Intraocular schwannoma: case series of 28 patients and literature review

• Published in: Eye (London) Vol. 38; no. 7; pp. 1314 - 1319
• Main Authors: Dong, Li, Xu, Xiao-Lin, Li, He-Yan, Yang, Qiong, Zhang, Rui-Heng, Wei, Wen-Bin, Liu, Yue-Ming
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.05.2024; Nature Publishing Group
• Subjects: 631/443; 631/67; Adolescent; Adult; Child; Choroid Neoplasms - diagnosis; Choroid Neoplasms - pathology; Choroid Neoplasms - surgery; Ciliary Body - pathology; Ciliary Body - surgery; Eye Neoplasms - diagnosis; Eye Neoplasms - pathology; Eye Neoplasms - surgery; Female; Humans; Laboratory Medicine; Literature reviews; Male; Medicine; Medicine & Public Health; Melanoma; Metastases; Middle Aged; Neurilemmoma - diagnosis; Neurilemmoma - pathology; Neurilemmoma - surgery; Ophthalmology; Patients; Pharmaceutical Sciences/Technology; Retrospective Studies; Schwann cells; Surgery; Surgical Oncology; Tumors; Uveal Neoplasms - diagnosis; Uveal Neoplasms - pathology; Uveal Neoplasms - surgery; Visual Acuity - physiology; Visual field; Vitrectomy; Young Adult
• ISSN: 0950-222X; 1476-5454; 1476-5454
• DOI: 10.1038/s41433-023-02890-5

Purpose Intraocular schwannoma is a rare tumour, which is often misdiagnosed. We presented the demographics and clinical characteristics of patients with intraocular schwannoma. Methods Retrospective case series were collected between May 2005 and July 2021 in Beijing Tongren Hospital. Results A total of 28 patients were diagnosed with intraocular schwannoma on histopathological examination of surgical specimen. The median age was 39 years (range: 12–64). Fourteen patients were female and 14 were male. Among the all subjects, 21/28 patients (75.0%) presented as visual loss, and 3/28 patients (10.7%) had visual field loss. Intraocular schwannoma presented as nonpigmented mass in the ciliary body in 12/28 cases (42.9%), in the choroid in 9/28 cases (32.1%), and in ciliochoroid in 7/28 cases (25.0%). Intraocular schwannoma was often clinically misdiagnosed as uveal melanoma, which occurred in 16/28 patients (57.1%). Tumour excision with pars plana vitrectomy was performed for all included patients. Endoresection with lens removal was performed for tumours in the choroid, while transscleral resection was performed for tumours located in ciliary body or ciliochoroid. Increased light transmission was detected in 12/28 cases (42.9%). In the consecutive follow-up (median: 73 months, range: 7–193 months), no cases of recurrence or metastatic disease were detected. Conclusions Intraocular schwannoma is a rare benign tumour. It usually presents as nonpigmented mass, which can easily be misdiagnosed as nonpigmented uveal melanoma.