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Eye neoplasms can affect all parts of the eye, and can be a benign tumor or a malignant tumor ( cancer). Eye cancers can be primary (starts within the eye) or
metastatic cancer Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then, ...
(spread to the eye from another organ). The two most common cancers that spread to the eye from another organ are
breast cancer Breast cancer is cancer that develops from breast tissue. Signs of breast cancer may include a lump in the breast, a change in breast shape, dimpling of the skin, milk rejection, fluid coming from the nipple, a newly inverted nipple, or a r ...
and lung cancer. Other less common sites of origin include the prostate, kidney, thyroid, skin, colon and blood or bone marrow.


Types

Tumors in the eye and orbit can be
benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not ...
like
dermoid cyst A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair ...
s, or malignant like
rhabdomyosarcoma Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts. There are four sub ...
and retinoblastoma.


Malignant

The most common eyelid tumor is called
basal cell carcinoma Basal-cell carcinoma (BCC), also known as basal-cell cancer, is the most common type of skin cancer. It often appears as a painless raised area of skin, which may be shiny with small blood vessels running over it. It may also present as a raise ...
. This tumor can grow around the eye but rarely spreads to other parts of the body. Other types of common eyelid cancers include squamous carcinoma,
sebaceous carcinoma Sebaceous carcinoma, also known as sebaceous gland carcinoma (SGc), sebaceous cell carcinoma, and meibomian gland carcinoma is an uncommon malignant cutaneous tumor. Most are typically about 1.4 cm at presentation. SGc originates from sebace ...
and
malignant melanoma Melanoma, also redundantly known as malignant melanoma, is a type of skin cancer that develops from the pigment-producing cells known as melanocytes. Melanomas typically occur in the skin, but may rarely occur in the mouth, intestines, or eye (u ...
. The most common orbital malignancy is ''
orbital lymphoma Orbital lymphoma is a common type of non-Hodgkin lymphoma that occurs near or on the eye. Common symptoms include decreased vision and uveitis. Orbital lymphoma can be diagnosed via a biopsy of the eye and is usually treated with radiotherapy or ...
''. This tumor can be diagnosed by biopsy with histopathologic and immunohistochemical analysis. Most patients with orbital lymphoma can be offered
chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...
or radiation therapy.


Adults

*The most common malignant primary intraocular tumor in adults is
uveal melanoma Uveal melanoma is a type of eye cancer in the uvea of the eye. It is traditionally classed as originating in the iris, choroid, and ciliary body, but can also be divided into class I (low metastatic risk) and class II (high metastatic risk). Symp ...
. These tumors can occur in the
choroid The choroid, also known as the choroidea or choroid coat, is a part of the uvea, the vascular layer of the eye, and contains connective tissues, and lies between the retina and the sclera. The human choroid is thickest at the far extreme rear of ...
,
iris Iris most often refers to: *Iris (anatomy), part of the eye *Iris (mythology), a Greek goddess * ''Iris'' (plant), a genus of flowering plants * Iris (color), an ambiguous color term Iris or IRIS may also refer to: Arts and media Fictional ent ...
and
ciliary body The ciliary body is a part of the eye that includes the ciliary muscle, which controls the shape of the lens, and the ciliary epithelium, which produces the aqueous humor. The aqueous humor is produced in the non-pigmented portion of the ciliar ...
. The latter are sometimes called iris or
ciliary body melanoma Ciliary body melanoma is a type of cancer arising from the coloured part (uvea) of the eye. About 12% of uveal melanoma arise from the ciliary body. Clinical features It occurs most commonly in the sixth decade of life. * External signs includ ...
. *The next most common is primary
intraocular lymphoma Intraocular lymphoma is a rare malignant form of eye cancer. Intraocular lymphoma may affect the eye secondarily from a metastasis from a non-ocular tumor or may arise within the eye primarily (primary intraocular lymphoma, PIOL). PIOL is a subset ...
(PIOL) which is usually non-Hodgkin’s, large cell lymphoma of the B-cell type, although T cell lymphomas have also been described.


Children

*The most common malignant intraocular tumor in children is retinoblastoma, affecting approximately 325 children per year in North America. Early detection has allowed for cures exceeding 95%. *The second most common is
medulloepithelioma Medulloepithelioma is a rare, primitive, fast-growing brain tumour thought to stem from cells of the embryonic medullary cavity.
(also called diktyoma) which can occur in the ciliary body and the uvea of the eye.


Benign

Orbital
dermoid cyst A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair ...
s are benign choristomas which are typically found at the junction of sutures, most commonly at the fronto-zygomatic suture. Large deep orbital dermoid cysts can cause pressure effects on the muscles and optic nerve, leading to diplopia and loss of vision.


