Gorodezki David, Tonagel Felix, Zipfel Julian, Mezger Markus, Haas-Lude Karin, Holzer Ursula, Nägele Thomas, Schuhmann Martin Ulrich, Ebinger Martin
Department of Hematology and Oncology, University Children's Hospital Tübingen, Tübingen, Germany.
Centre for Ophthalmology, University Eye Hospital, University of Tübingen, Tübingen, Germany.
Childs Nerv Syst. 2025 Jul 11;41(1):230. doi: 10.1007/s00381-025-06890-6.
Optic pathway gliomas (OPGs) bear a high risk of visual acuity (VA) impairment, while balancing disease-related morbidity and potential therapy-related sequelae remains challenging. We assess the predictive value of MRI-based, three-dimensionally assessed tumor burden and growth velocity for VA outcomes in NF1-associated and sporadic OPGs.
Thirty-three OPG patients were longitudinally observed over a median follow-up period of 10.3 years, while sequential tumor volumetry was performed to assess tumor burden and tumor growth velocity. VA was assessed as minimal angle of resolution (logMAR) with age-appropriate measures during regular ophthalmology visits.
During observation, therapeutic intervention was initiated in 64% of individuals due to VA deterioration or radiological progress. Impaired VA occurred in 55 vs 80% of NF1-associated vs sporadic OPG patients, respectively, while blindness of at least one eye occurred in 36% of individuals. Tumor burden showed significant correlation with VA impairment at diagnosis and individual logMAR change during follow-up (R = .22, p = .011), while individual tumor growth velocity during follow-up showed correlation with VA deterioration in non-NF1 OPG (R = .35, p = .04). ROC analyses identified a threshold tumor burden (> 11.1 cm, AU ROC 0.76) and growth velocity (> 0.1 cm/month, AU ROC 0.81) to predict loss of VA with modest sensitivity and specificity.
These data indicate a predictive value of three-dimensionally assessed tumor burden and growth velocity for VA outcomes in OPG. Prospective evaluation within future trials may facilitate further implementation for advanced stratification, potentially identifying patients who may benefit from early therapeutic intervention.
视路胶质瘤（OPG）具有较高的视力（VA）损害风险，而平衡疾病相关的发病率和潜在的治疗相关后遗症仍然具有挑战性。我们评估基于MRI的三维评估肿瘤负荷和生长速度对神经纤维瘤病1型（NF1）相关和散发性OPG患者视力预后的预测价值。
对33例OPG患者进行了为期10.3年的纵向观察，同时进行连续肿瘤体积测量以评估肿瘤负荷和肿瘤生长速度。在定期眼科就诊时，采用适合年龄的测量方法将VA评估为最小分辨角（logMAR）。
在观察期间，64%的患者因视力恶化或影像学进展而开始接受治疗干预。NF1相关OPG患者和散发性OPG患者的视力受损发生率分别为55%和80%，而至少一只眼睛失明的发生率为36%。肿瘤负荷与诊断时的视力损害以及随访期间个体logMAR变化显著相关（R = 0.22，p = 0.011），而非NF1型OPG患者随访期间的个体肿瘤生长速度与视力恶化相关（R = 0.35，p = 0.04）。ROC分析确定了预测视力丧失的肿瘤负荷阈值（> 11.1 cm，AU ROC 0.76）和生长速度阈值（> 0.1 cm/月，AU ROC 0.81），其敏感性和特异性中等。
这些数据表明三维评估的肿瘤负荷和生长速度对OPG患者的视力预后具有预测价值。未来试验中的前瞻性评估可能有助于进一步实施高级分层，潜在地识别可能从早期治疗干预中获益的患者。
