatic metastatic disease was excluded by whole body computed tomography and bone scintigraphy. MRI and FDG-PET were performed with a mean of 19 days (range: 1-30) before surgery. Imaging findings were compared with surgical (including intraoperative ultrasonography) and histological findings on a lesion by lesion analysis. RESULTS: R0 resection was performed in 12 patients. A total of 28 lesions were resected with 27 histologically proven metastases. Nine lesions were smaller than 5mm, 7 measured 5-10mm and 11 were larger than 10mm. Sensitivity and positive predictive value were 67% and 95% for MRI compared to 41% and 100% for FDG-PET. The difference between the two modalities was statistically significant (p=0.01; McNemar test). In remaining 3 patients, diffuse miliary disease (>10 capsular lesions) was discovered intraoperatively, and was suspected on preoperative MRI in 2 cases. Only one extrahepatic lesion identified by FDG-PET was falsely positive. CONCLUSIONS: In this preliminary study, MRI was superior to FDG-PET for staging of liver metastases from uveal melanoma. Although miliary disease was suggested by MRI in some cases, preoperative confirmation remains imperfect.
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Fluorodesoxiglucosa F18 , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/secundario , Imagen por Resonancia Magnética , Melanoma/diagnóstico , Melanoma/secundario , Tomografía de Emisión de Positrones , Radiofármacos , Neoplasias de la Úvea/patología , Adulto , Anciano , Femenino , Humanos , Neoplasias Hepáticas/cirugía , Masculino , Melanoma/cirugía , Persona de Mediana Edad , Estadificación de Neoplasias , Valor Predictivo de las Pruebas , Sensibilidad y EspecificidadRESUMEN
BACKGROUND: Uveal melanoma is characterised by a high prevalence of liver metastases and a poor prognosis. AIM: To review the evolving surgical management of this challenging condition at a single institution over a 16-year period. PATIENTS AND METHODS: Between January 1991 and June 2007, among 3873 patients with uveal melanoma, 798 patients had liver metastases. We undertook a detailed retrospective review of their clinical records and surgical procedures. The data was evaluated with both uni- and multivariate statistical analysis for predictive survival indicators. RESULTS: 255 patients underwent surgical resection. The median interval between ocular tumour diagnosis and liver surgery was 68 months (range 19-81). Liver surgery was either microscopically complete (R0; n = 76), microscopically incomplete (R1; n = 22) or macroscopically incomplete (R2; n = 157). The median overall postoperative survival was 14 months, but increased to 27 months when R0 resection was possible. With multivariate analysis, four variables were found to independently correlate with prolonged survival: an interval from primary tumour diagnosis to liver metastases >24 months, comprehensiveness of surgical resection (R0), number of metastases resected (< or = 4) and absence of miliary disease. CONCLUSIONS: Surgical resection, when possible, is able to almost double the survival and appears at present the optimal way of improving the prognosis in metastatic uveal melanoma. Advances in medical treatments will be required to further improve survival.
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Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/cirugía , Neoplasias de la Úvea/patología , Anciano , Femenino , Hepatectomía , Humanos , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
Kaposi sarcoma is rare and occurs in four forms (classic, endemic, post-transplant and epidemic), which all have the same histological appearance associated with the same viral agent: human herpes virus type 8 (HHV-8). Conjunctival and palpebral locations are, however, rare, and only 30 cases have been described in the literature. We present the case of a 34-year-old woman with conjunctival Kaposi angiosarcoma, with rare bulbar location associated with superior palpebral telangiectases against a rare immunodepression syndrome (common variable immunodeficiency). The treatment consisted of an excision biopsy with an over-and-over suture of a conjunctival rotation flap, combined with anti-HHV-8 chemotherapy to effect a regression of the palpebral lesion. However, the reappearance of a conjunctival lesion on the rim of the excision site required local radiotherapy of approximately 30 Gy, with no recurrence after 1 month.
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Neoplasias de la Conjuntiva/patología , Sarcoma de Kaposi/patología , Adulto , Femenino , HumanosRESUMEN
INTRODUCTION: Eosinophilic fasciitis (EF) is a rare inflammatory and fibrosing disorder with clinical, biological and histological characteristics. It has sometimes been reported with hematologic malignancy, but has never been associated with malignant uveal melanoma. CASE REPORT: A 67-year-old woman with choroidal melanoma was found to have metastatic skeletal extension of her melanoma while she developed swelling and pain in the lower limbs. Histologic examination confirmed an EF and oral corticosteroid treatment was started. A treatment for the ophthalmologic and skeletal extension of her melanoma was initiated. The paraneoplastic character of this EF was entertained. CONCLUSION: The simultaneous occurrence of EF and metastatic skeletal melanoma is suggestive of the possibility of EF constituting a paraneoplastic syndrome.
