Ophthalmic treatments are successful in managing uveal melanomas achieving good local control. However, a large number still metastasise, primarily to the liver, resulting in mortality. There is no consensus across the world on the mode, frequency, duration or utility of regular liver surveillance for metastasis and there are no published protocols. The Scottish Ocular Oncology Service (SOOS) constituted a Scottish Consensus Statement Group (SCSG) which included ocular oncologists, medical oncologists, radiologists and a uveal melanoma patient as a lay member. This group carried out an extensive review of literature followed by discussions to arrive at a consensus regarding surveillance planning for posterior uveal melanoma patients in Scotland. The Consensus Statement would provide a framework to guide each patient\'s surveillance plan and provide all patients with clarity and transparency on the issue. The SCSG was unable to find adequate evidence on which to base the strategy. The consensus statement recommends a risk-stratified approach to surveillance for these patients dividing them into low to medium-risk and high-risk groups defining the mode and duration of surveillance for each. It supplements the UK-wide Uveal Melanoma National Guidelines and allows a more uniform consensus-based approach to surveillance in Scotland. It has been adopted nationally by all health care providers in Scotland as a guideline and is available to patients on a publicly accessible website.
摘要: 眼科能成功治疗葡萄膜黑色素瘤, 并有效控制葡萄膜黑色素瘤的发展转移。尽管如此, 仍有大量患者发生转移, 葡萄膜黑色素瘤主要转移到肝脏并导致死亡。目前世界上关于葡萄膜黑色素瘤肝脏转移定期监测的模式、频率、持续时间及实用程度并没有共识, 且没有已发表的方案。苏格兰眼肿瘤协会(SOOS)建立了苏格兰共识声明小组(SCSG), 小组成员包括眼肿瘤医生、肿瘤科医生、放射科医生以及一位作为非专业人员的葡萄膜黑色素瘤患者。该小组对文献进行了广泛回顾和讨论, 并且对苏格兰后葡萄膜黑色素瘤患者的监测达成共识。该共识声明小组将提出框架以指导每位患者的病情监测, 并且在病情上对所有患者保持公开透明。SCSG并未找到充足证据作为该策略的基础。该临床共识建议将所有患者根据风险分级分为低、中、高风险组, 定义每个组的监测模式及持续时间。该共识补充说明了全英国葡萄膜黑色素瘤国家指南, 并促使在苏格兰采用更统一的并基于共识的监测方法。它已在全苏格兰内被所有医生作为指南, 并向患者提供一个公众开放的网站。.

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.

Uveal melanomas are the most frequent primary malignant eye tumor. Enucleation was historically the gold standard. Since then, several studies showed that conservative treatments did not increase the risk of metastasis or survival. Choroidal melanomas are both radioresistant and located close to visual structures (the optic nerve and macula) of the eye, which may be preserved in some settings without compromising tumor control, as this is the first priority. Different types of radiation therapy may be used for such tumors: brachytherapy and charged particles, including proton beam therapy. If visual prognosis is dependent to the local treatment, the vital prognosis is dependent on the metastatic risk, with a risk of liver involvement in 20 to 50% of patients, depending on tumor size and genomics. Median survival after the discovery of liver metastases is about 15 months. The management of these patients is often complex. Systemic therapies (chemotherapy, targeted therapies, immunotherapy, etc.) yield limited response rates and although local treatments of liver metastases are promising, they are only feasible in selected patients. The mission of the MELACHONAT national network is to improve the management of patients regardless of the stage of the disease. The patient association ANPACO is dedicated to help uveal melanoma patients in their health care path and to promote knowledge dissemination within the patient community. The aim of this review is to focus on the local treatments of uveal melanomas as well as the management of their metastatic evolution.

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.

OBJECTIVE: 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.
CONCLUSIONS: 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.

Retinoblastoma is the most common intraocular malignant childhood tumor in need of prospective clinical trials to address important unanswered questions about biology, treatment, and prognostic factors. Currently, there is controversy about the definitions for choroidal invasion and an inconsistency in the handling of eyes with retinoblastoma. The International Retinoblastoma Staging Working Group (IRSWG) composed of 58 participants from 24 countries on 4 continents had a series of Internet meetings to discuss the staging and tissue handling guidelines to reach consensus for adequate processing, establishing definitions of histopathologic risk factors, and reporting of enucleated eyes with retinoblastoma to serve as the basis for clinical trials and studies to validate the proposed criteria. The meetings were facilitated by the International Outreach Program of the St. Jude Children\'s Research Hospital through Cure4Kids. The retinoblastoma guidelines from the Children\'s Oncology Group, the French Society for Pediatric Cancers, the Association of Directors of Anatomic and Surgical Pathology, and some published data were the basis for this consensus document. Discussions of the feasibility, practicality, and efficacy of the guidelines and criteria resulted in this report. The consensus definitions reached included definition of massive choroidal invasion stated as a maximum diameter of invasive tumor focus of 3 mm or more that may reach the scleral tissue. Focal choroidal invasion is defined as a tumor focus of less than 3 mm and not reaching the sclera. Optic nerve invasion is classified as prelaminar, laminar, retrolaminar, or tumor at surgical margin, and the measurement of the depth of invasion should also be recorded. These guidelines also address handling of the enucleated eye with retinoblastoma in an efficient, practical, and feasible manner for a meaningful diagnosis. The consensus criteria reached by the IRSWG should be validated through prospective clinical trials and studies.