Eye cancer · Conditions

Intraocular Tumors: Benign Eye Tumors and Lesions That Look Like Melanoma

An intraocular tumor is any growth inside the eye. Many are benign — freckle-like nevi, flat pigment spots called CHRPE, bony osteomas or small blood-vessel tumors — and several can look like melanoma, which is why specialists call them pseudomelanomas. A few benign lesions point to inherited conditions such as von Hippel-Lindau disease, tuberous sclerosis or familial adenomatous polyposis. Multimodal imaging usually tells them apart, so harmless lesions are simply watched and only those that threaten sight or health are treated.
Pseudomelanomas
benign lesions that can mimic melanoma
CHRPE
seen in about 1.2% of people examined
Von Hippel-Lindau disease
retinal hemangioblastomas in about 70%
Tuberous sclerosis
multiple retinal hamartomas in about a third
Main tests
photos, ultrasound, OCT, angiography
Usual management
observation; treatment if sight is threatened

An intraocular tumor is any abnormal growth inside the eye — in the retina, the choroid beneath it, the ciliary body, the iris or the optic disc. The words sound alarming, but many of these growths are benign, and some are not true tumors at all but spots present from birth that simply look unusual. The specialist's main task is to separate the few lesions that need treatment from those that need only photographs and checks.

This page covers benign tumors, melanoma lookalikes and the inherited conditions some of them signal. It is part of our eye cancer guide.

What is an intraocular tumor?

Each part of the eye has its own typical tumors. The retina can develop small blood-vessel tumors, benign growths of its supporting (glial) cells and, in young children, retinoblastoma. The pigment layer beneath the retina produces flat dark spots such as CHRPE. The choroid hosts nevi, hemangiomas, osteomas, melanoma and most metastases; the iris has its own nevi, cysts and melanomas.

Cross-section of the eye and its surroundings, showing where the main eye tumors arise. 1 2 3 4 5 Eyelid Conjunctiva Cornea Iris Lens Ciliary body Sclera Retina Vitreous Choroid Optic nerve Orbit (eye socket) 1 · Uveal melanoma (iris, ciliary body, choroid) 2 · Retinoblastoma (retina, children) 3 · Conjunctival tumors (surface) 4 · Eyelid tumors 5 · Orbital tumors (behind the eye)
Figure Cross-section of the eye and its surroundings, showing where the main eye tumors arise.

Is an intraocular tumor benign or malignant?

The table summarizes the lesions on this page and their usual management; every plan is individual.

Lesion (linked condition) Benign or malignant? Typical management
Choroidal nevus Benign; rarely becomes melanoma Photographs and periodic checks
CHRPE, solitary or grouped Benign Baseline photograph, occasional review
Multiple atypical pigment spots (familial adenomatous polyposis) Benign in themselves Family history; genetic and bowel evaluation
Astrocytic hamartoma (tuberous sclerosis complex) Benign Yearly checks; treatment only if it leaks or grows
Retinal hemangioblastoma (von Hippel-Lindau disease) Benign Laser, cryotherapy, PDT or radiation; genetic testing
Choroidal hemangioma (Sturge-Weber syndrome, diffuse type) Benign PDT if fluid affects vision
Choroidal osteoma Benign Observation; injections if new vessels form
Melanocytoma Benign; transformation rare Photographs, OCT, visual fields
Vasoproliferative tumor Benign Cryotherapy, laser, PDT or plaque if leaking
Myelinated nerve fibers, torpedo maculopathy Benign; not true tumors Usually none
Iris pigment epithelial cyst Benign Observation; rarely laser or surgery
Uveal melanoma Malignant Radiotherapy, laser or surgery
Retinoblastoma (inherited RB1 variant in about 4 in 10) Malignant Urgent specialist treatment
Vitreoretinal lymphoma Malignant Eye injections with hemato-oncology care
Choroidal metastasis Malignant Planned with the oncologist

The malignant tumors each have a detailed page: uveal melanoma, the most common primary cancer inside the adult eye; retinoblastoma, the most common one in children; vitreoretinal lymphoma, which often imitates uveitis; and choroidal metastasis, spread from a cancer elsewhere.

What is a pseudomelanoma?

A pseudomelanoma is a lesion that can be mistaken for melanoma: for example a choroidal nevus, CHRPE, bleeding under the retina, choroidal hemangioma, choroidal osteoma, melanocytoma, posterior scleritis, uveal effusion or a metastasis. Inflammation can do the same — in Retinal Cases & Brief Reports (2017), Prof. Türkoğlu reported a sarcoid granuloma that simulated a prostate cancer metastasis (see choroidal metastasis).

The distinction matters both ways: a harmless lesion should not receive radiation, and a small melanoma should not be dismissed as a freckle. Specialists therefore rely on multimodal imaging rather than appearance alone.

Which benign tumors can grow inside the eye?

Choroidal nevus

A common, freckle-like cluster of pigment cells in the choroid. Very few turn into melanoma; features such as thickness, fluid and orange pigment decide how often it is checked. See choroidal nevus.

