07 Retinal Oncology (Nonvascular Tumors)

What is a choroidal nevus?

Acquired focal accumulation of melanocytes within the choroid

What is another name for choroidal nevus?

benign choroidal melanoma

What is the appearance of a choroidal nevus? (9)

-Flat or minimally elevated

-<1.5mm thick

-<6mm in diameter (<5DD)

-Distinct margins

-Stable or slow growing

-Melanotic or amelanotic

-Surface drusen (80%)

-RPE alterations possible

-Low internal reflectivity on A-scan

What are symptoms of a choroidal nevus? (2)

-Asymptomatic unless macular involvement

-May have a sensory retinal detachment

How does a choroidal nevus evolve?

-Increase in incidence into the mid 30's, then levels off

-Changes in size and pigmentation can occur at the onset of pregnancy (and may progress to malignancy)

-5-10% grow without conversion to malignancy

What are the complications of choroidal nevi?

conversion to malignant melanoma

What are differential diagnoses for choroidal nevi? (4)

-Malignant Choroidal Melanoma*

-Choroidal Hemangioma (would be pink-orange in color)

-Choroidal Metastases (cancer that has metastasized to the eye, can be brown in color)

-Choroidal Osteoma (yellow in color, a good differential for amelanotic choroidal nevi)

How are choroidal nevi managed? (3)

-Photodocumentation

-If suspicious for melanoma, refer to a retinal specialist or ultrasound biomicroscopy

-Routine evaluation (interval based on risk)

What is a choroidal osteoma?

an acquired, slow-growing, intrachoroidal, bone-like tumor

(a bone in the choroid)

What is the appearance of a choroidal osteoma? (5)

-Yellow-white to orange-red

-Variable size/thickness

-Slightly elevated

-Located on/near optic nerve

-Highly reflective on ultrasound with shadowing behind it*

What is the laterality of choroidal osteomas?

unilateral

What are symptoms of choroidal osteomas? (4)

-Asymptomatic

-Reduced VA

-Metamorphopsia

-Scotoma

How do choroidal osteomas evolve?

-Arises in late childhood or early adulthood

-May increase or decrease in size or remain stable

What are complications of choroidal osteomas? (3)

-Choroidal neovascular membrane (high risk)

-Chronic retinal detachment

-RPE atrophy

True or False: Men are more at risk for choroidal osteomas than women.

False

What are differential diagnoses for choroidal osteomas?

-Amelanotic Choroidal Melanoma

-Amelanotic Choroidal Nevus

-Disciform Scar

-Metastatic Carcinoma

How are choroidal osteomas managed? (3)

-Refer to retinal specialist (if neovascularization, subretinal fluid, or tumor growth)

-Laser photocoagulation (can lead to local resorption of bone or complete disappearance of the tumor)

-Anti-VEGF (for CNV)

What is a melanocytoma?

benign, primary tumor of the optic nerve that arises from melanocytes

What is the appearance of a melanocytoma?

-Elevated/flat gray-black lesion on optic disc

-Flayed edges

-May involve juxtapapillary choroid

-Small (<2mm)

What is the laterality of melanocytomas?

unilateral

What are symptoms of melanocytomas? (3)

-Asymptomatic

-VF defect (if RNFL thins adjacent to tumor)

-VA reduction (if exudation from tumor)

How do melanocytomas evolve?

-Slow growth over 5-20 years

-Low potential for malignant transformation

What are complications of melanocytomas? (6)

-Optic nerve edema

-Small retinal hemorrhages

-Retinal edema

-Retinal exudates

-Subretinal fluid

-Vitreous seeding

True or False: Females are more at risk for melanocytomas than males.

True

What are differential diagnoses for melanocytomas? (5)

-Juxtapapillary choroidal melanoma (will change over time, melanocytomas will not)

-Choroidal nevus (melanocytomas occur at the disc)

-CHRPE (similar color, but there are no RPE cells at disc)

-Adenoma of the retinal pigment epithelium (similar color, but there are no RPE cells at disc)

-Metastatic melanoma of the optic disc

How are melanocytomas managed?

