Primary orbital melanoma. A case series

Purpose. Retrospective analysis of clinical, instrumental and pathomorphologic features of primary orbital melanoma based on our own observations between 2007 and 2024.

Materials and methods. 6 cases of primary melanoma of the orbit were examined and treated at the Department of Ophthalmic Oncology and Radiology of the Helmholtz Medical Center of the Russian Ministry of Health. The age of the patients at the time of diagnosis ranged from 18 to 65 years (mean — 48.0 ± 17.6 years). 2 patients had oculodermal melanosis. The clinical course of the disease at the stages of dynamic follow-up of the patients and the peculiarities of vital prognosis taking into account the management tactics and pathomorphological features of the tumor were evaluated.

Results. The duration of the history varied from 3 months to 3 years. The main clinical signs included exophthalmos (n = 6), restricted eye movement (n = 4) and partial ptosis (n = 3). The development of metastatic disease occurred in 3 of 6 patients: to the lungs, liver, and brain in 12, 18, and 48 months after the start of treatment, respectively. Epithelioid and mixed cell type tumors with high number of mitotic cells, congenital melanosis were the indicators of unfavorable prognosis. Life expectancy was higher in patients who underwent remote irradiation after tumor removal.

Conclusions. Primary orbital melanoma is a rare, aggressive orbital tumor that does not have specific clinical and instrumental symptoms, which complicates the diagnosis and simulates a benign orbital tumor. It is necessary to be alert to the development of this pathology in patients with primary oculodermal melanosis, which requires lifelong dynamic monitoring. The vital prognosis of patients is influenced by both the histological type of the tumor and early radiation therapy after surgical treatment. For a better understanding of the pathogenesis of the disease, it is necessary to consolidate and conduct multicenter studies with the study of the genetic profile of the disease to subsequently search for and develop optimal therapy.

1. Shields CL, Shields JA. Ocular melanoma: relatively rare but requiring respect. Clin Dermatol. 2009; 27: 122–33. doi:10.1016/j.clindermatol.2008.09.010

2. Grishina E.E., Lerner M.Yu, Gemdzhian E.G. Epidemiology of uveal melanomas in Moscow. Almanac of clinical medicine. 2017; 45 (4): 321–5 (In Russ.). doi: 10.18786/2072-0505-2017-45-4-321-325

3. Amirian A.G., Saakian S.V. Prognostic factors for uveal melanoma. Vestnik Oftal’mologii. 2015; 131 (1): 90–5 (In Russ.). doi:10.17116/oftalma2015131190-94

4. Rose AM, Luthert PJ, Jayasena CN, Verity DH, Rose GE. Primary orbital melanoma: Presentation, treatment, and long-term outcomes for 13 patients. Front Oncol. 2017; 7: 316. doi: 10.3389/fonc.2017.00316

5. Ferreira AF, da Silveira Filho LG, Dias EL. Primary orbital melanoma in an anophthalmic socket. Radiol Bras. 2019; 52 (5): 347–8. doi:10.1590/0100-3984.2017.0183

6. Figueira E, Rajak S, McKelvie P, et al. Primary orbital melanoma: a case series and literature review. Orbit. 2018; 37 (5): 352–7. doi: 10.1080/01676830.2017.1423354

7. Adetunji MO, McGeehan B, Lee V, Maguire MG, Brice o CA. Primary orbital melanoma: A report of a case and comprehensive review of the literature. Orbit. 2021; 40 (6): 461–9. doi:10.1080/01676830.2020.1818265

8. Buntinx-Krieg T, Ouyang J, Cartwright M. An orbital malignant melanoma arising in cellular blue nevus in a patient with nevus of Ota. Cureus. 2016; 8 (7): 698. doi: 10.7759/cureus.698

9. Radhadevi CV, Charles KS, Lathika VK. Orbital malignant melanoma associated with nevus of Ota. Indian J Ophthalmol. 2013; 61 (6): 306–9. doi:10.4103/0301-4738.109526

10. El-Sawy T, Bakhoum MF, Tetzlaff M, et al. Primary orbital melanoma in association with cellular blue nevus. Digit J Ophthalmol. 2014; 20 (3): 35–40. doi: 10.5693/djo.01.2014.02.001