Signs and symptoms

* Melanomas (choroidal, ciliary body and uveal) - In the early stages there may be no symptoms (the person does not know there is a tumor until an
ophthalmologist Ophthalmology ( ) is a surgical subspecialty within medicine that deals with the diagnosis and treatment of eye disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Following a medic ...
or optometrist looks into the eye with an ophthalmoscope during a routine test). As the tumor grows, symptoms can be blurred vision, decreased vision, double vision, eventual vision loss and if they continue to grow the tumor can break past the retina causing retinal detachment. Sometimes the tumor can be visible through the pupil. * A nevus is a benign, freckle in the eye. These should be checked out and regular checks on the eye done to ensure it has not turned into a melanoma. * Iris and conjuctival tumors (melanomas) - Present as a dark spot. Any spot which continues to grow on the iris and the conjunctiva should be checked out. * Retinoblastoma - Strabismus (crossed eyes), a whitish or yellowish glow through the pupil, decreasing/loss of vision, sometimes the eye may be red and painful. Retinoblastoma can occur in one or both eyes. This tumor occurs in babies and young children. It is called RB for short. Check photographs, normal healthy eyes would have the red eye reflex, but a white/yellow dot instead of the red eye reflex can indicate a tumor or some other kind of eye disease. Any photos of a child/children which have a white/yellow dot instead of the red eye reflex should be evaluated by an eye doctor.


Diagnosis


Classification


Choroidal tumors

* Choroidal hemangioma * Choroidal melanoma * Choroidal metastasis * Choroidal nevus * Choroidal osteoma * Ciliary body melanoma * The nevus of Ota


Conjunctival tumors

* Conjunctival Kaposi's sarcoma * Epibulbar
dermoid A dermoid cyst is a teratoma of a cystic nature that contains an array of developmentally mature, solid tissues. It frequently consists of skin, hair follicles, and sweat glands, while other commonly found components include clumps of long hair, ...
* Malignant conjunctival tumors * Lymphoma of the conjunctiva * Melanoma and PAM with
atypia Atypia (from Greek, ''a'' + ''typos'', without type; a condition of being irregular or nonstandard) is a histopathologic term for a structural abnormality in a cell, i.e. it is used to describe atypical cells. Atypia can be caused by an infection ...
* Pigmented conjunctival tumors * Pingueculum *
Pterygium Pterygium (plural ''pterygia'' or ''pterygiums'') refers to any wing-like triangular membrane occurring in the neck, eyes, knees, elbows, ankles or digits. The term comes from the Greek word ''pterygion'' meaning "wing". Types * Popliteal ptery ...
* Squamous carcinoma and
intraepithelial neoplasia Intraepithelial neoplasia (IEN) is the development of a benign neoplasia or high-grade dysplasia in an epithelium. The exact dividing line between dysplasia and neoplasia has been very difficult to draw throughout the era of medical science. It va ...
of the conjunctiva


Treatment

*
Laser therapy Laser medicine consists in the use of lasers in medical diagnosis, treatments, or therapies, such as laser photodynamic therapy, photorejuvenation, and laser surgery. Lasers Lasers used in medicine include in principle any type of laser, but ...
* Plaque therapy * Radiotherapy - The ophthalmologist decides in conjunction with the radiation oncologist which type of radiation therapy is most suitable, based on size and location of the tumour. Today, modern radiation treatment modalities, such as proton therapy, are likely to be chosen, for providing superior accuracy in dose delivery, helping to spare healthy tissue and the sensitive optic nerves.Boris Peter Selby, et al. (2007) Pose estimation of eyes for particle beam treatment of tumors. In: Medical Image Processing 2007. Algorithms - Systems - Applications; Springer Press Berlin, Heidelberg
/ref> *
Proton therapy In medicine, proton therapy, or proton radiotherapy, is a type of particle therapy that uses a beam of protons to irradiate diseased tissue, most often to treat cancer. The chief advantage of proton therapy over other types of external beam ra ...
* Enucleation of the eye - Removal of the eye, but the muscles and eyelids are left intact. An implant is inserted, then the person wears a conformer shield and later the person will have their prosthesis made and fitted (the prosthesis is made by an ocularist and is made to look like the person's real eye) *
Evisceration Evisceration (pronunciation: /ɪvɪsəˈreɪʃən/) is disembowelment, i.e., the removal of viscera (internal organs, especially those in the abdominal cavity). The term may also refer to: * Evisceration (autotomy), ejection of viscera as a defe ...
- Removal of the eye contents, leaving the sclera or the white part of the eye. * Exenteration - Removal of the eye, all orbital contents, which can involve the eyelids as well. A special prosthesis is made to cover the defect and improve appearance. * Iridectomy - Removal of the affected piece of the iris * Choroidectomy - Removal of the choroid layer (the vascular tissue sandwiched between the sclera and the retina) * Iridocyclectomy - Removal of the iris plus the ciliary body muscle. * Eyewall resection - Cutting into the eye to remove a tumor e.g. melanoma. This operation can be quite difficult to perform. *
Chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...


Ocular oncology

Ocular oncology is the branch of medicine dealing with tumors relating to the eye and its adnexa. Ocular oncology takes into consideration that the primary requirement for patients is preservation of life by removal of the tumor, along with best efforts directed at preservation of useful vision, followed by cosmetic appearance. The treatment of ocular tumors is generally a multi-specialty effort, requiring coordination between the ophthalmologist, medical oncologist, radiation specialist, head & neck surgeon/ENT surgeon, pediatrician/internal medicine/
hospitalist Hospital medicine is a medical specialty that exists in some countries as a branch of family medicine or internal medicine, dealing with the care of acutely ill hospitalized patients. Physicians whose primary professional focus is caring for hos ...
and a multidisciplinary team of support staff and nurses.


References


Further reading

*


External links


Eye Cancer - Medline Plus


* DeAngelis, D; Hurwitz, J

eMedicine.com. June 8, 2005. {{Authority control Ocular neoplasia