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RETINAL AND CHOROIDAL TUMORS

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Choroidal Hemangioma  
 

Choroidal hemangioma is a vascular hamartoma and can exist in two distinct forms: the diffuse and the circumscribed type, which...

Choroidal hemangioma is a vascular hamartoma and can exist in two distinct forms: the diffuse and the circumscribed type, which differ clinically and histopathologically.

The circumscribed choroidal hemangioma is seen as a well defined red-orange, slightly elevated dome-shaped lesion usually located at the posterior pole.

It usually exhibits slow enlargement in early life, and by young adulthood overlying atrophic changes in the RPE can develop, as can cystoid degeneration of the sensory retina, and sensory retinal detachment.

The diffuse choroidal hemangioma, which permeates throughout the entire choroid, is frequently associated with facial hemangioma (Sturge-Weber disease).

Choroidal Osteoma  
 

Choroidal osteoma is a benign tumor, unilateral or bilateral, acquired, and a slowly growing choroidal mass usually in the juxtapapillary...

Choroidal osteoma is a benign tumor, unilateral or bilateral, acquired, and a slowly growing choroidal mass usually in the juxtapapillary region.

This tumor is usually found in healthy young women between the second and the third decade of life.

The pathological finding of choroidal osteoma is a plaque of mature bone surrounding the optic disc in the full thickness of the choroid, usually sparing the RPE.

This benign tumor may cause visual loss due to subfoveal osteoma, serous retinal detachment, and choroidal neovascularization.

Uveal Melanoma  
 

The uveal melanoma is the intraocular primary malignant neoplasia most common in adults. These tumors are usually unilateral and detected...

The uveal melanoma is the intraocular primary malignant neoplasia most common in adults. These tumors are usually unilateral and detected at between 55 and 60 years of age.

Choroidal melanoma is a solid tumor, ranging from dark brown to golden in color. The earliest pathognomic sign is appearance of orange patches in the pigment epithelium due to accumulation of the lipofuscin. In about 20% of cases the tumor breaks through Bruch's membrane and the retinal pigment epithelium, growing into the retina in the form of a mushroom. It is therefore associated with rhegmatogenous retinal detachment and subretinal edema.

Initially patients are asymptomatic but may have blurred vision, visual field defects, flashes and floaters.

Most patients with melanoma of the choroid are submitted to radiotherapy as enucleation is reserved for advanced cases.

Ciliary body melanoma is another subtype of uveal melanoma. Patients may present with severe ocular pain secondary to high intraocular pressure because of acute angle-closure glaucoma. Growth of the melanoma into the lens may produce its subluxation, lenticular astigmatism, or cataract.

Iris melanomas form only a small proportion of all uveal melanomas and may be circumscribed or diffuse. Circumscribed melanomas have a nodular shape and are more common in the inferior iris. Diffuse melanomas cause acquired heterochromia and are associated with glaucoma.

Choroidal Metastasis  
 

Choroidal metastases usually develop late in the disease, and are more common in those with metastases to the lung or...

Choroidal metastases usually develop late in the disease, and are more common in those with metastases to the lung or brain. Breast cancer is the most common cancer to metastasize to the choroid.

Choroidal metastases are typically yellow in color, and are associated with subretinal fluid. They are usually anatomically located between the macula and equator.

Symptoms include blurred vision, flashes, floaters, and pain.

Ultrasound can be very useful in diagnosis. An A-scan will typically show moderate to high internal reflectivity. A B-scan will identify an echogenic choroidal mass and may show secondary retinal detachment.

Intraocular Lymphoma  
 

The spectrum of intraocular lymphoid tumors ranges from benign reactive lymphoid hyperplasia to various types of malignant lymphoma.

Intraocular lymphoma...

The spectrum of intraocular lymphoid tumors ranges from benign reactive lymphoid hyperplasia to various types of malignant lymphoma.

Intraocular lymphoma is commonly a non-Hodgkin’s large B cell lymphoma of the eye and the CNS.

The ocular findings include large clumps of cells in the vitreous, and the fundus examination is significant for multifocal, large, yellow, sub-retinal and sub-RPE infiltrative lesions.

Patients with intraocular lymphoma can be divided into several distinct subgroups, associated with: central nervous system non-Hodgkin’s lymphoma, systemic non-Hodgkin’s lymphoma, acquired immunodeficiency syndrome, and other rare forms of systemic lymphoma.

