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Iris Melanoma

Choroidal Melanoma

Iris melanoma is a rare tumor in clinical practice and its development is primarily associated with increased exposure to ultraviolet radiation. The clinical presentation, apart from unilateral heterochromia, may be asymptomatic, but the unilateral development of glaucomas, as well as cataracts, have been described in the literature. The diagnosis rests on a detailed ophthalmological workup.


Presentation

Iris melanoma, despite being a very rare occurrence, is the most common malignant disease of the iris [1] [2]. Although the exact pathogenesis remains unknown, ultraviolet radiation is by far the most important risk factor which may explain (at least in part) why countries like New Zealand (due to the profoundly higher exposure to UV-B light) report higher incidence rates of this tumor [1] [3]. Solid evidence, however, is yet to be discovered. Conflicting reports exist when it comes to the age of presentation - some authors claim that iris melanoma is most frequently encountered in the first few decades of life [3], whereas others have documented a mean age of approximately 50 years [4]. Manifestations of iris melanoma are primarily of visual nature [2] [3]. Most patients present with a pigmented (yellow, brown, or tan-colored) spot on the iris that is often noticed incidentally, and such findings are typical for circumscribed iris melanoma, the most common form [2] [3]. Unilateral heterochromia of the eye is the characteristic symptom of diffuse iris melanoma [2] [3] [4] [5] [6]. In up to 30% of patients, additional symptoms and signs are absent, while some of the principal complaints seen in advanced stages of the disease include blurred vision, glaucoma, corectopia, ectropion, hyphema, and anterior chamber inflammation [3] [4] [6].

Turkish
  • History Play ENTRENus Play ENTRENuk Play ENTRENau Meanings of "iris melanoma" in Turkish English Dictionary : 1 result(s) Category English Turkish Medical 1 Medical iris melanoma iris melanomu Pronunciation of iris melanoma Term Options[tureng.com]
Aspiration
  • This case demonstrates the usefulness of fine needle aspiration biopsy for both diagnosis and optimum therapy in selected cases.[ncbi.nlm.nih.gov]
Hypertension
  • A 35-year-old male with unilateral ocular hypertension in the right eye (RE). Anterior segment examination of the RE showed a melanotic lesion. Intraocular pressure (IOP) was 40mmHg in RE.[ncbi.nlm.nih.gov]
  • There were no enucleations caused by ocular hypertension. Conclusions Baerveldt shunts were effective in lowering IOP, with few complications, in eyes treated with total anterior segment irradiation for AUM.[bjo.bmj.com]
  • Aggressive ocular hypertension was observed in the several eyes prior to shunt implantation. Two eyes were enucleated for non-glaucoma related sequelae.[iovs.arvojournals.org]
  • Anterior Chamber Brain and Optical Pathway Choroid Congenital Syndromes, Malformations and Abnomalities Conjunctiva Cornea Glaucomas, Ocular Hypertension Hypertensive Retinopathy Iris and Ciliary Body Albinism (see: Congenital Syndromes) Anterior Uveitis[atlasophthalmology.net]
Papule
  • These will look yellow-brown on slit-lamp examination and look like dome-shaped papules. Cogan Reese syndrome will have an irregular corneal endothelium and pedunculated or diffuse pigmented nodules.[journals.lww.com]
Heterochromia Iridis
  • Heterochromia iridis was present in all 25 cases (100%), corectopia in 23 (92%), and ectropion iridis in 21 (84%).[ncbi.nlm.nih.gov]
  • Of 126 patients, 14 (11.1%) had iris heterochromia (ie, heterochromia iridis) ( Table 4 ). The tumors' surface contours were noted to be either smooth in 51 of 130 eyes (39.2%) or irregular in 79 of 130 eyes (60.8%).[jamanetwork.com]
Confusion
  • We have seen other such patients who have melanoma growing out of their filtering sites (unpublished data). (3) Probably because of the diagnostic confusion with treatment delay there is significantly higher tumour related mortality in patients with iris[ncbi.nlm.nih.gov]

Workup

The asymptomatic nature of an iris melanoma may significantly affect the ability of the physician to make the diagnosis, and up to 30 months might pass before the tumor is detected [4]. Having in mind the known risk of metastatic disease (ranging from 5%-13% according to various reports) [4] [7], the importance of a properly obtained patient history and a detailed physical examination must be emphasized. If a new lesion on the iris (or a change in eye color) is reported, physicians must include iris melanoma in the differential diagnosis. Because of the fact that this tumor is most often diagnosed while still being very small (2.7mm in average) [8], a thorough inspection of both eyes is mandatory in order to recognize the lesion. More advanced methods are necessary, however, and techniques such as slit-lamp examination, ultrasound biomicroscopy, fluorescein angiography, and optical coherence tomography (OCT) are recommended in the workup [2] [3] [9]. Histology and immunohistochemistry have also been described in the literature as valuable tools for differentiation of malignant diseases of the iris [2] [4]. A viable sample for testing can be obtained through fine needle aspiration biopsy (FNAB), incision biopsy, or a direct approach through the cornea, which might be favored due to the potential risk of seeding malignant cells during the former two procedures [2] [9] [10].

