Rose, AM;
Luthert, PJ;
Jayasena, CN;
Verity, DH;
Rose, GE;
(2017)
Primary Orbital Melanoma: Presentation, Treatment, and Long-term Outcomes for 13 Patients.
Frontiers in Oncology
, 7
, Article 316. 10.3389/fonc.2017.00316.
Preview |
Text
fonc-07-00316.pdf - Published Version Download (492kB) | Preview |
Abstract
Background: Periocular melanoma is a rare but often deadly malignancy that arises in the uvea (commonest origin), conjunctiva or orbit (rarest primary site). Melanoma accounts for 5–10% of metastatic/secondary orbital malignancies, but only a tiny proportion of primary orbital neoplasia. Primary orbital melanoma (POM) is exceedingly rare, with approximately 50 cases reported to date. Methods: All patients seen in the orbital unit at a tertiary referral hospital (1991–2016) with a biopsy-proven diagnosis of POM were identified from a diagnostic database and were studied. The case notes, imaging, surgical approach, and histology were reviewed. Results: Thirteen patients (five male; 38%) presented with isolated malignant melanoma of the orbit, for which no other primary site was identified at presentation or during an average follow-up of 44 months (median 22; range 0–13 years). The patients presented between the ages of 40 and 84 years (mean 55.5; median 48 years) and typically gave a short history of rapidly increasing proptosis and eyelid swelling. On the basis of history, a malignant lesion was suspected in most patients and all underwent incisional biopsy, with debulking of the mass in 10 (77%) patients, and skin-sparing exenteration in 3/13 (23%). Ten patients underwent orbital radiotherapy and the survival to date ranged from 9 months to 14 years (mean 55 months; median 23 months); two patients received solely palliative care for widespread disease and one patient refused orbital radiotherapy. Five of the 13 (38%) patients died from the disease. Discussion: POM is a very rare malignancy, but clinical analysis of this cohort gives insight into disease presentation and prognosis. The tumor typically presents with a rapidly progressive, well-defined mass that is, in some cases, amenable to macroscopically intact excision. Unusual for malignant melanoma, some of these patients can show an unusually long period of quiescent disease after surgical debulking and radiotherapy.
| Type: | Article |
|---|---|
| Title: | Primary Orbital Melanoma: Presentation, Treatment, and Long-term Outcomes for 13 Patients |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3389/fonc.2017.00316 |
| Publisher version: | https://doi.org/10.3389/fonc.2017.00316 |
| Language: | English |
| Additional information: | This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Oncology, primary orbital melanoma, orbital malignancy, ocular melanoma, melanoma, orbital surgery, MALIGNANT-MELANOMA, UVEAL MELANOMA, BLUE NEVUS, TUMORS, METASTASIS, MANAGEMENT, FEATURES, LECTURE |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10041341 |
Archive Staff Only
 |
View Item |
