Eye neoplasm
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Eye neoplasms can affect all parts of the eye, 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 b ...
). 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 a r ...
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; blo ...
,
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 thy ...
,
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 de ...
, 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 s ...
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 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 subt ...
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 it is almost exclusively fo ...
.


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 Squamous-cell carcinomas (SCCs), also known as epidermoid carcinomas, comprise a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on th ...
,
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 sebaceous ...
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 ...
. 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. 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. Radi ...
.


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). Sym ...
. These tumors can occur in the choroid,
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 inclu ...
. *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 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 it is almost exclusively fo ...
, 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 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 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 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 ski ...
* 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 tissu ...
* 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 ÅŒta of Japan in 1939. Cau ...


Conjunctival tumors

* Conjunctival
Kaposi's sarcoma Kaposi's sarcoma (KS) is a type of cancer that can form masses in 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 limit ...
* 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 hai ...
*
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 enla ...
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 pter ...
*
Squamous carcinoma Squamous-cell carcinomas (SCCs), also known as epidermoid carcinomas, comprise a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on th ...
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 *
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 other ...
- 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 , first ...
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 An iridectomy, also known as a surgical iridectomy or corectomy, is the surgical removal of part of the iris.Cline D; Hofstetter HW; Griffin JR. ''Dictionary of Visual Science''. 4th ed. Butterworth-Heinemann, Boston 1997. 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 practice of caring for a patient, managing the diagnosis, prognosis, prevention, treatment, palliation of their injury or disease, and promoting their health. Medicine encompasses a variety of health care pr ...
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 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 (''à ...
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 (''ó ...
, 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 ho ...
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