Uveal melanoma overview: Difference between revisions
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==Overview== | ==Overview== | ||
Uveal melanoma is a cancer of the eye involving the [[iris]], [[ciliary body]], or [[choroid]]. Uveal melanoma arise from [[melanocytes]] in the [[uvea]]. | Uveal melanoma is a cancer of the eye involving the [[iris]], [[ciliary body]], or [[choroid]]. Uveal melanoma arise from [[melanocytes]] in the [[uvea]]. Uveal melanoma is a rare disease, but the most common primary intraocular malignancy. | ||
Iris melanomas are less common than choroidal melanomas. Uveal melanoma may be classified into several subtypes based on their location, based on ophthalmoscopic examination and based on the cell type. According to the Callender classification, there are four subtypes of uveal melanoma based on the cell type. [[Genes]] involved in the pathogenesis of uveal melanoma include ''GNAQ'', ''GNA11'', and ''BAP1''. Uveal melanoma is caused by a [[mutation]] in the [[DNA]]. The incidence of uveal melanoma in the United States is approximately 0.43 per 100,000 individuals. The majority of uveal melanoma cases are reported in Europe and United States. The incidence of uveal melanoma increases with age; the median age at diagnosis is 60 years. Males are more commonly affected with uveal melanoma than females. Uveal melanoma commonly affects individuals older than twenty years of age. Caucasians are more commonly affected with uveal melanoma than Africans. | Iris melanomas are less common than choroidal melanomas. Uveal melanoma may be classified into several subtypes based on their location, based on ophthalmoscopic examination and based on the cell type. According to the Callender classification, there are four subtypes of uveal melanoma based on the cell type. [[Genes]] involved in the pathogenesis of uveal melanoma include ''GNAQ'', ''GNA11'', and ''BAP1''. Uveal melanoma is caused by a [[mutation]] in the [[DNA]]. The incidence of uveal melanoma in the United States is approximately 0.43 per 100,000 individuals. The majority of uveal melanoma cases are reported in Europe and United States. The incidence of uveal melanoma increases with age; the median age at diagnosis is 60 years. Males are more commonly affected with uveal melanoma than females. Uveal melanoma commonly affects individuals older than twenty years of age. Caucasians are more commonly affected with uveal melanoma than Africans. Common risk factors in the development of uveal melanoma are [[cutaneous]] and [[iris]] [[nevi]], host pigmentation factors, ultraviolet light exposure, caucasian race, and certain inherited skin disorders such as [[dysplastic nevus syndrome]] and [[ocular]] melanocytosis. If left untreated, 50% of patients with uveal melanoma may progress to develop [[metastasis]]. Common complications of uveal melanoma include [[glaucoma]], [[vision loss]], and [[metastasis]]. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal [[tumors]] is 77 percent respectively. Symptoms of uveal melanoma include [[eye pain]], [[eye redness]], loss of peripheral vision, poor or blurry vision in one eye, a dark spot on the iris, flashing lights, floaters, and symptoms of glaucoma. | ||
The optimal therapy for uveal melanoma depends on the size of the tumor. The various treatment modalities for uveal melanoma include [[enucleation]], [[plaque ]] [[brachytherapy]], external beam [[proton therapy]], transupillary [[thermotherapy]], [[Gamma Knife]] [[stereotactic radiosurgery]], and resection of the tumor. | The optimal therapy for uveal melanoma depends on the size of the tumor. The various treatment modalities for uveal melanoma include [[enucleation]], [[plaque ]] [[brachytherapy]], external beam [[proton therapy]], transupillary [[thermotherapy]], [[Gamma Knife]] [[stereotactic radiosurgery]], and resection of the tumor. | ||
==Classification== | ==Classification== | ||