DIFFERENTIAL DIAGNOSIS of Intraocular-CNS Lymphoma:

Subretinal and Sub-RPE Infiltrates

  • Acute posterior multifocal placoid pigment epitheliopathy
  • Amelanotic choroidal melanoma
  • Bilateral diffuse uveal melanocytic proliferation
  • Birdshot retinochoroidopathy
  • Choroidal granuloma (e.g., sarcoidosis, tuberculosis)
  • Choroidal metastasis
  • Diffuse unilateral subacute neuroretinitis
  • Fundus flavimaculatus
  • Multifocal choroiditis
  • Multiple evanescent white dot syndrome
  • Pneumocystis choroiditis
  • Sympathetic ophthalmia
  • Vogt-Koyanagi-Harada syndrome

Retinal and Retinal Vascular Infiltrates

  • Fungal retinochoroiditis
  • Retinal periphlebitis (e.g., sarcoidosis, toxoplasmosis)
  • Retinal vascular occlusion
  • Retinal vasculitis (e.g., frosted branch angiitis, Behçet’s disease)
  • Toxoplasmosis
  • Viral retinitis (e.g., acute retinal necrosis, cytomegalovirus retinitis)

Vitreous

  • Amyloidosis
  • Intermediate or posterior uveitis
  • Old vitreous hemorrhage
  • Senile vitritis
  • Optic nerve
  • Infiltrative disease (e.g., leukemia, metastasis, granuloma)
  • Optic disc edema
Retinocytoma  
 

The retinocytoma is a rare, benign form of retinal tumor related to the retinoblastoma. Like retinoblastomas, retinocytomas arise from a...

The retinocytoma is a rare, benign form of retinal tumor related to the retinoblastoma. Like retinoblastomas, retinocytomas arise from a mutation in the RB1 gene.

The retinocytoma should be differentiated from the classically described regressed retinoblastoma that occurs in a phthisical eye, and which consists of ghost-like remnants of necrotic tumor cells that are embedded within a partially calcified matrix of basophilic material. They have a translucent “fish flesh” appearance, contain abundant calcification that has been likened to cottage cheese, and are surrounded by a ring of retinal pigment epithelium depigmentation.

This tumor can manifest as spontaneously regressed retinoblastoma, can follow treatment of retinoblastomas, or can be seen in untreated tumors.

Choroidal Nevus  
 

Choroidal nevus is a benign tumour, which presents itself as a flat, dark gray-brownish pigmented lesion, occurring more commonly in...

Choroidal nevus is a benign tumour, which presents itself as a flat, dark gray-brownish pigmented lesion, occurring more commonly in the post-equatorial region, often with overlying drusen and a yellowish ring around the base (halo nevus). It has a slightly well demarcated margin which size is mostly not greater than a disc diameter.

Multiple nevi may occur in patients with neurofibromatosis.

The nevus can grow during puberty, but in adults the growth should be watched.  The chance of a nevus converting into a melanoma is extremely low (1/20,000). Features such as subretinal fluid or overlying orange pigment imply an active mass and could be an early sign of a malignant transformation of the lesion.

Choroidal neovascularization membrane associated with exudation or hemorrhage may develop.

The features in the fluorescein angiogram will vary depending on the degree of pigmentation. When the nevi encroaches or replaces part of the choriocapillaris, the lesions may appear hypofluorescent.

Differential Diagnosis

  • Choroidal melanoma
  • Combined hamartoma
  • Congenital hypertrophy of the RPE
  • Hyperplasia of the RPE
  • Subretinal hemorrhage
Congenital Hypertrophy of the Retinal Pigment Epithelium  
 

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is an uncommon benign fundus lesion, which is usually asymptomatic and detected...

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is an uncommon benign fundus lesion, which is usually asymptomatic and detected on routine ophthalmic examination.

CHRPE is the result of diffuse proliferation of pigment epithelial cells and is a well-defined lesion which is flat or slightly elevated, round, solitary, dark brown-to-black in colour, and has scalloped edges. In older patients, it may have foci of depigmentation.

No treatment is required, but if the lesion increases in diameter or gives rise to an elevated nodule, this may represent an adenoma or adenocarcinoma.

CHRPE can present in the context of familial adenomatous polyposis (Gardner's syndrome), albeit, bilaterally with multifocal, smaller, ovoid lesions.

In fluorescein angiogram lesions have blackened hypofluorescence noted in all phases.

Differential Diagnosis

  • Adenoma or adenocarcinoma of retinal pigment epithelium
  • Combined hamartoma of retina
  • Massive gliosis of retina
  • Melanotic choroidal nevus or melanoma
  • Metastatic melanoma to retina
  • Reactive hyperplasia of retinal pigment epithelium
  • Syndrome of bilateral diffuse uveal melanocytic proliferation (BDUMP) associated with systemic carcinoma