Treatment

  • Most patients will need treatment for glaucoma and some may require treatment for tear-film instability and/or stem cell failure.[ncbi.nlm.nih.gov]

Prognosis

  • This contrasts with choroidal melanoma, where the presence of epithelioid cells is the strongest pathological marker for a poor prognosis.[ncbi.nlm.nih.gov]
  • Prognosis Among all uveal tumors, iris melanomas have the best prognosis, with the mortality being 2-3% in cases with metastasis.[news-medical.net]

Etiology

  • Etiology Despite a large amount of epidemiological and molecular studies, the etiology of uveal melanoma remains largely obscure.[atlasgeneticsoncology.org]
  • In this case an etiological link between latanoprost and malignancy is unlikely given the brief interval between exposure and clinical melanoma; however, benign melanosis is noted in the pigment epithelial layer of the iris ( Figure 2 ).[ncbi.nlm.nih.gov]
  • The etiology is unknown. They can arise from pre-existing benign melanomas or nevi of the iris stroma. They are most often congenital and have been observed for many years.[ijo.in]
  • True iris melanomas, originating from within the iris as opposed to originating elsewhere and invading the iris, are distinct in their etiology and prognosis, such that the other tumors are often referred to collectively as posterior uveal melanomas.[en.wikipedia.org]

Epidemiology

  • The clinical, epidemiological and pathological characteristics of iris melanomas are summarized and compared to those of other uveal melanomas.[ncbi.nlm.nih.gov]
  • Keywords: clinical (human) or epidemiologic studies: treatment/prevention assessment/controlled clinical trials • clinical (human) or epidemiologic studies: outcomes/complications • melanoma 2011, The Association for Research in Vision and Ophthalmology[iovs.arvojournals.org]
  • Objective To collaborate with multiple centers to identify representative epidemiological, clinical, and pathologic characteristics of melanoma of the iris.[jamanetwork.com]
  • Etiology Despite a large amount of epidemiological and molecular studies, the etiology of uveal melanoma remains largely obscure.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Pathophysiology

  • The exact pathophysiology of raised intraocular pressure remains unclear, but may involve invasion of the iridocorneal angle by tumour cells and by the tumour as well as postirradiation phenomena.[nature.com]
  • Pathophysiology Melanoma in the ciliary body poses a serious threat to life. It usually remains hidden behind the iris diaphragm, growing undetected for longer periods of time than melanoma in the iris or choroid.[emedicine.medscape.com]

Prevention

  • Treatment of slow-growing iris melanoma must be considered to prevent complications.[ncbi.nlm.nih.gov]
  • The goal of treatment in ocular melanoma is to prevent it from spreading (metastasis).[healthline.com]
  • Severe lesions can also prevent the iris muscles from functioning properly, thereby making it difficult for the cat to constrict the pupil in response to bright light.[eyespecialistsforanimals.com]
  • With medial tumours, chloramphenicol ointment was administered instead of drops, to prevent dellen formation.[nature.com]
  • Keywords: clinical (human) or epidemiologic studies: treatment/prevention assessment/controlled clinical trials • clinical (human) or epidemiologic studies: outcomes/complications • melanoma 2011, The Association for Research in Vision and Ophthalmology[iovs.arvojournals.org]

References

Article

  1. Michalova K, Clemett R, Dempster A, Evans J, Allardyce RA. Iris melanomas: are they more frequent in New Zealand? Br J Ophthalmol. 2001;85(1):4-5.
  2. Henderson E, Margo CE. Iris melanoma. Arch Pathol Lab Med. 2008;132(2):268-272.
  3. Jovanovic P, Mihajlovic M, Djordjevic-Jocic J, Vlajkovic S, Cekic S, Stefanovic V. Ocular melanoma: an overview of the current status. Int J Clin Exp Pathol. 2013;6(7):1230-1244.
  4. Demirci H, Shields CL, Shields JA, Eagle RC Jr, Honavar SG. Diffuse iris melanoma: a report of 25 cases. Ophthalmology. 2002;109(8):1553-1560.
  5. Conway R, Chua W, Qureshi C, Billson F. Primary iris melanoma: diagnostic features and outcome of conservative surgical treatment. The British Journal of Ophthalmology. 2001;85(7):848-854.
  6. Skalicky SE, Giblin M, Conway RM. Diffuse iris melanoma: Report of a case with review of the literature. Clin Ophthalmol. 2007;1(3):339-342.
  7. Shields CL, Shields JA, Materin M, Gershenbaum E, Singh AD, Smith A. Iris melanoma: risk factors for metastasis in 169 consecutive patients. Ophthalmology. 2001;108(1):172-178.
  8. Shields CL, Furuta M, Thangappan A, et al. Metastasis of uveal melanoma millimeter-by-millimeter in 8033 consecutive eyes. Arch Ophthalmol. 2009;127:989–998.
  9. Conway RM, Chew T, Golchet P, Desai K, Lin S, O’Brien J. Ultrasound biomicroscopy: role in diagnosis and management in 130 consecutive patients evaluated for anterior segment tumours. Br J Ophthalmol. 2005;89(8):950-955.
  10. Shields CL, Manquez ME, Ehya H, Mashayekhi A, Danzig CJ, Shields JA. Fine-needle aspiration biopsy of iris tumors in 100 consecutive cases: technique and complications. Ophthalmology. 2006;113(11):2080-2086.

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Last updated: 2019-07-11 22:07