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Uveal melanoma is caused by a [[mutation]] in the [[DNA]]. | Uveal melanoma is caused by a [[mutation]] in the [[DNA]]. | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Uveal melanoma must be differentiated from pigmented [[nevi]], [[congenital]] [[hypertrophy]] of the [[retinal pigment epithelium]], [[optic disc ]][[melanocytoma]], [[hypertrophy]] of the [[retinal pigment epithelium]], [[hyperplasia]] of [[retinal pigment epithelium]], [[choroidal]] [[hemangioma]], [[choroidal]] [[metastasis]], [[choroidal]] [[osteoma]], peripheral [[exudative]] hemorrhagic chorioretinopathy, [[choroidal]] [[hemorrhage]], hemorrhagic detachment of [[retina]] and [[retinal pigment epithelium]], [[posterior]] [[nodular]] [[scleritis]], intraocular [[leiomyoma]], [[adenoma]] of [[retinal pigment epithelium]], [[retinoblastoma]], uveal [[metastasis]], choroidal detachment, [[choroidal]] [[hemangioma]], [[choroidal]] [[cyst]], uveal [[neurofibroma]], and uveal [[schwannoma]]. | Uveal melanoma must be differentiated from pigmented [[nevi]], [[congenital]] [[hypertrophy]] of the [[retinal pigment epithelium]], [[optic disc ]] [[melanocytoma]], [[hypertrophy]] of the [[retinal pigment epithelium]], [[hyperplasia]] of [[retinal pigment epithelium]], [[choroidal]] [[hemangioma]], [[choroidal]] [[metastasis]], [[choroidal]] [[osteoma]], peripheral [[exudative]] hemorrhagic chorioretinopathy, [[choroidal]] [[hemorrhage]], hemorrhagic detachment of [[retina]] and [[retinal pigment epithelium]], [[posterior]] [[nodular]] [[scleritis]], intraocular [[leiomyoma]], [[adenoma]] of [[retinal pigment epithelium]], [[retinoblastoma]], uveal [[metastasis]], choroidal detachment, [[choroidal]] [[hemangioma]], [[choroidal]] [[cyst]], uveal [[neurofibroma]], and uveal [[schwannoma]]. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The incidence of uveal melanoma in the United States is approximately 0.43 per 100,000 individuals. The majority of uveal melanoma cases are reported in Europe and United States. The incidence of uveal melanoma increases with age; the median age at diagnosis is 60 years. Males are more commonly affected with uveal melanoma than females. | The incidence of uveal melanoma in the United States is approximately 0.43 per 100,000 individuals. The majority of uveal melanoma cases are reported in Europe and United States. The incidence of uveal melanoma increases with age; the median age at diagnosis is 60 years. Males are more commonly affected with uveal melanoma than females. | ||
Uveal melanoma commonly affects individuals older than twenty years of age. Caucasians are more commonly affected with uveal melanoma than Africans. | Uveal melanoma commonly affects individuals older than twenty years of age. Caucasians are more commonly affected with uveal melanoma than Africans. | ||
==Risk Factors== | ==Risk Factors== | ||
Common risk factors in the development of uveal melanoma are [[cutaneous]] and [[iris]] [[nevi]], host pigmentation factors, ultraviolet light exposure, caucasian race, and certain inherited skin disorders such as [[dysplastic nevus syndrome]] and [[ocular]] melanocytosis. | Common risk factors in the development of uveal melanoma are [[cutaneous]] and [[iris]] [[nevi]], host pigmentation factors, ultraviolet light exposure, caucasian race, and certain inherited skin disorders such as [[dysplastic nevus syndrome]] and [[ocular]] melanocytosis. | ||
==Screening== | ==Screening== | ||
According to the United States Preventive Services Task Force, screening for uveal melanoma is not recommended. | According to the United States Preventive Services Task Force, screening for uveal melanoma is not recommended. | ||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
If left untreated, 50% of patients with uveal melanoma may progress to develop [[metastasis]]. Common complications of uveal melanoma include [[glaucoma]], [[vision loss]], and [[metastasis]]. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively. | If left untreated, 50% of patients with uveal melanoma may progress to develop [[metastasis]]. Common complications of uveal melanoma include [[glaucoma]], [[vision loss]], and [[metastasis]]. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively. | ||
==Staging== | ==Staging== | ||
