Malignant choroidal melanoma (MCM) is a rare, sporadic cancer of the eye that arises from the choroid [1]. It is even more unusual in children [2]. It is found mostly in individuals of European descent and has male predominance [3]. Those with blue or green iris seem to be more frequently affected. MCM is usually diagnosed in middle age, on average within the fifth decade of life [2].

MCM typically has a delayed onset of symptoms, more so if the tumor is anteriorly located. As a result, it is often discovered incidentally.

When signs do arise, they consist of visual disturbances such as floaters, decreased visual acuity, photopsia, scotoma, and visual field defects. Photoreceptors receive their blood supply from the blood vessels in the choroid. Due to the growth of MCM, there is disruption of the vasculature, leading to reduced perfusion and thus ischemia of the retinal cells [4]. A further cause of visual loss is the retinal detachment. Moreover, other possible ocular findings include the abnormal formation of blood vessels in the iris (rubeosis), inflammation, and in a minority of cases, ocular pain. The latter is likely due to nerve impingement, tissue necrosis or glaucoma (increased intraocular pressure), caused by the melanoma.

Non-visual features of MCM entail systemic signs of malignancy, which include fatigue and weight loss. MCM may occur as a metastatic lesion.

• Falling

  Int Ophthalmol Clin 37 (4): 87-100, 1997 Fall. [PUBMED Abstract] Eye and ocular adnexa. In: Rosai J: Ackerman’s Surgical Pathology. 8th ed. St. Louis, Mo: Mosby, 1996, pp 2449-2508. Uveal melanoma. [cancer.gov]

• Pleural Effusion

  Light Malignant pleural effusions Pleural diseases, 2. a, pp. 97-114 [3.] J.T.T. Chen, N.S. Dahmash, C.E. Ravin, D.K. Heaston, C.E. Putman, H.F. [archbronconeumol.org]

• Nausea

  Common side effects of certain immunotherapy and targeted therapy melanoma treatments include, but are not limited to: Diarrhea Vitiligo (loss of pigment) Skin rash Lymphedema Thyroid issues Colitis Fatigue Nausea Itching Fever Constipation Joint pain [melanoma.org]

  Typically, the prominent feature of omental metastasis is swelling or bloating of the abdomen, some other symptoms include dull pain of the abdomen; indigestion that is persistent; nausea or gas; discomfort in the pelvic region; unexplained appetite loss [academic.oup.com]

• Hypertension

  Occasionally, the tumour may spread anteriorly, thus affecting segments at the front of the eye and so resulting in cataract formation, ocular hypertension/hypotension or iris rubeosis. [patient.info]

  CASE REPORT A 65-year-old Caucasian woman with hypertension was referred in 2003 for evaluation of an asymptomatic choroidal mass in the right eye (OD). Visual acuity was 20/25 OD and 20/20 in the left eye (OS). [retinatoday.com]

  The patient was also being treated for ocular hypertension by her primary ophthalmologist, who commenced Teoptic 2% b.i.d. OU in March 1994 because of an intraocular pressure of 26 mmHg in each eye. [nature.com]

  Past Ocular History: Myopia and presbyopia Past Medical History: Hypertension, no known primary malignancies Ocular Examination: Best corrected visual acuity: 20/20 OD, 20/20 -2 left eye (OS) Applanation tonometry: 15 mmHg OD, 15 mmHg OS Confrontation [webeye.ophth.uiowa.edu]

  The patient’s systemic history was significant for hypertension, heart problems (having had two valve replacements) and type 2 diabetes that was controlled with oral medications. [reviewofoptometry.com]

• Hypotension

  Occasionally, the tumour may spread anteriorly, thus affecting segments at the front of the eye and so resulting in cataract formation, ocular hypertension/hypotension or iris rubeosis. [patient.info]

