Eye neoplasms can affect all parts of the
eye
Eyes are organs of the visual system. They provide living organisms with vision, the ability to receive and process visual detail, as well as enabling several photo response functions that are independent of vision. Eyes detect light and conv ...
, and can be a benign tumor or a malignant tumor (
cancer
Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal bl ...
). 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, the ...
(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 ...
and
lung cancer
Lung cancer, also known as lung carcinoma (since about 98–99% of all lung cancers are carcinomas), is a malignant lung tumor characterized by uncontrolled cell growth in tissues of the lung. Lung carcinomas derive from transformed, malign ...
. Other less common sites of origin include the
prostate
The prostate is both an accessory gland of the male reproductive system and a muscle-driven mechanical switch between urination and ejaculation. It is found only in some mammals. It differs between species anatomically, chemically, and phys ...
,
kidney
The kidneys are two reddish-brown bean-shaped organs found in vertebrates. They are located on the left and right in the retroperitoneal space, and in adult humans are about in length. They receive blood from the paired renal arteries; bloo ...
,
thyroid
The thyroid, or thyroid gland, is an endocrine gland in vertebrates. In humans it is in the neck and consists of two connected lobes. The lower two thirds of the lobes are connected by a thin band of tissue called the thyroid isthmus. The t ...
,
skin
Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation.
Other cuticle, animal coverings, such as the arthropod exoskeleton, have diffe ...
,
colon and
blood or bone marrow.
Types
Tumors in the eye and orbit can be
benign 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 ...
s, or
malignant
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 s ...
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 subty ...
and
retinoblastoma.
Malignant
The most common eyelid tumor is called
basal cell carcinoma. 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 and
malignant melanoma. The most common orbital malignancy is ''
orbital lymphoma''. 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. Chemothe ...
or
radiation therapy
Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Rad ...
.
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). ...
. These tumors can occur in the
choroid,
iris and
ciliary body. The latter are sometimes called iris or
ciliary body melanoma.
*The next most common is primary
intraocular lymphoma (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
Orbital may refer to:
Sciences Chemistry and physics
* Atomic orbital
* Molecular orbital
* Hybrid orbital Astronomy and space flight
* Orbit
** Earth orbit
Medicine and physiology
* Orbit (anatomy), also known as the ''orbital bone''
* Orbito ...
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 ...
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 surgery, 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. Followin ...
or
optometrist
Optometry is a specialized health care profession that involves examining the eyes and related structures for defects or abnormalities. Optometrists are health care professionals who typically provide comprehensive primary eye care.
In the Un ...
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
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, ...
*
Choroidal nevus
* Choroidal
osteoma
An osteoma (plural: "osteomata") is a new piece of bone usually growing on another piece of bone, typically the skull. It is a benign tumor.
When the bone tumor grows on other bone it is known as "homoplastic osteoma"; when it grows on other tiss ...
* Ciliary body
melanoma
* The
nevus of Ota
Nevus of Ota is a hyperpigmentation that occurs on the face, most often appearing on the white of the eye. It also occurs on the forehead, nose, cheek, periorbital region, and temple.
It was first reported by Masao Ōta of Japan in 1939.
Ca ...
Conjunctival tumors

* Conjunctival
Kaposi's sarcoma
* Epibulbar
dermoid
*
Malignant
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 s ...
conjunctival tumors
*
Lymphoma
Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enl ...
of the conjunctiva
*
Melanoma and
PAM with
atypia
*
Pigmented conjunctival tumors
*
Pingueculum
A pinguecula is a common type of conjunctival stromal degeneration in the human eye, eye. It appears as an elevated yellow-white plaque in the bulbar conjunctiva near the limbus. Calcification may also seen occasionally.
Etiology
The exact etiol ...
*
Pterygium
*
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 ...
of the conjunctiva
Treatment
*
Laser therapy
*
Plaque therapy
*
Radiotherapy
Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Ra ...
- 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
* 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 defen ...
- Removal of the eye contents, leaving the sclera or the white part of the eye.
* Exenteration
Eye surgery, also known as ophthalmic or ocular surgery, is surgery performed on the eye or its adnexa, by an ophthalmologist or sometimes, an optometrist. Eye surgery is synonymous with ophthalmology. The eye is a very fragile organ, and requ ...
- 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. Chemothe ...
Ocular oncology
Ocular oncology is the branch of medicine
Medicine is the science and Praxis (process), practice of caring for a patient, managing the diagnosis, prognosis, Preventive medicine, prevention, therapy, treatment, Palliative care, palliation of their injury or disease, and Health promotion ...
dealing with tumors relating to the eye
Eyes are organs of the visual system. They provide living organisms with vision, the ability to receive and process visual detail, as well as enabling several photo response functions that are independent of vision. Eyes detect light and conv ...
and its adnexa.
Ocular oncology
Oncology is a branch of medicine that deals with the study, treatment, diagnosis and prevention of cancer. A medical professional who practices oncology is an ''oncologist''. The name's etymological origin is the Greek word ὄγκος (''ó ...
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
Pediatrics ( also spelled ''paediatrics'' or ''pædiatrics'') is the branch of medicine that involves the medical care of infants, children, adolescents, and young adults. In the United Kingdom, paediatrics covers many of their youth until th ...
/internal medicine/ hospitalist 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