According to the American Joint Committee on Cancer, there are four stages of uveal melanoma based on the [[tumor]] [[size]], spread to nearby [[lymph nodes]], and [[metastasis]]. Each stage is assigned a number 0 through 4 that indicate increasing severity and letters T, N, and M that designate the tumor size, spread to nearby lymph nodes, and metastasis respectively. | According to the American Joint Committee on Cancer, there are four stages of uveal melanoma based on the [[tumor]] [[size]], spread to nearby [[lymph nodes]], and [[metastasis]]. Each stage is assigned a number 0 through 4 that indicate increasing severity and letters T, N, and M that designate the tumor size, spread to nearby lymph nodes, and metastasis respectively. | ||
==History and Symptoms== | ==History and Symptoms== | ||
Symptoms of uveal melanoma include [[eye pain]], [[eye redness]], loss of [[peripheral vision]], poor or blurry vision in one eye, a dark spot on the iris, [[flashing lights]], floaters, and symptoms of glaucoma. | Symptoms of uveal melanoma include [[eye pain]], [[eye redness]], loss of [[peripheral vision]], poor or blurry vision in one eye, a dark spot on the iris, [[flashing lights]], floaters, and symptoms of glaucoma. | ||
==Physical Examination== | ==Physical Examination== | ||
Common physical examination findings of uveal melanoma include presence of dark spot on the iris, [[anisicoria]], loss of peripheral vision, decreased [[visual acuity]] and [[photopsia]]. | Common physical examination findings of uveal melanoma include presence of dark spot on the iris, [[anisicoria]], loss of peripheral vision, decreased [[visual acuity]] and [[photopsia]]. | ||
==Laboratory Tests== | ==Laboratory Tests== | ||
Some patients with uveal melanoma may have elevated concentration of complete and differential [[blood counts]] and elevated [[liver enzyme]] levels, which is usually suggestive of metastasis of uveal melanoma to the [[liver]]. | Some patients with uveal melanoma may have elevated concentration of complete and differential [[blood counts]] and elevated [[liver enzyme]] levels, which is usually suggestive of metastasis of uveal melanoma to the [[liver]]. | ||
==Chest X Ray== | ==Chest X Ray== | ||
Chest x-rays may be performed to detect metastases of uveal melanoma to the [[lungs]]. | Chest x-rays may be performed to detect metastases of uveal melanoma to the [[lungs]]. | ||
==CT Scan== | ==CT Scan== | ||
On [[head]] and [[neck]] [[CT scan]], uveal melanoma is characterized by elevated, hyperdense sharply marginated lenticular or mushroom shaped [[lesions]] that enhance with administration of contrast. | On [[head]] and [[neck]] [[CT scan]], uveal melanoma is characterized by elevated, hyperdense sharply marginated lenticular or mushroom shaped [[lesions]] that enhance with administration of contrast. | ||
==MRI scan== | ==MRI scan== | ||
Head and neck MRI scan is diagnostic of uveal melanoma. On [[head]] and [[neck]] [[MRI]], uveal melanoma is characterized by isointense to hypointense mass on T1-weighted MRI and hyperintense mass on T2-weighted MRI. | Head and neck MRI scan is diagnostic of uveal melanoma. On [[head]] and [[neck]] [[MRI]], uveal melanoma is characterized by isointense to hypointense mass on T1-weighted MRI and hyperintense mass on T2-weighted MRI. | ||
==Ultrasound== | ==Ultrasound== | ||
On ultrasound, uveal melanoma is characterized by internal vascularity, collar button shape, and solid low to medium echotexture. | On ultrasound, uveal melanoma is characterized by internal vascularity, collar button shape, and solid low to medium echotexture. | ||
==Other Imaging Studies== | ==Other Imaging Studies== | ||
Other imaging studies of uveal melanoma include PET scan, [[ultrasound biomicroscopy]], [[optical coherence tomography]], [[color fundus photography]], [[fluorescein angiography]], [[indocyanine green angiography]], [[transillumination]], and [[photography]]. | Other imaging studies of uveal melanoma include PET scan, [[ultrasound biomicroscopy]], [[optical coherence tomography]], [[color fundus photography]], [[fluorescein angiography]], [[indocyanine green angiography]], [[transillumination]], and [[photography]]. | ||
==Other Diagnostic Tests== | ==Other Diagnostic Tests== | ||
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==Medical therapy== | ==Medical therapy== | ||