CHRPE: when can dark spots point to bowel polyps?

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a flat, sharply outlined dark spot present from birth, seen in about 1.2% of people in an optometric study. It can be solitary, often with pale holes (lacunae) that enlarge slowly; grouped in clusters of small patches nicknamed "bear tracks"; or multiple and atypical — small oval or fish-shaped spots scattered through the retina.

Solitary and grouped CHRPE are benign. Many enlarge very slowly (46–83% over 3 or more years in studies), and only rarely does a nodule develop within one, so a baseline photograph is useful.

Multiple atypical lesions are different. Depending on the gene variant, they occur in up to 80–90% of people with familial adenomatous polyposis (FAP), an inherited condition caused by changes in the APC gene, and affect both eyes in about 78%. Without preventive surgery, bowel cancer is almost inevitable in classic FAP (at an average age of about 39), so affected families begin colonoscopy at around 10–15 years of age. If you have such spots, your eye doctor will ask about your family history and may recommend genetic and bowel evaluation.

Astrocytic hamartoma and tuberous sclerosis

A retinal astrocytic hamartoma is a benign overgrowth (hamartoma) of the retina's supporting glial cells, flat and semi-transparent or raised and calcified like a small mulberry. Most stay stable and spare vision; rarely one grows, leaks or bleeds and is treated with laser, PDT or injections.

Multiple hamartomas are a major diagnostic sign of tuberous sclerosis complex (TSC), a genetic condition (TSC1 or TSC2 gene) affecting many organs. About a third of people with TSC have them in GeneReviews data (some studies report more), while they are rare in the general population. People with TSC are advised to have a yearly eye examination, plus vision testing if they take the epilepsy drug vigabatrin. In a young child, a white retinal lesion must always be distinguished from retinoblastoma.

Retinal hemangioblastoma and von Hippel-Lindau disease

A retinal hemangioblastoma is a benign tumor of small blood vessels in the peripheral retina or on or next to the optic disc. It is the typical eye sign of von Hippel-Lindau (VHL) disease, an inherited condition affecting about 1 in 36,000 births that also causes tumors of the brain, spinal cord, kidneys and pancreas.

These tumors develop in about 70% of people with VHL, first detected at about 25 years of age on average (5% before age 10), and are often multiple and in both eyes. Because they threaten sight through leakage, bleeding and retinal detachment, people with VHL are advised to have a yearly eye examination from about age 1, and anyone with a retinal hemangioblastoma is usually offered VHL genetic testing. Small tumors are typically treated with laser; larger or peripheral ones with cryotherapy, PDT or plaque radiotherapy.

Belzutifan (Welireg), a HIF-2α inhibitor tablet, was approved by the US FDA in August 2021 for adults with VHL needing treatment for kidney cancer, brain or spinal cord hemangioblastomas or pancreatic neuroendocrine tumors not requiring immediate surgery. In the approval trial, 63% of patients with brain or spinal cord hemangioblastomas responded, and retinal tumors also improved. As of September 2026 it is not approved specifically for retinal tumors, so its use for the eye alone is off-label and limited to adults; anemia is a very common side effect.

Choroidal hemangioma

An orange-red, benign blood-vessel tumor of the choroid that can mimic an unpigmented melanoma or a metastasis. Photodynamic therapy is the main treatment when fluid blurs vision; see choroidal hemangioma.

Choroidal osteoma

A benign patch of bone inside the choroid, typically found in young women in their teens or twenties, usually in one eye, as a yellow-white to orange lesion near the optic disc. Ultrasound shows a bright echo that casts a shadow, and CT confirms the bone. In a series of 74 eyes followed for 10 years, osteomas grew in 41–51% and developed abnormal new blood vessels (choroidal neovascularization) in 31–47%; 56–58% of eyes ended with vision of 20/200 or worse. The new vessels are treated with anti-VEGF injections.

Melanocytoma

A very dark, benign variant of a nevus, most often on the optic disc. It usually causes no symptoms, although it can affect the pupil's response to light or part of the visual field. A minority enlarge slowly and transformation into melanoma is reported to be rare, so it is photographed and followed.

Vasoproliferative tumor of the retina

A benign vascular growth in the far periphery of the retina, usually appearing in the twenties or thirties. About 74% arise on their own; the rest follow other eye diseases such as uveitis, retinitis pigmentosa, Coats disease or injury. More than 80% leak fluid or exudates that can reach the macula; leaking tumors are treated with cryotherapy, laser, PDT or plaque brachytherapy.

Myelinated nerve fibers and torpedo maculopathy

Myelinated retinal nerve fibers are white patches where nerve fibers carry a myelin coating that the retina normally lacks; they are benign and usually stable. Torpedo maculopathy is a harmless congenital spot of the pigment layer near the center of the retina, named for its shape. Neither needs treatment, but both must be recognized so that they are not mistaken for a tumor, particularly in children.

