Primary orbital neuroblastoma: Case report and literature review

Neuroblastoma, the most common extracranial solid tumor in children, typically originates in the adrenal glands but can occasionally present as primary orbital neuroblastoma. This rare condition, arising from the ciliary ganglion, poses significant diagnostic and therapeutic challenges. We report a case of primary orbital neuroblastoma in a 2.5-year-old female with progressive, painless swelling of the right lower eyelid. Clinical examination showed mild proptosis and a firm lower lid mass. Imaging and histopathology confirmed neuroblastoma, with no other primary sites identified, indicating primary orbital neuroblastoma. The patient responded well to chemotherapy and radiotherapy. A review of the literature revealed only 12 previously reported cases. This malignancy often presents with ocular symptoms and lacks a primary site elsewhere. Diagnosis relies on histopathology and immunohistochemistry. Due to its rarity, there are no standardized treatment protocols, necessitating individualized, multidisciplinary management and long-term follow-up to improve outcomes and understand prognosis.

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Publication Details

Journal
Saudi Journal of Ophthalmology
Published
2025-02-21
DOI
https://doi.org/10.4103/sjopt.sjopt_271_24
Primary Topic
Neuroblastoma Research and Treatments
Type
article
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article

Primary orbital neuroblastoma: Case report and literature review

Surbhi Saboo, Sima Das, Chhavi Gupta, Arpan Gandhi
Saudi Journal of Ophthalmology
Neuroblastoma Research and Treatments
article

Primary orbital neuroblastoma: Case report and literature review

Surbhi Saboo, Sima Das, Chhavi Gupta, Arpan Gandhi
article en

Abstract

Neuroblastoma, the most common extracranial solid tumor in children, typically originates in the adrenal glands but can occasionally present as primary orbital neuroblastoma. This rare condition, arising from the ciliary ganglion, poses significant diagnostic and therapeutic challenges. We report a case of primary orbital neuroblastoma in a 2.5-year-old female with progressive, painless swelling of the right lower eyelid. Clinical examination showed mild proptosis and a firm lower lid mass. Imaging and histopathology confirmed neuroblastoma, with no other primary sites identified, indicating primary orbital neuroblastoma. The patient responded well to chemotherapy and radiotherapy. A review of the literature revealed only 12 previously reported cases. This malignancy often presents with ocular symptoms and lacks a primary site elsewhere. Diagnosis relies on histopathology and immunohistochemistry. Due to its rarity, there are no standardized treatment protocols, necessitating individualized, multidisciplinary management and long-term follow-up to improve outcomes and understand prognosis.

Saudi Journal of OphthalmologyVol. 39(3)
Dr. Shroff’s Charity Eye Hospital (IN)
Openalex Percentile: Top 95%
Neuroblastoma Research and Treatments
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Primary orbital neuroblastoma: Case report and literature review — Surbhi Saboo, Sima Das, et al. · Saudi Journal of Ophthalmology (2025) | TGRS Research Map | TGRS