The optimal therapy for uveal melanoma depends on the size of the tumor. The various treatment modalities for uveal melanoma include [[enucleation]], [[plaque brachytherapy]], external beam [[proton therapy]], [[transupillary]] [[thermotherapy]], [[Gamma Knife]] [[stereotactic radiosurgery]], and rection of the tumor. | The optimal therapy for uveal melanoma depends on the size of the tumor. The various treatment modalities for uveal melanoma include [[enucleation]], [[plaque brachytherapy]], external beam [[proton therapy]], [[transupillary]] [[thermotherapy]], [[Gamma Knife]] [[stereotactic radiosurgery]], and rection of the tumor. | ||
==Surgical therapy== | ==Surgical therapy== | ||
Surgery is the mainstay of treatment for uveal melanoma. | Surgery is the mainstay of treatment for uveal melanoma. | ||
==Primary Prevention== | ==Primary Prevention== | ||
There is no established method for prevention of uveal melanoma. | There is no established method for prevention of uveal melanoma. | ||
==Secondary Prevention== | ==Secondary Prevention== | ||
Secondary prevention strategies following uveal melanoma include dilated [[fundus]] examination, [[liver function tests]], abdominal [[MRI]] and abdominal [[ultrasound]]. | Secondary prevention strategies following uveal melanoma include dilated [[fundus]] examination, [[liver function tests]], abdominal [[MRI]] and abdominal [[ultrasound]]. | ||
== References == | == References == | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 00:00, 27 May 2019
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Uveal melanoma overview On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Uveal melanoma is a cancer of the eye involving the iris, ciliary body, or choroid. Uveal melanoma arise from melanocytes in the uvea. Uveal melanoma is a rare disease, but the most common primary intraocular malignancy. Iris melanomas are less common than choroidal melanomas. Uveal melanoma may be classified into several subtypes based on their location, based on ophthalmoscopic examination and based on the cell type. According to the Callender classification, there are four subtypes of uveal melanoma based on the cell type. Genes involved in the pathogenesis of uveal melanoma include GNAQ, GNA11, and BAP1. Uveal melanoma is caused by a mutation in the DNA. The incidence of uveal melanoma in the United States is approximately 0.43 per 100,000 individuals. The majority of uveal melanoma cases are reported in Europe and United States. The incidence of uveal melanoma increases with age; the median age at diagnosis is 60 years. Males are more commonly affected with uveal melanoma than females. Uveal melanoma commonly affects individuals older than twenty years of age. Caucasians are more commonly affected with uveal melanoma than Africans. Common risk factors in the development of uveal melanoma are cutaneous and iris nevi, host pigmentation factors, ultraviolet light exposure, caucasian race, and certain inherited skin disorders such as dysplastic nevus syndrome and ocular melanocytosis. If left untreated, 50% of patients with uveal melanoma may progress to develop metastasis. Common complications of uveal melanoma include glaucoma, vision loss, and metastasis. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively. Symptoms of uveal melanoma include eye pain, eye redness, loss of peripheral vision, poor or blurry vision in one eye, a dark spot on the iris, flashing lights, floaters, and symptoms of glaucoma. The optimal therapy for uveal melanoma depends on the size of the tumor. The various treatment modalities for uveal melanoma include enucleation, plaque brachytherapy, external beam proton therapy, transupillary thermotherapy, Gamma Knife stereotactic radiosurgery, and resection of the tumor.
Classification
Uveal melanoma may be classified into several subtypes based on their location, based on ophthalmoscopic examination and based on the cell type. According to the Callender classification, there are four subtypes of uveal melanoma based on the cell type.
Pathophysiology
Genes involved in the pathogenesis of uveal melanoma include GNAQ, GNA11, and BAP1.
Causes
Uveal melanoma is caused by a mutation in the DNA.
