06 Retinal Oncology (Vascular Tumors)

What is a benign tumor?

mature cells that are natural to the adjacent tissue with low risk for invasion or metastasis

What is a malignant tumor?

cells that are not normally located where they are found and are likely to invade adjacent tissue to metastasize to other areas of the body

What is a retinal capillary hemangioma?

benign proliferation of retinal capillary pericytes and endothelial cells

congenital defect of the entire vascular unit (arteriole, capillary bed, venule)

(classified as a harmatoma)

What is a harmatoma?

tissue that is normally at the site, but is malformed or misarranged

What are the 2 types of retinal capillary hemangioma?

-Peripheral tumor

-Juxtapapillary tumor

What is the appearance of a peripheral retinal capillary hemangioma?

elevated spherical orange-red tumor with large feeder vessels

What is the appearance of a juxtapapillary retinal capillary hemangioma?

red/pink intraretinal lesions close to the optic nerve (or within the posterior pole) without large feeder vessels

In retinal capillary hemangioma, the capillary bed has a faulty endothelium. What can this lead to?

leaky plasma or blood, which can lead to intraretinal fluid (edema) or subretinal fluid (serous retinal detachment)

What are symptoms of retinal capillary hemangioma?

-Asymptomatic (if no leakage to macula)

-Reduced VA

How can a retinal capillary hemangioma evolve? (4)

-Early subtle lesion can enlarge over time

-May become exudative

-May become cicatrizing (scarring) lesion with vitreoretinal traction

-End stage may have retinal detachment, iris/retinal neovascularization, neovascular glaucoma

What systemic condition is retinal capillary hemangioma associated with?

Von-Hippel-Lindau (VHL) disease

What are complications of retinal capillary hemangioma? (4)

-Macular edema

-Exudates

-Serous/exudative retinal detachment

-Vitreal hemorrhage

What are differential diagnoses for retinal capillary hemangioma? (5)

-Coats disease (has a lot of exudate in retina)

-Retinoblastoma (maybe if tumor is close to disc)

-Racemose Hemangioma (different presentation, but similar name)

-Intraretinal macroaneurysm (isolated exudative lesion, may look similar to juxtapapillary tumor)

-Retinal Cavernous Hemangioma (different presentation, but similar name)

How are small retinal capillary hemangiomas without exudation/hemorrhage near the optic nerve managed?

monitor

How are peripheral retinal capillary hemangiomas managed? (5)

To prevent enlargement of lesion:

-Photocoagulation

-Cryotherapy

-Photodynamic therapy (PDT)

-Anti-VEGF

-Plaque radiotherapy

How are retinal capillary hemangiomas with exudative retinal detachment managed?

vitrectomy

How are retinal capillary hemangioma with intractable pain managed?

enucleation

What is a cavernous hemangioma of the retina?

vascular tumor of the retina composed of numerous venous aneurysms

What is the appearance of cavernous hemangiomas of the retina? (5)

-Grape-like clusters of dilated vascular sacs

-Varying size and location

-No feeder vessels

-Associated with glial membrane (may lead to growth of epiretinal membrane)

-Exudation/hemorrhage are rare

Cavernous hemangiomas of the retina are located in the _____ retinal layers.

inner

What is the laterality of cavernous hemangiomas of the retina?

usually unilateral

What are symptoms of cavernous hemangiomas of the retina? (2)

-Usually asymptomatic and good visual acuity (unless macula is involved)

-Scotoma at site of lesion

Cavernous hemangiomas of the retina are an ______ condition and are usually identified around age ___.

inherited, 23

How can cavernous hemangiomas of the retina evolve? (2)

-Vascular tumor remains stable in size

-Glial proliferation surrounding tumor may increase in size

What are complications of cavernous hemangiomas of the retina? (2)

-Vitreous hemorrhage (rare)

-Cutaneous/intracranial vascular lesions

What are differential diagnoses for cavernous hemangiomas of the retina? (3)

-Retinal capillary hemangioma (different presentation, but similar name)

-Racemose hemangioma (different presentation, but similar name)

-Coats disease (similar white appearance due to glial cells, but cavernous hemangiomas rarely have exudate)

How are cavernous hemangiomas of the retina usually managed?

usually no treatment (since they don't leak)

How are cavernous hemangiomas of the retina with vitreous hemorrhage managed?

laser or cryotherapy

How are cavernous hemangiomas of the retina with glial tissue involving the macula and greatly reduced acuity managed?

surgical intervention

How are cavernous hemangiomas of the retina with signs/symptoms of neuro-oculocutaneous involvement managed?

refer for CT or MRI

What is a racemose hemangioma?

arteriovenous malformation (AVM)

What is the appearance of racemose hemangiomas? (4)

-Large, dilated, tortuous retinal artery

-Passes from the optic disc for some distance into the fundus

-Communicates directly with a dilated retinal vein and then back to the optic disc

-Usually no exudation and no hemorrhage

What are symptoms of racemose hemangiomas?

