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Malignant Choroidal Melanoma

Malignant choroidal melanoma (MCM) is a rare diagnosis, although it is the most commonly reported intraocular malignancy. Furthermore, the choroid is the second most common site that melanomas occur.


Presentation

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.

Weight Loss
  • Non-visual features of MCM entail systemic signs of malignancy, which include fatigue and weight loss. MCM may occur as a metastatic lesion. Malignant choroidal melanomas are diagnosed via ophthalmologic examination.[symptoma.com]
  • Therefore, there may be a history of weight loss, fatigue, cough or change in bowel or bladder habits suggestive of systemic involvement. Signs Loss of visual acuity visual field defects.[patient.info]
  • loss and appearing of ascites.[academic.oup.com]
  • loss, general fatigue, malaise or illness.[reviewofoptometry.com]
Pseudotumor
  • […] the non-contrast diagnostically undifferentiable pseudotumors and the choroidal metastasis, revealed no significant change in density after the administration of contrast material.[ncbi.nlm.nih.gov]
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]
Plethora
  • […] associated with choroidal melanomas include vitreous hemorrhages or pigmented vitreous cells, drusen on the surface of the tumor, choroidal neovascular membranes, or even proptosis if the tumor invades the orbit. 4 Differential Diagnoses There are a plethora[reviewofoptometry.com]
Hepatosplenomegaly
  • Among the tests included in the metastatic workup in a patient with choroidal melanoma are: Abdominal examination looking for hepatosplenomegaly. Laboratory: Liver function tests. X ray: Chest X ray.[eyewiki.aao.org]
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]
Anterior Uveitis
  • Patients with malignant choroidal melanomas present with a wide variety of complaints, and optometrists need to be diligent in properly assessing the cause of seemingly common conditions, such as anterior uveitis, as well as performing routine dilated[ncbi.nlm.nih.gov]
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]
Corneal Edema
  • There were also diffuse corneal edema and iridocorneal adhesion. Anterior chamber was flat and the tumoral mass was evident in papillary space which filled the whole eye. No other fundus details could be detected.[ijo.in]
Mental Distress
  • Additional follow-up treatment does not seem to induce mental distress.[ncbi.nlm.nih.gov]

Workup

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.
Gliosis
  • […] differentials for amelonotic or melanotic choroidal melanomas include choroidal detachment, lymphoma, metastatic carcinoma, subretinal or sub-RPE hematoma, localized suprachoroidal hematoma, nodular posterior scleritis, reactive hyperplasia of RPE or massive gliosis[reviewofoptometry.com]

Treatment

  • PURPOSE: To evaluate the efficacy of transpupillary thermotherapy (TTT) as the only method of treatment for small choroidal melanoma.[ncbi.nlm.nih.gov]
  • Several modes of treatment are available for choroidal melanomas.[emedicine.medscape.com]
  • Treatments Small Choroidal Melanoma After a complete evaluation, treatment may be recommended.[westcoastretina.com]

Prognosis

  • 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]
  • 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 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]
  • Spindle cell melanomas have the best prognosis, epithelioid cell melanomas have the least favorable prognosis, and mixed-cell melanomas have an intermediate prognosis (9). Tumors made entirely of spindle A cells are nevi.[webeye.ophth.uiowa.edu]

Etiology

  • 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]

Epidemiology

  • 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]
  • 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]
  • 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]
  • *SEER Surveillance, Epidemiology, and End Results[cancer.org]
  • Diagnosis: Choroidal melanoma Epidemiology Malignant neoplasm arising from melanocytes in the uveal tract, accounting for 3% of all melanomas Most common primary intraocular malignancy in adults Overall incidence in U.S. is 4.3 per million/year (1) Average[webeye.ophth.uiowa.edu]
Sex distribution
Age distribution

Pathophysiology

  • 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

  • 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]
  • 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]
  • 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]
  • Treatment Once a tumor is diagnosed as melanoma, treatment is needed to prevent further growth of the tumor, or spread of the tumor from the eye to other sites.[ohsu.edu]
  • Survival Rates Brain Mets Not treated with Whole Brain Radiation Treatment (WBRT) or treated with corticosteroids alone: 1-2 months Median survival is increased to 3-6 months with WBRT & corticosteroids WBRT prevents any further deterioration of neurologic[slideshare.net]

References

Article

  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.

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Last updated: 2019-06-28 09:51