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Questions and Answers
What is the typical characterization of the RPE cells in Congenital Hypertrophy of the RPE?
What is the typical characterization of the RPE cells in Congenital Hypertrophy of the RPE?
Which of the following tumors of the uvea is considered benign?
Which of the following tumors of the uvea is considered benign?
What is a common feature of an iris nevus?
What is a common feature of an iris nevus?
What is the management strategy for a Choroidal Nevus?
What is the management strategy for a Choroidal Nevus?
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What is a characteristic appearance of a Choroidal Nevus?
What is a characteristic appearance of a Choroidal Nevus?
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Which of the following statements about Choroidal Nevus is incorrect?
Which of the following statements about Choroidal Nevus is incorrect?
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What color is a Choroidal Nevus typically observed to be?
What color is a Choroidal Nevus typically observed to be?
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Which type of cells are typically found in an iris nevus?
Which type of cells are typically found in an iris nevus?
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What is the typical risk of malignant transformation associated with a pigmented choroidal nevus?
What is the typical risk of malignant transformation associated with a pigmented choroidal nevus?
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Which of the following is a common characteristic of iris/choroidal melanocytoma?
Which of the following is a common characteristic of iris/choroidal melanocytoma?
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Which cell type is most commonly associated with uveal melanoma?
Which cell type is most commonly associated with uveal melanoma?
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What is the primary management recommendation for a pigmented choroidal nevus?
What is the primary management recommendation for a pigmented choroidal nevus?
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In what location does an iris/choroidal melanocytoma present a higher risk of malignant transformation?
In what location does an iris/choroidal melanocytoma present a higher risk of malignant transformation?
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What is the most common primary malignant intraocular tumor in adults?
What is the most common primary malignant intraocular tumor in adults?
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Which characteristic does NOT serve as a prognostic factor in uveal melanoma?
Which characteristic does NOT serve as a prognostic factor in uveal melanoma?
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Which statement is true regarding the management of iris/choroidal melanocytoma?
Which statement is true regarding the management of iris/choroidal melanocytoma?
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Which type of melanoma is identified as carrying the best prognosis?
Which type of melanoma is identified as carrying the best prognosis?
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What is a significant risk factor for the transformation of iris nevus to melanoma?
What is a significant risk factor for the transformation of iris nevus to melanoma?
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What ancillary test is mentioned for evaluating iris melanoma?
What ancillary test is mentioned for evaluating iris melanoma?
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Which of the following statements is NOT true regarding iris melanoma?
Which of the following statements is NOT true regarding iris melanoma?
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What is the approximate rate of metastasis for iris melanoma within 5 years?
What is the approximate rate of metastasis for iris melanoma within 5 years?
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What characteristic does ectropion uveae refer to in relation to iris nevi?
What characteristic does ectropion uveae refer to in relation to iris nevi?
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What management options are available for iris melanoma?
What management options are available for iris melanoma?
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Which feature is characteristic of ciliary body melanoma?
Which feature is characteristic of ciliary body melanoma?
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What is the 5-year survival rate for ciliary body melanoma?
What is the 5-year survival rate for ciliary body melanoma?
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Which of the following is a common risk factor for choroidal melanoma?
Which of the following is a common risk factor for choroidal melanoma?
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What is the most common age range for the diagnosis of choroidal melanoma?
What is the most common age range for the diagnosis of choroidal melanoma?
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What management option is most appropriate for localized ciliary body melanoma?
What management option is most appropriate for localized ciliary body melanoma?
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Which of the following symptoms is NOT commonly associated with choroidal melanoma?
Which of the following symptoms is NOT commonly associated with choroidal melanoma?
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What are the cell types involved in the pathogenesis of choroidal melanoma?
What are the cell types involved in the pathogenesis of choroidal melanoma?
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What is the incidence rate of new cases of choroidal melanoma in the U.S.?
What is the incidence rate of new cases of choroidal melanoma in the U.S.?
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Which management option is typically utilized as a last resort for ciliary body melanoma?
Which management option is typically utilized as a last resort for ciliary body melanoma?
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Which of the following is a typical feature of choroidal melanoma?
Which of the following is a typical feature of choroidal melanoma?
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What complication is commonly associated with choroidal melanoma?
What complication is commonly associated with choroidal melanoma?
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Which ancillary test is primarily used for assessing the structure of choroidal melanoma?
Which ancillary test is primarily used for assessing the structure of choroidal melanoma?
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In ultrasonography of choroidal melanoma, what characteristic feature is typically observed?
In ultrasonography of choroidal melanoma, what characteristic feature is typically observed?