None

(typically in the periphery and don't involve the macula)

How do racemose hemangiomas evolve?

Genetic or developmental factors that occur early in gestation lead to dysgenesis of the embryologic vascular plexus.

Usually remains stable.

What systemic condition is racemose hemangioma associated with?

Wyburn Mason Syndrome

What are differential diagnoses for racemose hemangiomas? (3)

-Congenital tortuosity

-Capillary hemangioma (has a capillary bed tumor)

-Macrovessel

How are racemose hemangiomas managed? (2)

-Observation

-Consult to internist to rule out Wyburn Mason Syndrome

What is a choroidal hemangioma?

benign vascular tumor of the choroid

Where are choroidal hemangiomas located on the fundus?

posterior pole

What is the appearance of choroidal hemangiomas? (4)

-Red-orange in color

-Indistinct margins

-May have overlying RPE changes or orange pigment

-Associated intraretinal or subretinal fluid

What is the appearance of choroidal hemangiomas on IVFA?

early, irregular hyperfluoresence of the large choroidal vessels with late staining of the outer retina

How can we differentiate between choroidal hemangioma and choroidal melanoma?

Choroidal hemangiomas will have high internal reflectivity on A-scan echography.

Choroidal melanomas will have low internal reflectivity on A-scan echography.

What is the appearance of choroidal hemangiomas on A-scan echography?

high internal reflectivity

What are symptoms of choroidal hemangiomas?

-None (if macula is uninvolved and there is no RD)

-Reduced vision and metamorphopsia (if macula is involved with lesion or associated RD)

How do choroidal hemangiomas evolve? (3)

-Present at birth but may remain undetected for many years

-Subretinal fluid and degenerative retinal changes may occur later

-Non-rhegmatogenous (serous/exudative) retinal detachment is common

What systemic condition is choroidal hemangioma associated with?

Sturge-Weber Syndrome

What are differentials for choroidal hemangiomas? (7)

-Malignant Choroidal Melanoma (low internal reflectivity with A-scan)

-Amelanotic Choroidal Melanoma (low internal reflectivity with A-scan)

-Choroidal Metastatic Carcinoma

-Choroidal Osteoma

-Capillary Hemangioma (different appearance, but similar name)

-Disciform lesion

-Posterior Scleritis

How are choroidal hemangiomas managed? (2)

-No intervention necessary unless leakage creates serous retinal detachment that threatens macular function

-Consult internist to rule out Sturge-Weber Syndrome

How are choroidal hemangiomas with leakage that creates serous retinal detachment threatening macular function managed? (5)

-Photodynamic therapy (treatment of choice)

-Laser photocoagulation

-Transpupillary thermotherapy

-Radiation

-Anti-VEGF

What is a cavernous hemangioma of the orbit?

congenital, benign, slowly progressive vascular neoplasm of endothelial-lined spaces surrounded by a fibrous capsule that occurs in the orbit

What is the appearance of cavernous hemangiomas of the orbit? (9)

-Proptosis

-Optic disc edema

-VF defects

-EOM restriction

-Strabismus

-Venous stasis retinopathy

-Optociliary shunt formation

-Choroidal folds

-APD

What are symptoms of cavernous hemangiomas of the orbit? (3)

-Proptosis

-Decreased VA

-Diplopia

How do cavernous hemangiomas of the orbit evolve?

-The classic findings is a women, in the 2nd to 6th decade of life

-Presents with slowly progressive, painless proptosis

-Gradual onset of symptoms over a period of 6 months to 2 years

What are cavernous hemangiomas of the orbit associated with?

Rarely, they may present simultaneously with a cavernous hemangioma of the brain

What are differential diagnoses for cavernous hemangiomas of the orbit? (4)

-Lymphangioma

-Rhabdomyosarcoma

-Metastatic Mass

-Varices

How are cavernous hemangiomas of the orbit that are small and asymptomatic managed?

periodic observation

How are cavernous hemangiomas of the orbit that are larger and causing diplopia or visual disturbances managed?

surgical excision is the treatment of choice