  Exoresection Exoresection (eye wall resection, trans-scleral choroidectomy) involves the surgical resection of the tumor intact, through a large scleral trapdoor, without damaging the adjacent retina, and using hypotensive anesthesia to reduce hemorrhage [retinalphysician.com]

  There is a substantial risk of retinal detachment, intraocular bleeding, and complications associated with the anesthesia-induced hypotension used to decrease the risk of bleeding. [cancer.gov]

• Scotoma

  When signs do arise, they consist of visual disturbances such as floaters, decreased visual acuity, photopsia, scotoma, and visual field defects. Photoreceptors receive their blood supply from the blood vessels in the choroid. [symptoma.com]

  Melanoma of the eye may be discovered during a routine examination of the fundus or via the occurrence of recent visual problems such as lowering of visual acuity, a spot in the visual field (scotoma), flashes of light, or visual field loss (retinal detachment [institut-curie.org]

  The main symptoms are decreased visual acuity, visual field defects (scotomas), flashes and floaters. The presence of symptoms is more commonly found in melanomas than in choroidal nevi. [eyewiki.aao.org]

  A paracentral scotoma. Painless and progressive visual field loss. Floaters or flashes ('ball of light' slowly moving across vision). Occasionally severe ocular pain. [patient.info]

  Photocoagulation may be attempted for small tumors (< 3mm thickness, < 7mm basal diameter). 4,5 Like photocoagulation for any other reason, a permanent scotoma will result in the photocoagulated areas. [reviewofoptometry.com]

• Visual Impairment

  Fifty-four patients took part in both surveys, the number of patients with visual impairment increased from 21 preoperatively to 34 postoperatively. [ncbi.nlm.nih.gov]

  Observation is reserved for asymptomatic patient with a widespread metastatic disease, and radiotherapy is the reserved treatment when there is visual impairment ( 23 ). Enucleation is the traditional method of treating choroidal melanomas. [academic.oup.com]

• Strabismus

  J Pediatr Ophthalmol Strabismus 1991; 28:38-40. Shields JA, Shields CL, Donoso LA. Management of posterior uveal melanoma - Major review. Surv Ophthalmol 1991;36:161-5. Byrne SF, Green RL. In: Ultrassound of eye and orbit. [siicsalud.com]

• Macula

  Thirty-three static and serial CT examinations made on 29 patients with choroidal melanoma, three with pseudotumors of the macula and one with choroidal metastasis revealed the choroidal melanoma to be usually a hyperdense, markedly perfused tumor, while [ncbi.nlm.nih.gov]

  The right eye had a visual acuity of 1/60, moderate cataract, and a surgical choroidal coloboma involving optic disc and macula, with no evidence of local tumour recurrence. [nature.com]

  With plaque radiotherapy, eye salvage is achieved, and, particularly for cases in which the tumor is located away from the optic disc or macula, useful vision can be retained after treatment. [austinpublishinggroup.com]

  Sometimes the tumor develops from preexisting nevi. [2] Clinically, the lesions near the macula present earlier, as compared to lesions in the periphery, which present later after the development of glaucoma. [ijri.org]

  Fundus Exam OS: Figure 3: (click image for higher resolution) There are a few small hard drusen in the macula. The fundus is otherwise unremarkable. [webeye.ophth.uiowa.edu]

• Hyperpigmentation

  In addition to the sclera, uveal hyperpigmentation is usually present. Hyperpigmentation can also affect the ocular orbit and meninges. Ocular melanocytosis increases the risk of choroidal melanoma, which is 1:400 in these patients. [200.98.68.239]

Malignant choroidal melanomas are diagnosed via ophthalmologic examination. The medical history normally does not contain information that would suggest MCM. Despite this, diagnostic accuracy with regards to MCM has improved [5] [6]. It may be of some importance to note a history of any other malignancies, as these could be signs of metastatic disease or primary tumors that are sometimes associated with MCM [7].