Iris pigment epithelial cysts

Cysts of the pigment layer on the back of the iris are the most common iris cysts and can mimic a pigmented iris tumor. Ultrasound biomicroscopy (UBM), the reference test, shows a thin wall around fluid and so separates a cyst from a solid melanoma. Most need no treatment; if one narrows the eye's drainage angle, laser, aspiration or surgery can be used. See iris melanoma and iris spots.

How are intraocular tumors diagnosed?

Most are diagnosed without surgery. A dilated examination is combined with wide-field photographs, ultrasound, OCT, autofluorescence and fluorescein or indocyanine green angiography; UBM and anterior segment OCT image the iris, and CT or MRI answers specific questions. A biopsy is used selectively, for example when a metastasis from an unknown cancer is suspected. See how eye tumors are diagnosed.

Getting a specialist opinion in Antalya

Many benign lesions are first noticed by an optometrist or general ophthalmologist who wants confirmation. Prof. Türkoğlu reviews the full imaging record and explains whether a lesion is benign, whether it could signal a condition elsewhere and how often it should be checked. When a lesion points to VHL, tuberous sclerosis or FAP, care is coordinated with genetics and other specialists.

From abroad, a remote second opinion based on photographs, ultrasound and OCT scans can answer many questions, although some findings need an examination in person. Eye doctors can find referral criteria on our page for referring doctors.

Frequently asked questions

Is a benign eye tumor dangerous?

Most benign intraocular tumors are harmless to life, and many never affect vision. Some can threaten sight by leaking fluid or bleeding, such as retinal hemangioblastomas and choroidal hemangiomas, and a few are markers of inherited conditions affecting other organs. That is why even benign lesions are documented and followed.

My doctor saw "bear tracks" in my retina — is that cancer?

No. "Bear tracks" is the nickname for grouped CHRPE: clusters of small, flat, dark spots present from birth. They are benign and are not the pattern linked to bowel polyps, which consists of small oval spots scattered in both eyes. A baseline photograph and occasional review are usually enough.

Why would an eye doctor ask about bowel cancer?

Because multiple small, oval pigment spots in both eyes can be a sign of familial adenomatous polyposis, an inherited condition that leads to bowel polyps and, without preventive surgery, almost always to bowel cancer. If relatives have had bowel polyps or early bowel cancer, tell your eye doctor.

Can a tablet treat eye tumors in von Hippel-Lindau disease?

Belzutifan, approved in 2021 for VHL-related kidney, brain, spinal cord and pancreatic tumors, also shrinks retinal hemangioblastomas in some patients. As of September 2026 it is not approved for eye tumors alone, so such use is off-label and limited to adults; laser and other local treatments remain standard.

Can a choroidal osteoma cause vision loss?

Yes. The osteoma grows slowly, but abnormal new blood vessels can form over it and leak or bleed; in a 10-year study, more than half of affected eyes ended with vision of 20/200 or worse. The vessels respond to anti-VEGF injections, so report new blur or distortion promptly.

Are these conditions inherited?

Some are. Von Hippel-Lindau disease, tuberous sclerosis complex and familial adenomatous polyposis can run in families, so relatives may be offered testing. Solitary CHRPE, choroidal osteoma and circumscribed choroidal hemangioma are not inherited, and Sturge-Weber syndrome arises by chance rather than being passed on.

References

  1. EyeWiki (American Academy of Ophthalmology). Congenital Hypertrophy of the Retinal Pigment Epithelium. eyewiki.org
  2. GeneReviews (NCBI Bookshelf). [APC-associated polyposis conditions, including familial adenomatous polyposis]. ncbi.nlm.nih.gov
  3. GeneReviews (NCBI Bookshelf). [Tuberous sclerosis complex: diagnostic criteria and surveillance]. ncbi.nlm.nih.gov
  4. GeneReviews (NCBI Bookshelf). [Von Hippel-Lindau syndrome: clinical features, surveillance and treatment]. ncbi.nlm.nih.gov
  5. US Food and Drug Administration. [Approval of belzutifan for cancers associated with von Hippel-Lindau disease, August 2021]. fda.gov
  6. EyeWiki (American Academy of Ophthalmology). Intraocular Vascular Tumors. eyewiki.org
  7. EyeWiki (American Academy of Ophthalmology). Choroidal Osteoma. eyewiki.org
  8. EyeWiki (American Academy of Ophthalmology). Iris Cysts. eyewiki.org
  9. American Cancer Society. [Key statistics for retinoblastoma, including the share of heritable cases]. cancer.org
  10. Turkoglu EB, Say EAT, Shields CL. Spontaneous devascularization and detachment of optic nerve hemangioblastoma in a patient with von Hippel-Lindau disease. JAMA Ophthalmology, 2016;134(9):e161119. PubMed
  11. Rao R, Turkoglu EB, Say EAT, Shields CL. Clinical features, imaging, and natural history of myelinated retinal nerve fiber layer. Retina, 2019;39(6):1125-1132. PubMed
  12. Turkoglu EB, Erol MK, Karaca BO. Coexistence of torpedo maculopathy and retinoblastoma: differentiation the lesions with hand-held optical coherence tomography. Photodiagnosis and Photodynamic Therapy, 2021;34:102331. PubMed

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