骨侵蚀在眼眶横纹肌肉瘤中的预后作用：来自欧洲儿科软组织肉瘤研究组（EpSSG）的报告。
Prognostic role of bone erosion in orbital RMS: a report from the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG).
作者信息
Di Carlo Daniela, Fichera Giulia, Minard-Colin Veronique, Coppadoro Beatrice, Orbach Daniel, Cameron Alison, Albiac Ramos Monica, Ben Arush Myriam, Merks Johannes H M, Bisogno Gianni
机构信息
Department of Women's and Children's Health, University of Padova, Padova, Italy.
Pediatric Hematology-Oncology Division, University Hospital of Padova, Padova, Italy.
出版信息
Front Oncol. 2024 Dec 12;14:1497193. doi: 10.3389/fonc.2024.1497193. eCollection 2024.
BACKGROUND
Orbital rhabdomyosarcoma (RMS) is often limited to the orbital cavity and has a favorable prognosis. In some cases, the tumor can erode the orbital bone and behave as a parameningeal RMS (PM-RMS); thus, it is treated more intensively. However, the current protocols do not provide any guidance on how to consider different grades of bone erosion (BE) that can vary widely, hampering a uniform classification and the subsequent treatment assignment. With the aim of clarifying the role of BE as a risk factor, we analyzed patients with orbital RMS included in the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol.
METHODS
We retrospectively analyzed the radiological reports of 199 patients with orbital RMS (PM or not) and defined three grades of BE: minimal (thinning of the bone), moderate (focal bone lysis), and extensive (complete cortical destruction).
RESULTS
BE was present in 55 of the 199 (27.6%) patients, which was classified as minimal in 27, moderate in 7, and extensive in 21. Tumors with extensive BE were more frequently large (>5 cm, = 0.0008) and invasive (T2, = 0.001). With a median follow-up of 70.4 months (range = 7.1-167.7), a total of 183 patients are alive, with 5-year event-free survival (EFS) and overall survival (OS) rates of 76% (95%CI = 69.2-81.3) and 92% (95%CI = 86.7-94.8), respectively. Patients without any BE had better OS (95% . 81%, = 0.001), but not EFS. Patients with no/minimal/moderate BE had better EFS and OS compared with patients with extensive BE [EFS of 78.1 (95%CI = 71.1-83.5) . 57.1 (95%CI = 33.8-74.9), = 0.0114, respectively, and OS of 94.0 (95%CI = 89.2-96.8) . 71.1 (95%CI = 46.6-85.9), < 0.0001, respectively]. Events and metastatic relapses (in all cases CNS/meningeal) were more frequent in patients with extensive BE.
CONCLUSIONS
Only those patients with orbital RMS and extensive BE should be considered as PM and should be treated accordingly.
背景
眼眶横纹肌肉瘤（RMS）通常局限于眼眶腔，预后良好。在某些情况下，肿瘤可侵蚀眼眶骨，表现为脑膜旁横纹肌肉瘤（PM-RMS）；因此，其治疗更为强化。然而，目前的方案未就如何考虑不同程度的骨侵蚀（BE）提供任何指导，而骨侵蚀程度差异很大，这妨碍了统一分类及后续的治疗安排。为阐明BE作为危险因素的作用，我们分析了纳入欧洲儿科软组织肉瘤研究组（EpSSG）方案的眼眶RMS患者。
方法
我们回顾性分析了199例眼眶RMS患者（无论是否为PM-RMS）的放射学报告，并定义了三个BE等级：轻度（骨变薄）、中度（局限性骨质溶解）和重度（皮质完全破坏）。
结果
199例患者中有55例（27.6%）存在BE，其中27例为轻度，7例为中度，21例为重度。伴有重度BE的肿瘤更常体积较大（>5 cm，P = 0.0008）且具有侵袭性（T2，P = 0.001）。中位随访70.4个月（范围 = 7.1 - 167.7个月），共有183例患者存活，5年无事件生存率（EFS）和总生存率（OS）分别为76%（95%CI = 69.2 - 81.3）和92%（95%CI = 86.7 - 94.8）。无任何BE的患者OS更好（95%……81%，P = 0.001），但EFS并非如此。与伴有重度BE的患者相比，无/轻度/中度BE的患者EFS和OS更好[EFS分别为78.1（95%CI = 71.1 - 83.5）……57.1（95%CI = 33.8 - 74.9），P = 0.0114，OS分别为94.0（95%CI = 89.2 - 96.8）……71.1（95%CI = 46.6 - 85.9），P < 0.0001]。重度BE患者的事件和转移复发（所有病例均为中枢神经系统/脑膜）更为常见。
结论
只有那些伴有重度BE的眼眶RMS患者应被视为PM-RMS，并应相应地进行治疗。
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