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Which of the following represents a possible metastatic site for choroidal melanoma?
Which of the following represents a possible metastatic site for choroidal melanoma?
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What feature on fluorescein angiography might indicate the presence of an elevated mass?
What feature on fluorescein angiography might indicate the presence of an elevated mass?
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Which of the following best describes the shape of a choroidal melanoma that has broken through Bruch’s membrane?
Which of the following best describes the shape of a choroidal melanoma that has broken through Bruch’s membrane?
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What is a common finding in the dome of a choroidal melanoma mass?
What is a common finding in the dome of a choroidal melanoma mass?
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Study Notes
Congenital Hypertrophy of the RPE
- Characterized by taller, more pigmented retinal pigment epithelium (RPE) cells.
- Features large, compact spherical melanosomes.
- Management typically involves observation.
Uveal Tumors Overview
- Types include uveal nevus, uveal melanocytoma, uveal melanoma, choroidal hemangioma, and choroidal osteoma.
- All tumors in this category are benign except uveal melanoma.
Iris Nevus
- Clinical features: localized or diffuse lesions, variably pigmented, relatively flat, slow growth possible.
- Composed of low-grade spindle cells and considered benign.
- Management involves observation.
Choroidal Nevus
- Clinical features: flat or minimally elevated, slate gray or green (can be amelanotic), oval/round with distinct borders, located at the posterior pole or mid-peripheral fundus.
- Found in 5-20% of the population; asymptomatic and typically an incidental finding.
- Pathology includes low-grade spindle cells; 0.01-0.02% risk of malignant transformation.
- Management includes consideration of risk factors and periodic observation every 3-12 months.
Iris/Choroidal Melanocytoma
- Tumor composed of deeply pigmented round cells, generally benign and typically requires no treatment.
- Issues include potential local growth and risk of malignant transformation, especially if located near the optic nerve head.
Uveal Melanoma
- Most common primary malignant intraocular tumor in adults, with 90% originating in the choroid.
- Prognostic factors include cell type (spindle, epithelioid), tumor size, anterior margin location, ciliary body involvement, and extraocular extension.
- Spindle cell melanoma has the best prognosis; epithelioid has the worst.
Iris Melanoma
- Predominantly occurs in Caucasians with light-colored irides.
- Characterized by spindle cells; can present as small discrete lesions or as diffuse/infiltrative growth.
- Associated symptoms include heterochromia, chronic uveitis, hyphema, and secondary glaucoma with a metastasis rate of about 3% within 5 years.
Transformation of Iris Nevus to Melanoma
- Approximate transformation rates: ~5% within 5 years, ~8% within 15 years.
- Risk factors include age ≤ 40 years at presentation, spontaneous hyphema, inferior quadrant location, diffuse flat tumor, ectropion uveae, and ill-defined margins.
Management of Iris Melanoma
- Includes observation, local resection, plaque radiation therapy, and enucleation.
Ciliary Body Melanoma
- Characterized by a pigmented mass, often difficult to detect at an early stage due to its occult location.
- May require interventions such as plaque radiation, local tumor resection, or enucleation.
- Prognosis is worse compared to iris melanoma, with a 5-year survival rate around 70%.
Choroidal Melanoma
- Epidemiology shows an incidence of 5-6 cases per 1,000,000, with a diagnosis mean age of 55-60 years.
- Risk factors include Caucasian race, light-colored irides, positive family history, pre-existing nevi, and chromosomal abnormalities.
Symptoms and Clinical Features of Choroidal Melanoma
- Typically asymptomatic unless involving optic nerve or macula, leading to blurry vision, scotoma, and vision loss.
- Clinically presents as an elevated dome-shaped mass, possibly with exudative retinal detachment.
Complications of Choroidal Melanoma
- Include choroidal neovascularization, retinal detachment, vitreous hemorrhage, uveitis, secondary glaucoma, and metastasis (commonly liver, lungs, skin).
Ancillary Testing for Choroidal Melanoma
- Techniques include indirect ophthalmoscopy, ultrasonography, fluorescein/ICG angiography, CT, MRI, fine needle aspiration biopsy, and liver function tests.
Ultrasonography Characteristics
- Choroidal melanoma shows low to medium reflectivity, acoustic hollowness, and can reveal extrascleral extension.
Fluorescein Angiography
- Used for closer examination of choroidal tumors and their effects on retinal structures.
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Description
This quiz covers the pathology and management of Congenital Hypertrophy of the Retinal Pigment Epithelium (RPE). It focuses on the characteristics of RPE cells and treatment approaches such as observation. Ideal for medical students and professionals in ophthalmology.