The method used to visualize the intraocular structures and diagnose MCM is indirect ophthalmoscopy (IO). IO may reveal a flat or raised, typically pigmented lesion, which can be multilobular, dome, button or mushroom-shaped [8]. The lesion often has margins that are well demarcated. It may be associated with retinal detachment, prominent episcleral blood vessels, and orange pigmentation. It is possible to have diffuse MCMs, which pose a diagnostic challenge as they are minimally elevated, and are more likely to cause retinal detachment [9]. Other features that may accompany MCM are vitreous hemorrhage and pigmented cells in the vitreous humor, as well as abnormalities that appear within the retina and choroid, namely drusen and choroidal neovascular membranes respectively. Large tumors may extend into the orbit, causing proptosis.

Imaging techniques have a major role in the diagnosis and evaluation of the extent of the tumor. These modalities include:

• Ultrasound: Both A and B scans are used. A scan ultrasound may show a blood vessel formation within the melanoma [4].
• Fundus fluorescein angiography.
• Fine needle aspiration cytology: This is only performed if other diagnostic measures have failed, or as a therapeutic procedure, as it is invasive and potentially harmful.

Imaging and laboratory tests that are carried out to detect the presence of metastasis include:

• Abdominal computerized tomography (CT).
• Chest X-ray.
• Magnetic resonance imaging (MRI) of the abdomen.
• Liver function tests.

• Pleural Effusion

  Light Malignant pleural effusions Pleural diseases, 2. a, pp. 97-114 [3.] J.T.T. Chen, N.S. Dahmash, C.E. Ravin, D.K. Heaston, C.E. Putman, H.F. [archbronconeumol.org]

PURPOSE: To evaluate the efficacy of transpupillary thermotherapy (TTT) as the only method of treatment for small choroidal melanoma. [ncbi.nlm.nih.gov]

Treatments Small Choroidal Melanoma After a complete evaluation, treatment may be recommended. [westcoastretina.com]

Several modes of treatment are available for choroidal melanomas. [emedicine.medscape.com]

However metastasis may appear even decades after treatment, so patients must be followed indefinitely after treatment to monitor for metastasis. [ohsu.edu]

Computer analysis of a series of risk factors and their effect on prognosis showed that there was no worsening of mortality as compared to the series as a whole and further that the post enucleation peak of mortality was the same in this small group as [ncbi.nlm.nih.gov]

Prognosis Average patient survival: 8-9 months after ocular diagnosis Primary tumor type determines prognosis Metastatic malignant melanoma: worst prognosis 1-2 months Metastatic breast carcinoma: prognosis of 12 months 22. [slideshare.net]

We have found that patients with disomy of chromosome 3 (less aggressive cell type) have a better life prognosis and those with monosomy of chromosome 3 (more aggressive cell type) have a more guarded prognosis and may require additional chemotherapy. [willseye.org]

Prognosis [ 18 ] The prognosis depends mostly on the genetic alterations and tumour size. Every millimetre increase in thickness leads to a 5% increased risk for metastasis. [ 1 ] Median survival after liver metastasis is poor (4-6 months). [patient.info]

Sections and Some Topics Include: • Basic Principles o Cancer etiology; cancer genetics, principles of laser therapy, and more • Eyelid Tumors o Benign epidermal tumors; melanoma of the eyelid, systemic associations, and more • Conjunctival and Corneal [books.google.de]

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]

*SEER = Surveillance, Epidemiology, and End Results [cancer.org]

Epidemiology Conjunctival melanomas account for less than 5-10% of all ocular melanomas. They usual present in adulthood [22]. There is no sex preponderance. [mmmp.org]

Epidemiology For American women lifetime risk breast cancer- one in eight (12.5%) & lifetime risk death from breast cancer is 3.4% Breast Cancer is the most common malignancy to metastasize to the uvea Mean age at ocular diagnosis is 58 years, most cases [slideshare.net]