11. Ranjit RU, Leyngold IM, Margo CE. Melanoma-associated spongiform scleropathy in oculodermal melanocytosis with primary orbital melanoma. Ocul Oncol Pathol. 2016; 2 (4): 276–9. doi:10.1159/000447595

12. Dickens TA, Franchina M, Gajdatsy A, Mesbah Ardakani N. Primary orbital melanoma arising in an atypical diffuse (plaque-like) blue naevus/ melanocytosis: a case report and review of literature. BMC Ophthalmol. 2021; 21 (1): 425. doi: 10.1186/s12886-021-02176-y

13. Amiryan A.G., Borodin Yu.I., Saakyan S.V. Oculodermal melanocytosis and orbital melanoma. Russian ophthalmological journal. 2010; 2: 46–8 (In Russ.).

14. Cichorek M, Wachulska M, Stasiewicz A, Tyminska A. Skin melanocytes: biology and development. Postepy Dermatol Alergol. 2013; 30: 30–41. doi: 10.5114/pdia.2013.33376

15. Sharan S, Grigg JR, Billson FA. Bilateral naevus of Ota with choroidal melanoma and diffuse retinal pigmentation in a dark-skinned person. Br J Ophthalmol. 2005; 89: 1529–15. doi: 10.1136/bjo.2005.070839

16. Acar A, Palamar M, Bayraktaroglu S, Akal n T, Karaarslan I. Primary orbital melanoma associated with nevus of Ota. Int J Dermatol. 2022; 61 (4):e141-e143. doi: 10.1111/ijd.15873

17. Ahmed S, McDonald N, Lowder L, Mahoney B. Development of malignant melanoma of the orbit in previous radiation field. Clin Nucl Med. 2015; 40 (11): e522-e525. doi: 10.1097/RLU.0000000000000865

18. Ke Y, Ren X, Zhu L, et al. Primary orbital melanoma combined with giant divided nevus of the eyelid. J Craniofac Surg. 2014; 25 (1): e4-e7. doi:10.1097/SCS.0b013e3182a32e89

19. Alsuhaibani A.H., Alhumayed M. Primary orbital melanoma with poliosis and a palpable mass. Arch Ophthalmol. 2011; 129 (10): 1382–3. doi: 10.1001/archophthalmol.2011.302

20. Hui VWK, Lau TC, Ng LPW, Yuen HKL, Cheuk W. Primary orbital melanoma. Hong Kong Med J. 2021; 27(3): 223.e1-223.e2. doi: 10.12809/hkmj208788

21. Haskins CP, Nurkic S, Fredenburg KM, Dziegielewski PT, Mendenhall WM. Primary orbital melanoma treated with orbital exenteration and postoperative radiotherapy: A cas e report and review of the literature. Head Neck. 2018; 40 (3): E17-E20. doi: 10.1002/hed.24983

22. Smoker WRK, Gentry LR, Yee NK, Reede DL, Nerad JA. Vascular lesions of the orbit: More than meets the eye. RadioGraphics. 2008; 28 (1): 185–204. doi:10.1148/rg.281075040

23. Furdova A, Babal P. Giant orbital melanoma in a heroin abuser. Case Rep Ophthalmol. 2017; 8 (1): 288–93. doi: 10.1159/000475521

24. Liu X, Chen Y, Wang Y, et al. Cancer risk in patients with hepatitis C virus infection: a population-based study in Sweden. Cancer Medicine. 2017; 6 (5): 1135–40. doi: 10.1002/cam4.988

25. Rose AM, Luo R, Radia UK, et al. Detection of mutations in SF3B1, EIF1AX and GNAQ in primary orbital melanoma by candidate gene analysis. BMC Cancer. 2018; 18 (1): 1262. doi: 10.1186/s12885-018-5190-z

26. Mudhar HS, Doherty RE, Salvi SM, et al. Genetic profiling of primary orbital melanoma: An analysis of 6 cases with clinicopathologic correlation. Ophthalmology. 2019; 126 (7): 1045–52. doi: 10.1016/j.ophtha.2018.12.047