Ocular melanoma

From Clinical Guidelines Wiki


There are two primary types of ocular melanoma, uveal melanoma (arising in the iris, choroid and ciliary body) and conjunctival melanoma. Both types are uncommon.[1][2]

Treatment for ocular melanoma

For uveal melanoma, eye-conserving plaque radiotherapy is the most common treatment and results in similar rates of local control to surgery for most tumours.[1] Other forms of treatment include periodic observation, transpupillary laser thermotherapy (TTT), photodynamic therapy (PDT), charged particle irradiation, local tumour resection, enucleation and rarely exenteration.[1][3] Despite effective local treatment, the survival rate of uveal melanoma has not changed over a 25-year period.[1] This may well reflect an inability to prevent or treat metastatic disease. Uveal melanoma has a unique biomolecular signature which is quite distinct from that of cutaneous melanoma. While there have been significant improvements in molecular prognostic testing to sub-classify patients, to date, this has not translated into improvements in patient survival.[1][4]

Similarly for conjunctival melanoma, there has been a move to using eye-conserving treatment.[2][5] Local resection is well established and commonly used. Topical chemotherapy, cryotherapy and radiotherapy have a definite role as adjunctive treatments[2][5] Conjunctival melanoma has a biomolecular signature which is more similar to that of cutaneous melanoma (compared to uveal melanoma) and patients with advanced disease have had similarly good outcomes with targeted systemic treatment and immunotherapies.[6]

Periocular melanoma includes eyelid and orbital melanoma; both are rare conditions.

The management of ocular melanoma is complex and should be conducted in specialised units where eye-conserving therapies and dedicated pathology services are available for ocular melanoma.


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Evidence summary and recommendations

Evidence summary Level References
Eye-conserving therapies for ocular melanoma result in similar rates of local control to enucleation. IV [1]
The first surgery is most important. Inappropriate primary surgery results in upstaging of disease and a worse prognosis due to inadvertent tumour seeding IV [2], [5]
Evidence-based recommendationQuestion mark transparent.png Grade
Ocular melanoma is a complex and uncommon form of melanoma that should be managed in specialised units where multidisciplinary ocular cancer services are available.
C


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References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 Dogrusöz M, Jager MJ, Damato B. Uveal Melanoma Treatment and Prognostication. Asia Pac J Ophthalmol (Phila) 2017 Mar;6(2):186-196 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/28399342.
  2. 2.0 2.1 2.2 2.3 Shields CL, Chien JL, Surakiatchanukul T, Sioufi K, Lally SE, Shields JA. Conjunctival Tumors: Review of Clinical Features, Risks, Biomarkers, and Outcomes--The 2017 J. Donald M. Gass Lecture. Asia Pac J Ophthalmol (Phila) 2017 Mar;6(2):109-120 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/28399347.
  3. Rundle P. Treatment of posterior uveal melanoma with multi-dose photodynamic therapy. Br J Ophthalmol 2014 Apr;98(4):494-7 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/24463441.
  4. Robertson AG, Shih J, Yau C, Gibb EA, Oba J, Mungall KL, et al. Integrative Analysis Identifies Four Molecular and Clinical Subsets in Uveal Melanoma. Cancer Cell 2017 Aug 14;32(2):204-220.e15 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/28810145.
  5. 5.0 5.1 5.2 Damato B, Coupland SE. An audit of conjunctival melanoma treatment in Liverpool. Eye (Lond) 2009 Apr;23(4):801-9 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/18535601.
  6. Molecular Characteristics of Conjunctival Melanoma Using Whole-Exome Sequencing. JAMA Ophthalmol 2017 Dec 1;135(12):1434-1437 Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/29121185.

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