A proposal for an international retinoblastoma staging system
A proposal for an international retinoblastoma staging system
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DOI：
10.1002/pbc.20606
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发表时间：
2006-11-01
影响因子：
3.2
通讯作者：
Zucker, Jean-Michel
中科院分区：
文献类型：
--
作者：
Chantada, Guillermo;Doz, Francois;Zucker, Jean-Michel
背景资料。虽然视网膜内肿瘤长期以来一直根据Reese-Ellsworth(R-E)系统进行手术前分期，但视网膜母细胞瘤与其他儿科肿瘤的不同之处在于，从来没有一个广泛接受的涵盖整个疾病谱系的分类系统。在考虑疾病扩展、眼外复发的危险因素和治疗反应的研究之间的比较需要一个普遍接受的眼外疾病分期系统。程序。一个由来自世界各地大型中心的视网膜母细胞瘤专家组成的委员会已经开发出一种共识分类，该分类可以涵盖所有视网膜母细胞瘤病例，并在此提出。根据疾病的程度和是否有明显的眼球外延对患者进行分类。此外，为了进一步区分I期和11期患者，还提出了考虑去核标本的组织病理学特征的分期建议。结果。以下是制定的分类系统摘要-第0期：保守治疗的患者(服从术前眼科分类)；第1期：眼球摘除，组织学完全切除；II期：眼球摘除，微小残留肿瘤；III期：区域性侵犯[(A)显性眼眶疾病，(B)耳前或颈部淋巴转移]；IV期：转移性疾病[(A)血行转移：(1)单发病灶，(2)多发病灶；(B)中枢神经系统延伸：(1)交叉前病变，(2)中枢神经系统肿块，(3)软脑膜疾病]。还提出了一项关于I期和11期眼球摘除的建议。结论。拟议的分期系统是国际努力的产物，目的是为视网膜母细胞瘤患者采用统一的分期系统，以涵盖疾病的全部范围。
Background. Although intra-retinal tumor has long been staged presurgically according to the Reese-Ellsworth (R-E) system, retinoblastoma differs from other pediatric neoplasms in never having had a widely accepted classification system that encompasses the entire spectrum of the disease. Comparisons among studies that consider disease extension, risk factors for extra-ocular relapse, and response to therapy require a universally accepted staging system for extra-ocular disease. Procedure. A committee of retinoblastoma experts from large centers worldwide has developed a consensus classification that can encompass all retinoblastoma cases and is presented herein. Patients are classified according to extent of disease and the presence of overt extra-ocular extension. In addition, a proposal for substaging considering histopathological features of enucleated specimens is presented to further discriminate between Stage I and 11 patients. Results. The following is a summary of the classification system developed-Stage 0: Patients treated conservatively (subject to presurgical ophthalmologic classifications); Stage 1: Eye enucleated, completely resected histologically; Stage II: Eye enucleated, microscopic residual tumor; Stage III: Regional extension [(a) overt orbital disease, (b) preauricularor cervical lymph node extension]; Stage IV: Metastatic disease [(a) hematogenous metastasis: (1) single lesion, (2) multiple lesions; (b) CNS extension: (1) prechiasmatic lesion, (2) CNS mass, (3) leptomeningeal disease]. A proposal is also presented for substaging of enucleated Stages I and 11 eyes. Conclusions. The proposed staging system is the product of an international effort to adopt a uniform staging system for patients with retinoblastoma to cover the whole spectrum of the disease.