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An eye neoplasm is a
tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
of the eye. A rare type of tumor, eye neoplasms can affect all parts of the
eye An eye is a sensory organ that allows an organism to perceive visual information. It detects light and converts it into electro-chemical impulses in neurons (neurones). It is part of an organism's visual system. In higher organisms, the ey ...
, and can either be benign or malignant (
cancerous Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Po ...
), in which case it is known as eye cancer. Eye cancers can be primary (starts within the eye) or metastatic cancer (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 a cancer that develops from breast tissue. Signs of breast cancer may include a Breast lump, lump in the breast, a change in breast shape, dimpling of the skin, Milk-rejection sign, milk rejection, fluid coming from the nipp ...
and
lung cancer Lung cancer, also known as lung carcinoma, is a malignant tumor that begins in the lung. Lung cancer is caused by genetic damage to the DNA of cells in the airways, often caused by cigarette smoking or inhaling damaging chemicals. Damaged ...
. Other less common sites of origin include the
prostate The prostate is an male accessory gland, accessory gland of the male reproductive system and a muscle-driven mechanical switch between urination and ejaculation. It is found in all male mammals. It differs between species anatomically, chemica ...
,
kidney In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organ (anatomy), organs that are a multilobar, multipapillary form of mammalian kidneys, usually without signs of external lobulation. They are located on the left and rig ...
,
thyroid The thyroid, or thyroid gland, is an endocrine gland in vertebrates. In humans, it is a butterfly-shaped gland located in the neck below the Adam's apple. It consists of two connected lobes. The lower two thirds of the lobes are connected by ...
,
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 animal coverings, such as the arthropod exoskeleton, have different ...
, 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; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
like dermoid cysts, or
malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
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 neoplasm, tumor are identified as rhabdomyoblasts. The four ...
and
retinoblastoma Retinoblastoma (Rb) is a rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common primary malignant intraocular cancer in children, and 80% of retinoblastoma cas ...
.


Malignant

The most common eyelid tumor is called
basal cell carcinoma Basal-cell carcinoma (BCC), also known as basal-cell cancer, basalioma, or rodent ulcer, is the most common type of skin cancer. It often appears as a painless, raised area of skin, which may be shiny with Telangiectasia, small blood vessels ru ...
. 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 chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
or
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
.


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). S ...
. 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. It contains connective tissues, and lies between the retina and the sclera. The human choroid is thickest at the far extreme rear o ...
, iris 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 ciliary ...
. 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 Retinoblastoma (Rb) is a rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common primary malignant intraocular cancer in children, and 80% of retinoblastoma cas ...
, affecting approximately 325 children per year in North America. Early detection has allowed for cures exceeding 95%. *The second most common is medulloepithelioma (also called diktyoma) which can occur in the ciliary body and the uvea of the eye.


Benign

Orbital dermoid cysts 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 the branch of medicine that deals with the diagnosis, treatment, and surgery of eye diseases and disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Following a ...
or
optometrist Optometry is the healthcare practice concerned with examining the eyes for visual defects, prescribing corrective lenses, and detecting eye abnormalities. In the United States and Canada, optometrists are those that hold a post-baccalaureate f ...
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 A hemangioma or haemangioma is a usually benign vascular tumor derived from blood vessel cell types. The most common form, seen in infants, is an infantile hemangioma, known colloquially as a "strawberry mark", most commonly presenting on the sk ...
* Choroidal
melanoma Melanoma is the most dangerous type of skin cancer; it develops from the melanin-producing cells known as melanocytes. It typically occurs in the skin, but may rarely occur in the mouth, intestines, or eye (uveal melanoma). In very rare case ...
* Choroidal
metastasis Metastasis is a pathogenic agent's spreading 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, ...
* Choroidal nevus * Choroidal osteoma * Ciliary body
melanoma Melanoma is the most dangerous type of skin cancer; it develops from the melanin-producing cells known as melanocytes. It typically occurs in the skin, but may rarely occur in the mouth, intestines, or eye (uveal melanoma). In very rare case ...
* The nevus of Ota


Conjunctival tumors

* Conjunctival
Kaposi's sarcoma Kaposi's sarcoma (KS) is a type of cancer that can form masses on the skin, in lymph nodes, in the mouth, or in other organs. The skin lesions are usually painless, purple and may be flat or raised. Lesions can occur singly, multiply in a limite ...
* Epibulbar dermoid *
Malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
conjunctival tumors *
Lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). The name typically refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlarged lymph node ...
of the conjunctiva *
Melanoma Melanoma is the most dangerous type of skin cancer; it develops from the melanin-producing cells known as melanocytes. It typically occurs in the skin, but may rarely occur in the mouth, intestines, or eye (uveal melanoma). In very rare case ...
and PAM with atypia * Pigmented conjunctival tumors * Pingueculum * Pterygium * Squamous carcinoma and intraepithelial neoplasia of the conjunctiva


Treatment

* Laser therapy * Plaque therapy *
Radiotherapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a treatment using ionizing radiation, generally provided as part of cancer therapy to either kill or control the growth of malignant cells. It is normally delivered by a linear particle ...
- 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 ...
*
Enucleation of the eye Enucleation is the removal of the eye that leaves the eye muscles and remaining orbital contents intact. This type of ocular surgery is indicated for a number of ocular tumors, in eyes that have sustained severe trauma, and in eyes that are oth ...
- 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 An ocularist specializes in the fabrication and fitting of ocular prostheses for people who have lost an eye or eyes due to trauma or illness.{{Cite journal , last1=Khandekar , first1=Rajiv , last2=Changal , first2=Nusrat , last3=AlRasheed , first3 ...
and is made to look like the person's real eye) * Evisceration - 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 chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...


Ocular oncology

Ocular oncology is the branch of
medicine Medicine is the science and Praxis (process), practice of caring for patients, managing the Medical diagnosis, diagnosis, prognosis, Preventive medicine, prevention, therapy, treatment, Palliative care, palliation of their injury or disease, ...
dealing with
tumors A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
relating to the
eye An eye is a sensory organ that allows an organism to perceive visual information. It detects light and converts it into electro-chemical impulses in neurons (neurones). It is part of an organism's visual system. In higher organisms, the ey ...
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 Etymology, etymological origin is the Greek word ὄγ ...
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 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 (''� ...
, radiation specialist, head & neck surgeon/ENT surgeon, pediatrician/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