Frontiers in medicine · 2026
Two distinctly located primary orbital melanomas following evisceration for congenital corneal staphyloma: a case report and brief literature review.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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Ошибки и ограничения
История · было → стало
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Zhou Chaona, Wu Pengsen, Ying Fangwei, Rao Jing, Wen Yanlin, Liu Guiqin
Аннотация
PURPOSE: To report an unusual case of two distinct primary orbital melanomas emerged 9 years following evisceration for congenital corneal staphyloma and implantation of a MEDPOR® prosthesis.
CASE DESCRIPTION: A 14-year-old female with congenital blindness and progressive right globe enlargement since birth, accompanied by multiple cutaneous blue nevi, initially underwent right eye evisceration with MEDPOR® implant placement in 2012, revealing congenital corneal staphyloma and proliferative vitreoretinopathy on histopathology. Nine years later, she presented with chronic right eye tearing and intermittent bloody discharge. Examination revealed a prominent scalp blue nevus with satellite lesions, mild orbital implant protrusion, conjunctival congestion, and a subconjunctival black mass. MRI identified a 1.9 cm T1-slight hyperintense, T2-hypointense mass superior to the implant, with inferior rectus thickening. Surgical excision yielded two masses: a 41 mm inferonasal lesion (partially pigmented, unencapsulated) and a 20 mm superior-posterior black, spherical mass. Histopathology confirmed orbital malignant melanoma, with tumor cells positive for S-100 (3+), Melan-A (3+), and HMB-45 (2+). NGS revealed no pathogenic variants, and PET/CT showed no metastasis. She subsequently underwent postoperative radiotherapy and maintains good overall health until June 2025.
DISCUSSION: This article reports a case of primary orbital melanoma presenting as two tumors within a single orbit following evisceration, with a focus on elucidating the origin of tumor cells and their relationship with pigmented nevi and orbital implants.
CONCLUSION: Blue nevi may represent an underrecognized risk factor for primary orbital melanoma, and enucleation may be preferable to evisceration for idiopathic corneal staphyloma to reduce melanoma risk.
CASE DESCRIPTION: A 14-year-old female with congenital blindness and progressive right globe enlargement since birth, accompanied by multiple cutaneous blue nevi, initially underwent right eye evisceration with MEDPOR® implant placement in 2012, revealing congenital corneal staphyloma and proliferative vitreoretinopathy on histopathology. Nine years later, she presented with chronic right eye tearing and intermittent bloody discharge. Examination revealed a prominent scalp blue nevus with satellite lesions, mild orbital implant protrusion, conjunctival congestion, and a subconjunctival black mass. MRI identified a 1.9 cm T1-slight hyperintense, T2-hypointense mass superior to the implant, with inferior rectus thickening. Surgical excision yielded two masses: a 41 mm inferonasal lesion (partially pigmented, unencapsulated) and a 20 mm superior-posterior black, spherical mass. Histopathology confirmed orbital malignant melanoma, with tumor cells positive for S-100 (3+), Melan-A (3+), and HMB-45 (2+). NGS revealed no pathogenic variants, and PET/CT showed no metastasis. She subsequently underwent postoperative radiotherapy and maintains good overall health until June 2025.
DISCUSSION: This article reports a case of primary orbital melanoma presenting as two tumors within a single orbit following evisceration, with a focus on elucidating the origin of tumor cells and their relationship with pigmented nevi and orbital implants.
CONCLUSION: Blue nevi may represent an underrecognized risk factor for primary orbital melanoma, and enucleation may be preferable to evisceration for idiopathic corneal staphyloma to reduce melanoma risk.