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]

• Severe Pain

  Key features are elevated eye pressure, forward movement of diaphragm combined with severe pain ( Yang et al, 2003 ). Posterior nodular scleritis is rare, but often underdiagnosed. [intechopen.com]

• Photopsia

  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]

  Clinical Features Uveal Mets Appearance: 99% choroidal mets from breast cancer were yellow in color 77% plateau-shaped 65% a/w subretinal fluid Exudation and hemorrhage rare Symptoms: - 75% Blurred vision - 6% Floaters - 5% Photopsias - 7% Asymptomatic [slideshare.net]

  This had been happening for about three weeks; he denied photopsia. 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]

  […] size of a nevus, have been identified. 4,5 These factors assist in the assessment of the potential for nevus transformation into melanoma, and they include thickness of 2 mm or more, presence of subretinal fluid, symptoms such as metamorphopsia and photopsia [retinatoday.com]

  Case Report A 52-year-old male with glaucoma presented with photopsia and black shadow of the right eye for 1 year. His vision was 20/20. [e-tjo.org]

• Eye Pain

  When symptoms occur, they include having blurry vision seeing spots seeing flashing lights having severe eye pain. Having these symptoms doesn't mean you have choroidal melanoma. [drugs.com]

  When present, symptoms can include: changes in your iris flashes of light vision changes, such as blurred vision or loss of certain parts of your vision eye pain Melanoma of the eye can develop when pigment cells in your eye grow out of control. [healthline.com]

  Signs and symptoms of glaucoma may include eye pain and redness, as well as blurry vision. Vision loss. [mayoclinic.org]

  CASE REPORT A 36-year-old Chinese male presented to the ocular clinic because of decreased vision and severe eye pain in 2005. He had no remarkable medical and ocular history. Pupils were equal, round and reactive to light. [academic.oup.com]

  In a single-center, phase I–II study of 57 evaluable patients treated with carbon ion-beam irradiation and followed for a median of 26 months, 26 patients developed neovascular glaucoma or severe eye pain from increased intraocular pressure, and 3 patients [cancer.gov]

• Lacrimation

  […] gland and duct C69.50 Malignant neoplasm of unspecified lacrimal gland and duct C69.51 Malignant neoplasm of right lacrimal gland and duct C69.52 Malignant neoplasm of left lacrimal gland and duct Reimbursement claims with a date of service on or after [icd10data.com]

  […] and Retinal Pigment Epithelium o Coats Disease; lymphoma of the retina and CNS; and more • Retinoblastoma o Geographic and environmental factors; local therapy; teletherapy, future directions • Orbital Tumors o Tumors of the optic nerve; tumors of the lacrimal [books.google.de]

  Spread can occur to adjacent eyelid skin, naso-lacrimal duct, orbit, eye, nasal sinuses and intracranial cavity. Metastatic spread can occur to regional lymph nodes and systemically. [mmmp.org]

  Collateral damage to the canaliculi, conjunctiva, and lacrimal gland can occur. Irradiation of the superior lid margin causes keratinization and pain on blinking. [retinalphysician.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]

• Night Blindness

  Fast and slow EOG oscillations in congenital and acquired night blindness. Ophthalmic Res 1979; 11: 206–11. Google Scholar 11. Aschoff U. Skotopische und photopische Anteile des Hellanstiegs im Elektrookulogramm (EOG). [link.springer.com]

• 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]

• Skin Rash

  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]

• Suggestibility

  Their presence in the vessel lumina suggested removal by the circulation. The resulting influence on the production of antibodies is also mentioned. [ncbi.nlm.nih.gov]

  The medical history normally does not contain information that would suggest MCM. Despite this, diagnostic accuracy with regards to MCM has improved. [symptoma.com]

  […] and metastasize late, as suggested by a patient whose tumor suddenly grew dramatically into a mushroom shape after years of indolent behavior. [retinalphysician.com]

  This type of cancer seems to affect men and women equally, although some studies have suggested men are more commonly affected. Ocular melanoma is more common in Caucasians. [healthline.com]

• Irritability

  Occasionally, there may be prolonged redness, irritation, or infection inside the eye. The patient may see double if the muscles are damaged during the operation to apply or remove the plaque. [pages.jh.edu]

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.

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.