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Neoplasias de la Coroides/complicaciones , Eosinofilia/complicaciones , Fascitis/complicaciones , Melanoma/complicaciones , Síndromes Paraneoplásicos , Administración Oral , Corticoesteroides/administración & dosificación , Corticoesteroides/uso terapéutico , Anciano , Anciano de 80 o más Años , Antiinflamatorios/administración & dosificación , Antiinflamatorios/uso terapéutico , Antineoplásicos Hormonales/administración & dosificación , Antineoplásicos Hormonales/uso terapéutico , Neoplasias Óseas/secundario , Neoplasias Óseas/cirugía , Neoplasias de la Coroides/tratamiento farmacológico , Neoplasias de la Coroides/radioterapia , Eosinofilia/diagnóstico , Eosinofilia/terapia , Fascitis/diagnóstico , Fascitis/tratamiento farmacológico , Femenino , Estudios de Seguimiento , Humanos , Radioisótopos de Yodo/uso terapéutico , Masculino , Melanoma/tratamiento farmacológico , Melanoma/radioterapia , Persona de Mediana Edad , Prednisona/administración & dosificación , Prednisona/uso terapéutico , Factores de TiempoRESUMEN
OBJECTIVES: Detail the most frequent problems encountered in the differential diagnosis of retinoblastoma. PATIENTS: and method: We conducted a retrospective study on the children referred to the Curie Institute for suspicion of retinoblastoma between 2000 and 2006. Diagnosis was made by fundus examination using the indirect ophthalmoscope, ultrasonography, and MRI. RESULTS: Of the 486 children seen during this period, 408 had unilateral or bilateral retinoblastoma and 78 (16%) had another lesion: Coats disease (20 children, 25%), congenital malformations (23 children, 30%; coloboma, PHPV, microphthalmia, isolated or associated with retinal dysplasia), other tumors (10 children, 13%; astrocytomas and medulloepithelioma), combined hamartomas (six children, 8%), inflammatory diseases (six children, 8%) (Toxocara canis, cat scratch eye disease, or toxoplasmosis), and other diseases (13 children, 16%; corneal opacities, congenital cataract, or retinal detachment). DISCUSSION: Compared to previous series, this study shows the proportion of erroneous diagnosis has lowered (16%) compared to earlier studies by Balmer (1986; 30%), and Shields (1991; 42%). No cases of retinopathy of prematurity were seen in our series, demonstrating that screening is good or of a lower frequency in France. The frequency of PHPV has dropped. Coats disease remains a frequent and sometimes difficult diagnosis to make, particularly in advanced stages of the disease.
Asunto(s)
Neoplasias de la Retina/diagnóstico , Retinoblastoma/diagnóstico , Adulto , Niño , Diagnóstico Diferencial , Errores Diagnósticos/estadística & datos numéricos , Humanos , Imagen por Resonancia Magnética , Oftalmoscopía , Neoplasias de la Retina/clasificación , Neoplasias de la Retina/diagnóstico por imagen , Neoplasias de la Retina/patología , Retinoblastoma/clasificación , Retinoblastoma/diagnóstico por imagen , Retinoblastoma/patología , Estudios Retrospectivos , UltrasonografíaRESUMEN
INTRODUCTION: Exudation from the tumour scar and glaucoma can be major problems after proton beam irradiation of uveal melanoma and can sometimes lead to secondary enucleation. We conducted a randomized study to determine whether systematic transpupillary thermotherapy (TTT) after proton beam radiotherapy could have a beneficial effect. PATIENTS AND METHOD: Between February 1999 and April 2003, all the patients treated by proton beam radiotherapy for uveal melanomas >/=7 mm thick or >/=15 mm in diameter were included in this study after giving their informed consent. One half of the patients received proton beam radiotherapy alone (60 Gy in 4 fractions) and the other half received the same dose of proton beam radiotherapy followed by TTT at 1, 6 and 12 months. All the information concerning the initial tumour parameters, treatments and follow-up was recorded and a statistical analysis was performed. RESULTS: We randomized 151 patients. The median follow-up was 38 months. The 2 groups of patients were similar in terms of age, gender and tumour characteristics. The patients treated with TTT showed a greater reduction of tumour thickness (p = 0.06), less retinal detachment at the latest follow-up (p = 0.14) and a lower secondary enucleation rate (p = 0.02). DISCUSSION: The present study is the first randomized analysis to demonstrate a significant decrease in the secondary enucleation rate in patients treated with TTT after proton beam radiotherapy. Further studies should be performed to determine whether TTT could be beneficial to smaller tumours and to define its optimal dose.