• Yi Zhang and Weimin He.
• Department of Ophthalmology, West China Hospital of Sichuan University, No. 37 Guoxue Xiang, Wuhou District, Chengdu, Sichuan Province, China.
• Medicine (Baltimore). 2020 Nov 6; 99 (45): e22922.

RationaleGanglioneuroblastoma (GNB) is a transitional tumor of sympathetic origin that has never been described as primarily involving the orbit. Herein we report an extremely rare case of GNB with primary orbital involvement and its treatment strategies.Patient ConcernsA 9-year-old girl presented with progressive and recurring right orbital mass for 2 years.DiagnosisComputed tomography (CT) showed a well-defined, well-circumscribed, and homogeneous extraconal soft tissue mass occupying most of the right superior orbital area. Magnetic resonance imaging (MRI) revealed that there was a neoplasm of the right superior orbit molding around the globe with long T1 and T2 signals, and contrast-enhanced MR image showed a heterogeneous enhancement of the mass. Histopathologic examinations were performed after surgery and the characteristics were consistent with a diagnosis of GNB.InterventionsSurgery was performed and the mass was completely resected.OutcomesPostoperatively, the patient was on a regular follow-up for 19 months and so far, has had no orbital mass recurrence.LessonsHerein we present a rare case of GNB primarily involving the orbit, and the findings showed that GNB could originate from the orbit. The patient underwent surgical tumor resection. The histopathological and immunohistochemical features were consistent with the diagnosis of GNB. For this case, there was no recurrence for 19 months after complete surgical excision of the tumor; however, a regular long-term follow-up is required.

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