J Korean Ophthalmol Soc > Volume 58(3); 2017 > Article
Journal of the Korean Ophthalmological Society 2017;58(3):333-336.
DOI: https://doi.org/10.3341/jkos.2017.58.3.333    Published online March 15, 2017.
Solitary Pigmented Plexiform Neurofibroma in Lower Eyelid: A Case Report.
Hyeon Jeong Yoon, Kyung Chul Yoon, Ga Eon Kim, Won Choi
1Department of Ophthalmology, Chonnam National University Medical School, Gwangju, Korea. wchoi82@hanmail.net
2Department of Pathology, Chonnam National University Medical School, Gwangju, Korea.
아래눈꺼풀에서 발생한 단독 색소 얼기신경섬유종 1예
윤현정1⋅윤경철1⋅김가언2⋅최 원1
전남대학교 의과대학 안과학교실1, 전남대학교 의과대학 병리학교실2
Correspondence:  Won Choi,
Email: wchoi82@hanmail.net
Received: 3 November 2016   • Revised: 12 December 2016   • Accepted: 1 February 2017
Solitary plexiform neurofibroma of the eyelid without neurofibromatosis is a rare disease. We report a case of solitary plexiform pigmented neurofibroma of the eyelid without neurofibromatosis. CASE SUMMARY: A 12-year-old male visited our clinic with a painless palpable subcutaneous mass on the right lower eyelid. He had a history of Batter syndrome and attention deficit hyperactivity disorder. On initial presentation, clinical features regarding neurofibromatosis such as Lisch nodule, optic nerve glioma, or high myopia were not observed. We performed excision and biopsy of the lower lid mass under general anesthesia. Macroscopically, the tumor was 4.0 × 1.5 × 1.5 cm in size with irregular nodules. Microscopically, the tumor consisted of multiple, variably sized tortous enlarged nerve fascicles with clusters of pigmented cells. Immunohistochemical results revealed expression of S-100 protein. Pigmented cells express both S-100 and melan-A proteins, while nonpigmented cells express S-100 protein only. The tumor was finally diagnosed as plexiform pigmented neurofibroma. Dermatological evaluation revealed no evidence of systemic neurofibromatosis. CONCLUSIONS: Plexiform neurofibroma should be considered in the differential diagnosis of an eyelid mass, even if the patient does not have a history or clinical features of neurofibromatosis. Plexiform neurofibroma can be successfully managed with surgical excision.
Key Words: Eyelid neurofibroma;Neurofibromatosis;Plexiform neurofibroma

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