Differential Diagnosis
Uveal melanoma must be differentiated from pigmented nevi, congenital hypertrophy of the retinal pigment epithelium, optic disc melanocytoma, hypertrophy of the retinal pigment epithelium, hyperplasia of retinal pigment epithelium, choroidal hemangioma, choroidal metastasis, choroidal osteoma, peripheral exudative hemorrhagic chorioretinopathy, choroidal hemorrhage, hemorrhagic detachment of retina and retinal pigment epithelium, posterior nodular scleritis, intraocular leiomyoma, adenoma of retinal pigment epithelium, retinoblastoma, uveal metastasis, choroidal detachment, choroidal hemangioma, choroidal cyst, uveal neurofibroma, and uveal schwannoma.
Epidemiology and Demographics
The incidence of uveal melanoma in the United States is approximately 0.43 per 100,000 individuals. The majority of uveal melanoma cases are reported in Europe and United States. The incidence of uveal melanoma increases with age; the median age at diagnosis is 60 years. Males are more commonly affected with uveal melanoma than females. Uveal melanoma commonly affects individuals older than twenty years of age. Caucasians are more commonly affected with uveal melanoma than Africans.
Risk Factors
Common risk factors in the development of uveal melanoma are cutaneous and iris nevi, host pigmentation factors, ultraviolet light exposure, caucasian race, and certain inherited skin disorders such as dysplastic nevus syndrome and ocular melanocytosis.
Screening
According to the United States Preventive Services Task Force, screening for uveal melanoma is not recommended.
Natural History, Complications and Prognosis
If left untreated, 50% of patients with uveal melanoma may progress to develop metastasis. Common complications of uveal melanoma include glaucoma, vision loss, and metastasis. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively.
Staging
According to the American Joint Committee on Cancer, there are four stages of uveal melanoma based on the tumor size, spread to nearby lymph nodes, and metastasis. Each stage is assigned a number 0 through 4 that indicate increasing severity and letters T, N, and M that designate the tumor size, spread to nearby lymph nodes, and metastasis respectively.
History and Symptoms
Symptoms of uveal melanoma include eye pain, eye redness, loss of peripheral vision, poor or blurry vision in one eye, a dark spot on the iris, flashing lights, floaters, and symptoms of glaucoma.
Physical Examination
Common physical examination findings of uveal melanoma include presence of dark spot on the iris, anisicoria, loss of peripheral vision, decreased visual acuity and photopsia.
Laboratory Tests
Some patients with uveal melanoma may have elevated concentration of complete and differential blood counts and elevated liver enzyme levels, which is usually suggestive of metastasis of uveal melanoma to the liver.
Chest X Ray
Chest x-rays may be performed to detect metastases of uveal melanoma to the lungs.
CT Scan
On head and neck CT scan, uveal melanoma is characterized by elevated, hyperdense sharply marginated lenticular or mushroom shaped lesions that enhance with administration of contrast.
MRI scan
Head and neck MRI scan is diagnostic of uveal melanoma. On head and neck MRI, uveal melanoma is characterized by isointense to hypointense mass on T1-weighted MRI and hyperintense mass on T2-weighted MRI.
Ultrasound
On ultrasound, uveal melanoma is characterized by internal vascularity, collar button shape, and solid low to medium echotexture.
Other Imaging Studies
Other imaging studies of uveal melanoma include PET scan, ultrasound biomicroscopy, optical coherence tomography, color fundus photography, fluorescein angiography, indocyanine green angiography, transillumination, and photography.
Other Diagnostic Tests
Other diagnostic studies for uveal melanoma include biopsy.
Medical therapy
The optimal therapy for uveal melanoma depends on the size of the tumor. The various treatment modalities for uveal melanoma include enucleation, plaque brachytherapy, external beam proton therapy, transupillary thermotherapy, Gamma Knife stereotactic radiosurgery, and rection of the tumor.
Surgical therapy
Surgery is the mainstay of treatment for uveal melanoma.
Primary Prevention
There is no established method for prevention of uveal melanoma.
Secondary Prevention
Secondary prevention strategies following uveal melanoma include dilated fundus examination, liver function tests, abdominal MRI and abdominal ultrasound.