Oncology Center SIREDO: Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer, Institut Curie, Paris, France.
Radiology Department, Institut Curie, Paris, France.
Pediatr Blood Cancer. 2020 Jan;67(1):e27998. doi: 10.1002/pbc.27998. Epub 2019 Oct 1.
Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high-risk patients.
Retrospective mono-institutional clinical, radiological, and histological review of patients with uni- or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed.
Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11-96). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10/11 patients-by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high-dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5-19.4]).
Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre-operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.
在诊断时通过影像学显示的伴有宏观视神经（ON）浸润的视网膜母细胞瘤仍然是一个主要问题，并且预后不良。我们旨在描述这些高危患者的治疗和结果。
对 Institut Curie 治疗的 936 例单侧或双侧视网膜母细胞瘤患者的临床、放射学和组织学进行回顾性单机构研究，这些患者的视神经通过计算机断层扫描或磁共振成像（MRI）显示明显的 ON 浸润，定义为放射学视神经扩大（RONE）。
在 1997 年至 2014 年期间，在 Institut Curie 接受治疗的 936 例视网膜母细胞瘤患者中，有 11 例可检测到 RONE。10 例为单侧，1 例为双侧。诊断时的中位年龄为 28 个月（范围，11-96）。视神经扩大延伸至眶内段 3 例，视神经管内段 5 例，视交叉前 2 例，视交叉内 1 例。9 例患者接受新辅助化疗，所有患者均获得部分缓解。11 例患者中，10 例行眼球摘除术，其中 3 例采用前路入路，7 例采用前路和额下入路。3 例视神经切除缘阳性（2/3 例为初次眼球摘除术后）。所有眼球摘除患者均接受辅助治疗（常规化疗：10 例，大剂量化疗：7 例，放疗：5 例）。4 例患者发生脑膜进展。7 例患者处于首次完全缓解期（中位随访时间：8 年[3.5-19.4]）。
新辅助化疗和显微镜下完全切除在伴有 RONE 的视网膜母细胞瘤的治疗中具有关键作用。MRI 推荐用于初始和术前的准确分期。如果存在后部神经侵犯，应请神经外科医生进行手术。如果切除不完全，需要放疗。
