Abstract

To report long-term outcomes after surgery for orbital solitary fibrous tumors. Retrospective review of patients with orbital solitary fibrous tumor, first seen between 1971 and 2022. Primary excisions were grouped as (A) surgically intact, (B) macroscopic, but with cell spillage, or (C) known incomplete excision. Fifty-nine patients (31 female; 53%) presented at a mean age of 43.0 years (19-82 years), with 5 patients (8.5%) having malignant solitary fibrous tumors. Average follow up was 11.4 years (median 7.8; range 1-43 years). There were 28 of 59 (47%) group A patients with 1 of 28 (3%) having recurrence, 20 of 59 (34%) group B having 6 of 20 (30%) recurrences, and 11 of 59 (19%) group C with 9 of 11 (82%) recurrence ( p < 0.001 for recurrence rate). At a mean of 8.9 (range 1-23.6) years after initial treatment, continued local tumor growth was evident in 16 (27%) patients, with higher-grade recurrence in 3 of 14 (21%) cases. No patient had systemic disease at presentation, but 2 of 59 patients (3%) developed metastases at 22 and 30 years after first treatment. The 10-year progression-free survival was 94% (group A), 60% (group B), and 36% (group C). Tumor disruption or incomplete excision (groups B + C) carries the highest risk of tumor recurrence (hazard ratio 15.0; 95% confidence interval, 1.98-114; p = 0.009), with no correlation to tumor size or histology. Orbital solitary fibrous tumors have a low recurrence rate with surgically intact excision; piecemeal excision, capsular disruption, or known incomplete resection have a high recurrence rate, which can occur decades later. Baseline postoperative scans is recommended, together with long-term clinical and interval imaging.

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