NCCN Guidelines Insights: Uveal Melanoma, Version 1.2019.

The NCCN Guidelines for Uveal Melanoma include recommendations for staging, treatment, and follow-up of patients diagnosed with uveal melanoma of the choroid or ciliary body. In addition, because distinguishing between uveal melanoma and benign uveal nevi is in some cases difficult, these guidelines also contain recommendations for workup of patients with suspicious pigmented uveal lesions, to clarify the tests needed to distinguish between those who should have further workup and treatment for uveal melanoma versus those with uncertain diagnosis and low risk who should to be followed and later reevaluated. These NCCN Guidelines Insights describe recommendations for treatment of newly diagnosed nonmetastatic uveal melanoma in patients who have already undergone a complete workup.

Impact of enucleation on adult retinoblastoma survivors' quality of life: A qualitative study of survivors' perspectives.

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.

New NCCN Guidelines for Uveal Melanoma and Treatment of Recurrent or Progressive Distant Metastatic Melanoma.

The NCCN Guidelines Panel for Melanoma debuted new guidelines for uveal melanoma at the NCCN 23rd Annual Conference. Although uveal melanoma and cutaneous melanoma share the same name, they do have different characteristics and treatments. The NCCN Guidelines describe how tumor size guides therapeutic options, which for most tumors is radiotherapy. Predictors of melanoma-related mortality include advanced age, larger tumor size, and histopathologic and molecular features. The NCCN Guidelines for Cutaneous Melanoma have not changed notably, but adjuvant therapy with immunotherapies is now recommended. The best second-line treatment in the metastatic setting remains unclear.

Evaluación y manejo de los insulinomas. Presentación de 4 casos y revisión de la literatura / Evaluation and management of the insulinomas. Report of four cases and review of the literature

Introducción: Los insulinomas son tumores neuroendocrinos poco frecuentes que habitualmente tienen buen pronóstico. El objetivo es la presentación y revisión de la literatura médica a propósito de 4 casos. Material y Métodos: Estudio descriptivo, observacional y retrospectivo de los pacientes diagnosticados e intervenidos en el Hospital Universitario de San Juan de Alicante de insulinomas, en un periodo de 18 años (1995-2013). Resultados: Nuestro grupo de estudio incluyó 4 pacientes, 1 caso en hombres (25%) y 3 en mujeres (75%), con una mediana de edad de años (rango 3; 72). La distribución de las lesiones fue : cuerpo (1 caso), proceso uncinado (1 caso), cabeza (1 caso), cola (1 caso). El diagnóstico preoperatorio se realizó mediante test del ayuno y Tac abdominal. La ecografía intraoperatoria se realizó en todos los casos. El procedimiento quirúrgico de elección fue la enucleación. Actualmente se encuentran libres de enfermedad en un seguimiento a 10 años. Conclusiones: Los insulinomas son tumores infrecuentes que presentan buen pronóstico a largo plazo. Su diagnóstico topográfico en ocasiones es complejo. El tratamiento de elección es la cirugía y el procedimiento quirúrgico empleado con mayor frecuencia es la enucleación

Background: Insulinomas are infrecuent neuroendocrine tumors that usually have a good prognosis. The goal is the presentation and review of the literature about 4 cases. Methods: A retrospective study of all patients diagnosed with insulinomas surgically managed at San Juan de Alicante Hospital during the period between 1995 and 2013 was performed. Results: We analyzed 4 patients, three females (75%) and one male (25%) with a range age (39 to 72 years). Distribution of the lesions were: body (1 case), uncinate process (1 case), head (1 case), tail (1 case). Preoperative diagnosis was made by fasting test and abdominal CT. In all cases, intraoperative ultraound was made. Surgical management of choice was enucleation. Currently, they are free of disease at 10 years of follow up. Conclusions: Insulinomas are infrecuent tumors with good long-term prognosis. Its topographic diagnosis is sometimes complex. The treatment of choice is surgery and the surgical procedure most often used is enucleation

Enucleaçäo e evisceraçäo no Instituto e Fundaçäo Hilton Rocha durante 8 anos (1987-1994) / Statistical evalution of enucleation and evisceration cases in Instituto e Fundaçäo Hilton Rocha between 1987-1994

Os autores fazem levantamento estatístico de um período de 8 anos (1987-1994) no Instituto e Fundaçäo Hilton Rocha e analisaram 501 olhos de 496 pacientes. Foram observados os seguintes aspectos: local e época das cirurgias, idade, sexo, cor profissäo, procedência, principais causas de evisceraçäo e enucleaçäo, exame anátomo-patológico, olho operado, relaçäo entre idade e tratamento prévio com tipo de cirurgia realizada. As causas mais frequentes de evisceraçäo foram: atrofia bulbar (42,4 por cento) endofitalmite/panoftalmite (18,2 por cento), glaucoma absoluto (11,4 por cento) e de enucleaçäo: retinoblastoma (27,3 por cento), atrofia bulbar (24,4 por cento), melanoma da coróide (18,9 por cento).