-Monitor for evolution with fundus photos

-Consult with retinal specialist if unsure of diagnosis or abnormal growth

What is an astrocytic hamartoma?

benign, congenital abnormality of the astrocytes in the optic nerve and nerve fiber layer

What is the appearance of an astrocytic hamartoma? (6)

-White oval lesion with smooth surface

-Elevated

-Multinodular appearance ("mulberry" lesion)

-May appear anywhere on the retina, but tend to occur on the disc

-High reflectivity on B scan, even with low gain

-(+)Autofluorescence

What are symptoms of astrocytic hamartomas? (2)

-Decreased VA

-VF defect

How do astrocytic hamartomas evolve?

non-progressive

What are astrocytic hamartomas associated with? (2)

-Tuberous sclerosis

-Neurofibromatosis

What are differential diagnoses for astrocytic hamartomas? (4)

-Optic nerve drusen (both show autofluorescence and high reflectivity with a low gain)

-Retinoblastoma

-Amelanotic Nevus (would not be lobulated)

-Myelinated NFL (feathery white streak appearance and (-)autofluorescence)

How are astrocytic hamartomas managed? (3)

-Routine follow-up

-Fundus photo helpful (to ensure it's not changing over time)

-Neurologic evaluation to rule out systemic disease

What is another name for optic nerve gliomas?

juvenile pilocytic granuloma

What is an optic nerve glioma?

most common primary neoplasm of the optic nerve affecting the glial tissue

What is the appearance of an optic nerve glioma? (2)

-Typically in the retro-orbital portion of the nerve

-Occasionally, a forward extension may be seen on fundoscopy (smooth, elevated, whitish mass over the disc)

What are possible effects of optic nerve gliomas being in the retro-orbital portion of the nerve? (5)

-Proptosis

-Strabismus

-Limited EOMs

-Progressive ptosis

-Venous occlusive disease

What are symptoms of optic nerve gliomas? (2)

-Reduced VA

-Diplopia

Diagnosis of optic nerve glioma between age 4-8 is considered relatively ______.

benign

What are optic nerve gliomas associated with?

neurofibromatosis

True or False: Optic nerve glioma is more common in adults than children.

False

Diagnosis of optic nerve glioma in adulthood is more likely the ______ form.

malignant (glioblastoma)

What are complications of optic nerve gliomas? (2)

-Optic atrophy

-Blindness

How are optic nerve gliomas managed? (6)

-Orbital imaging

Neurosurgical referral for:

-Observation

-Surgery

-Radiation

-Chemotherapy

-Rule out other tumors

What are differential diagnoses for optic nerve gliomas? (3)

-Optic nerve meningioma (have different characteristics on neuroimaging)

-Rhabdomyosarcoma

-Orbital metastatic lesion

What is an optic nerve meningioma?

benign neoplasm of the optic nerve sheath, usually found in the retrobulbar optic nerve

What are the signs of optic nerve meningioma? (9)

-Proptosis

-Optic disc edema

-Visual field defects

-EOM restriction

-Strabismus

-Venous stasis retinopathy

-Optociliary shunt formation

-Optic atrophy

-APD

What are the symptoms of optic nerve meningiomas? (5)

-Decreased VA

-Field defect

-Decreased contrast sensitivity

-Decreased color vision

-Headache and vomiting

How do optic nerve meningiomas evolve?

slow, painless, progressive proptosis and visual loss (insidious presentation)

If age of onset is <20 years, the optic nerve meningioma presentation is _____ aggressive.

more

If age of onset is >30 years, the optic nerve meningioma presentation is _____ aggressive.

less

What are the complications of optic nerve meningiomas? (2)

-Erosion into the bony orbit

-Extension into the brain

What are risk factors for optic nerve meningioma? (3)

-Female > Male

-Exposure to ionizing radiation

-Neurofibromatosis Type 2

What are differential diagnoses for optic nerve meningioma? (3)

-Optic nerve glioma

-Orbital Lymphoma

-Rhabdomyosarcoma

How are optic nerve meningiomas managed? (3)

Consult to Neurosurgeon or Ocular Oncology for:

-Observation (if good visual acuity)

-Surgical excision

-Radiation therapy