Primary orbital melanoma: a case series and literature review

ABSTRACT

Primary orbital melanoma (POM) is a very rare condition. We report further four cases and review all previously reported cases. We present a multicentre retrospective review of patients with POM. Clinical, radiological, surgical, histological, and follow-up data is presented. Four patients with POM were identified between 2000 and 2013. All presented with proptosis and diplopia without reduced vision. Two had known pre-existing blue cell naevi. All were stage T1N0M0. All underwent exenteration with adjuvant radiotherapy. All are disease free at follow-up durations of 24–151 months. The present three cases and review of all cases in the literature suggest a higher likelihood of disease-free survival from primary exenteration (7/8 disease-free survival, 1/8 death from metastatic disease) than wide local excision (7/16 disease-free survival, 9 recurrence or metastasis of whom 4 died). Adjuvant radiotherapy may additionally improve outcomes.

KEYWORDS:

• Adjuvant treatment
• blue cellular naevus
• congenital ocular melanocytosis
• exenteration
• orbit
• primary orbital melanoma

View correction statement:

Correction to: Figueira et al., Primary orbital melanoma: a case series and literature review

Acknowledgments

We would like to acknowledge the histopathological expertise of Dr Andrew Boon, Professor Kenneth MacLennon, and Dr Aruna Chakrabarty (all of Leeds Teaching Hospitals NHS Trust) who all examined the lesion of case 4.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.