The most common site of metastasis is the liver (95%). [ 2, 3 ] Less frequently, choroidal melanoma can metastasise locally into the orbit, the conjunctiva or the maxillofacial bones. [ 4 ] Epidemiology 98% of cases occur in Caucasians. [ 1 ] It is rarely [patient.info]

Epidemiologic aspects of uveal melanoma. Surv Ophthalmol 1988;32:239-51. 2. Bell DJ, Wilson MW. Choroidal melanoma: Natural history and management options. Cancer Control 2004;11:296-303. 3. Mittica N, Vemuganti GK, Duffy M, Torczynski E, Edward DP. [ijo.in]

Comprehensively updated to reflect everything you need to know regarding retinal diagnosis, treatment, development, structure, function, and pathophysiology, this monumental ophthalmology reference work equips you with expert answers to virtually any [books.google.de]

[…] however, this lesion is the wrong color) Choroidal metastasis (however, generally amelanotic unless from melanoma) Additional Investigations CT chest, abdomen, pelvis done for evaluation of metastatic disease and staging was negative Choroidal melanoma Pathophysiology [eyewiki.org]

[…] produce similar results to proton-beam radiotherapy. 28 Radiation-induced Complications After radiotherapy, many patients experience visual loss, with some also developing a painful eye requiring enucleation. 29-31 It is important to understand the pathophysiology [retinalphysician.com]

Prevention Because the causes of choroidal melanoma are not well understood, there is no known way to prevent it. Treatment Management and treatment of choroidal melanoma depends on the tumor's size. [drugs.com]

In these patients, the main benefits of ocular treatment are conservation of the eye and vision, if possible, and the prevention of pain and cosmetic loss. [retinalphysician.com]

The goal of treatment in ocular melanoma is to prevent it from spreading (metastasis). [healthline.com]

Sunglasses to prevent choroidal melanoma? There is a higher incidence of choroidal melanoma in people with blue eyes and people that work outdoors. [mastereyeassociates.com]

Limiting excessive ocular sunlight exposure through sunglasses or other means may have a theoretical preventive effect in patients with a predisposition to intraocular melanoma. [emedicine.medscape.com]

1. Scotto J, Fraumeni JF Jr, Lee JA. Melanomas of the eye and other noncutaneous sites: Epidemiologic aspects. J Natl Cancer Inst. 1976;56(3):489-491.
2. Shields CL, Shields JA, Milte J, De Potter P, Sabbagh R, Menduke H. Uveal Melanoma in teenagers and children: A report of 40 cases. Ophthalmology. 1991;98(11):1662-1666.
3. Mahoney MC, Burnett WS, Majerovics A, Tanenbaum H. The epidemiology of ophthalmic malignancies in New York State. Ophthalmology. 1990;97(9):1143-1147.
4. Singh P, Singh A. Choroidal melanoma. Oman J Ophthalmol. 2012;5(1):3-9.
5. Shields JA, Augsburger JJ, Brown GC, Stephens RF. The differential diagnosis of posterior uveal melanoma. Ophthalmology. 1980;87(6):518-522.
6. Char DH, Stone RD, Irvine AR, et al. Diagnosticmodalities in choroidal melanoma. Am J Ophthalmol. 1980;89(2):223-230.
7. Lishko AM, Seddon JM, Gragoudas ES, Egan KM, Glynn RJ. Evaluation of prior primary malignancy as a determinant of uveal melanoma. Ophthalmology. 1989;96(12):1716-1721.
8. Dhupper M, Biswas J, Gopal L, Kumar SK, Khetan V. Clinicopathological correlation of choroidal melanoma in Indian population: A study of 113 cases. Oman J Ophthalmol. 2012;5(1):42-45.
9. Shields CL, Kaliki S, Furuta M, Shields JA. Diffuse versus nondiffuse small (≤ 3 MM thickness) choroidal melanoma: comparative analysis in 1,751 cases. The 2012 F. Phinizy Calhoun lecture. Retina. 2013;33(9):1763-1776.