Primary orbital melanoma arising in an atypical diffuse (plaque-like) blue naevus/melanocytosis: a case report and review of literature

BMC Ophthalmology

Adam Gajdatsy¹  Tracey-Anne Dickens¹  Nima Mesbah Ardakani²  Maria Franchina³ 

[1] Centre for Ophthalmology and Visual Science, University of Western Australia, M581, 35 Stirling Highway, 6009, Perth, WA, Australia;Department of Anatomical Pathology, PathWest Laboratory Medicine, QEII Medical Centre, 6009, Perth, WA, Australia;School of Pathology and Laboratory Medicine, University of Western Australia, 35 Stirling Highway, 6009, Perth, WA, Australia;College of Science, Health, Engineering and Education, Murdoch University, 6150, Perth, WA, Australia;Lions Eye Institute, 2 Verdun Street, 6009, Nedlands, WA, Australia;

关键词: Atypical blue naevus;    Melanocytosis;    Primary orbital melanoma;    Case report;   

DOI  :  10.1186/s12886-021-02176-y

来源: Springer

【 摘 要 】

BackgroundPrimary orbital melanoma is a rare disease and can occasionally develop from a pre-existing neoplasm of the blue naevus family of melanocytic lesions.Case presentationHerein we report a rare case of primary orbital melanoma arising from an unusual atypical diffuse (plaque-like) blue naevus/melanocytosis. A 27 year old man presented with mild pain and swelling of the left eye. Magnetic Resonance Imaging revealed a left lateral episcleral orbital mass and an incisional biopsy confirmed the diagnosis of malignant melanoma. Skin-sparing total left orbital exenteration was performed. Histopathological examination of the exenteration specimen revealed a primary orbital melanoma arising in a pre-existing blue naevus like melanocytosis.We demonstrate the evidence for histological progression, characterise the molecular profile of this tumour and discuss the related literature.ConclusionsThis case emphasises the importance of a meticulous clinicopathological correlation in recognising such a tumour as a primary orbital melanoma rather than a metastasis, which is managed differently.

【 授权许可】

CC BY