網膜芽細胞腫における視神経層外浸潤に対する術後化学療法：世界多施設共同研究

Michelle Lin¹, Sarah B Pike¹, Mark W Reid²

¹The Vision Center, Children's Hospital Los Angeles, Los Angeles, CA, United States; USC Roski Eye Institute, University of Southern California Keck School of Medicine, Los Angeles, CA, United States.

Ophthalmology. Retina

|December 31, 2025

要旨

Purpose:

To evaluate outcomes of patients with retinoblastoma (RB) who underwent primary enucleation with histopathologic evidence of post-laminar optic nerve invasion (PLONI), comparing those who received adjuvant chemotherapy to those who did not.

Design:

Multicenter retrospective cohort study.

Participants:

292 patients across 9 countries with RB and histopathologic evidence of PLONI after primary enucleation.

Methods:

Patients who received adjuvant chemotherapy (n=276) were compared to those who did not (n=16) using Fisher's Exact tests, Welch's t-tests, and proportional hazard models (Cox and competing risks). Sub-analyses were performed for PLONI and massive choroidal invasion and isolated PLONI.

Main Outcome Measures:

Orbital tumor recurrence, metastasis, and death.

Results:

Patients who did not receive adjuvant chemotherapy had higher rates of orbital tumor recurrence (31% vs. 2%, P < 0.001), metastasis (31% vs. 6%, P = 0.001), and death (38% vs. 9%, P = 0.007). In time-to-event analyses, patients who did not receive adjuvant chemotherapy experienced greater hazard of orbital tumor recurrence (Sub-Hazard Ratio SHR = 19.31, 95% CI: 5.56-67.05), metastasis (SHR = 5.39, 95% CI: 1.75-16.60), and death (HR = 4.74, 95% CI: 1.75-12.81). Outcomes were similar among patients with PLONI and massive choroidal invasion. Among patients with isolated PLONI, those who did not receive adjuvant chemotherapy showed significantly greater hazard of metastasis over time than those who received adjuvant chemotherapy (SHR = 40.68, 95% CI: 9.65-171.55, P < 0.001). All patients with metastasis or orbital tumor recurrence eventually died during follow-up (mean duration: 75.3 months).

Conclusion:

In patients with RB and PLONI with additional high-risk histopathological features, initiating adjuvant chemotherapy after primary enucleation significantly reduced rates of orbital tumor recurrence, metastasis, and death. Although patients with isolated PLONI experienced fewer events and had a lower metastatic risk compared to those with multiple histopathological features, adjuvant chemotherapy still reduced metastatic risk over time.

キーワード:

網膜芽細胞腫術後化学療法死亡転移眼窩腫瘍再発視神経層外浸潤原発摘出術

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