Ocular oncology
Encyclopedia
Ocular oncology is the branch of medicine
dealing with tumors relating to the eye
and its adnexa
. Eye cancer can affect all parts of the eye
.
(spread to the eye from another organ). The two most common cancers that spread to the eye from another organ are breast cancer
and lung cancer
. Other less common sites of origin include the prostate
, kidney
, thyroid
, skin
, colon
and blood or bone marrow
.
like dermoid cyst
s, or malignant
like rhabdomyosarcoma
and retinoblastoma
.
. 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
or radiation therapy
.
dermoid cyst
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 have cause pressure effects on the muscles and optic nerve, leading to diplopia and loss of vision.
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 and a multidisciplinary team of support staff and nurses.
Medicine
Medicine is the science and art of healing. It encompasses a variety of health care practices evolved to maintain and restore health by the prevention and treatment of illness....
dealing with tumors relating to the eye
Human eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
and its adnexa
Adnexa
In anatomy, adnexa refers to the appendages of an organ. The term adnexa stems from a Latin word meaning appendages.More specifically, it can refer to:* Adnexa of eye * Adnexa of uterus...
. Eye cancer can affect all parts of the eye
Human eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
.
Origin and location
Eye cancers can be primary (starts within the eye) and metastatic cancerMetastasis
Metastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
(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
Breast cancer is cancer originating from breast tissue, most commonly from the inner lining of milk ducts or the lobules that supply the ducts with milk. Cancers originating from ducts are known as ductal carcinomas; those originating from lobules are known as lobular carcinomas...
and lung cancer
Lung cancer
Lung cancer is a disease characterized by uncontrolled cell growth in tissues of the lung. If left untreated, this growth can spread beyond the lung in a process called metastasis into nearby tissue and, eventually, into other parts of the body. Most cancers that start in lung, known as primary...
. Other less common sites of origin include the prostate
Prostate
The prostate is a compound tubuloalveolar exocrine gland of the male reproductive system in most mammals....
, kidney
Kidney
The kidneys, organs with several functions, serve essential regulatory roles in most animals, including vertebrates and some invertebrates. They are essential in the urinary system and also serve homeostatic functions such as the regulation of electrolytes, maintenance of acid–base balance, and...
, thyroid
Thyroid
The thyroid gland or simply, the thyroid , in vertebrate anatomy, is one of the largest endocrine glands. The thyroid gland is found in the neck, below the thyroid cartilage...
, skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
, colon
Colon (anatomy)
The colon is the last part of the digestive system in most vertebrates; it extracts water and salt from solid wastes before they are eliminated from the body, and is the site in which flora-aided fermentation of unabsorbed material occurs. Unlike the small intestine, the colon does not play a...
and blood or bone marrow
Leukemia
Leukemia or leukaemia is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells called "blasts". Leukemia is a broad term covering a spectrum of diseases...
.
Types
Tumors in the eye and orbit can be benignBenign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
like dermoid cyst
Dermoid cyst
A dermoid cyst is a cystic teratoma that contains developmentally mature skin complete with hair follicles and sweat glands, sometimes clumps of long hair, and often pockets of sebum, blood, fat, bone, nails, teeth, eyes, cartilage, and thyroid tissue. Because it contains mature tissue, a dermoid...
s, or malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
like rhabdomyosarcoma
Rhabdomyosarcoma
A rhabdomyosarcoma is a type of cancer, specifically a sarcoma , in which the cancer cells are thought to arise from skeletal muscle progenitors. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location...
and retinoblastoma
Retinoblastoma
Retinoblastoma is a rapidly developing cancer that develops in the cells of retina, the light-detecting tissue of the eye. In the developed world, Rb has one of the best cure rates of all childhood cancers , with more than nine out of every ten sufferers surviving into...
.
Malignant
The most common eyelid tumor is called basal cell carcinomaBasal cell carcinoma
Basal-cell carcinoma is the most common type of skin cancer. It rarely metastasizes or kills. However, because it can cause significant destruction and disfigurement by invading surrounding tissues, it is still considered malignant. Statistically, approximately 3 out of 10 Caucasians may develop a...
. 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
Sebaceous carcinoma is an uncommon and aggressive malignant cutaneous tumor. Most are typically about 10 mm in size at presentation. This neoplasm is thought to arise from sebaceous glands in the skin and, therefore, may originate anywhere in the body where these glands are found. Because the...
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
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
or radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
.
Adults
- The most common malignant primary intraocular tumor in adults is uveal melanomaUveal melanomaUveal melanoma is a cancer of the eye involving the iris, ciliary body, or choroid . Tumors arise from the pigment cells that reside within the uvea giving color to the eye...
. These tumors can occur in the choroidChoroidThe choroid, also known as the choroidea or choroid coat, is the vascular layer of the eye, containing connective tissue, and lying between the retina and the sclera. The human choroid is thickest at the far extreme rear of the eye , while in the outlying areas it narrows to 0.1 mm...
, irisIris (anatomy)The iris is a thin, circular structure in the eye, responsible for controlling the diameter and size of the pupils and thus the amount of light reaching the retina. "Eye color" is the color of the iris, which can be green, blue, or brown. In some cases it can be hazel , grey, violet, or even pink...
and ciliary bodyCiliary bodyThe ciliary body is the circumferential tissue inside the eye composed of the ciliary muscle and ciliary processes. It is triangular in horizontal section and is coated by a double layer, the ciliary epithelium. This epithelium produces the aqueous humor. The inner layer is transparent and covers...
. The latter are sometimes called iris or ciliary body melanomaCiliary body melanomaCiliary Body Melanoma is a type of cancer arising from the coloured part of the eye.About 12% of uveal melanoma arise from the ciliary body.-Clinical features:It occurs most commonly in the sixth decade....
. - The next most common is primary intraocular lymphomaIntraocular lymphomaIntraocular 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 . PIOL is a subset of primary central nervous system 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 retinoblastomaRetinoblastomaRetinoblastoma is a rapidly developing cancer that develops in the cells of retina, the light-detecting tissue of the eye. In the developed world, Rb has one of the best cure rates of all childhood cancers , with more than nine out of every ten sufferers surviving into...
. Affecting approximately 325 children per year in North America, early detection has allowed for cures exceeding 95%. - The second most common is medulloepitheliomaMedulloepitheliomaMedulloepithelioma is a rare, primitive, fast growing brain tumour thought to stem from cells of the embryonic medullary cavity. Tumours originating in the ciliary body of the eye are referred to as embryonal medulloepitheliomas, or diktyomas....
(also called diktyoma) which can occur in the ciliary body and the uvea of the eye.
Benign
OrbitalOrbit (anatomy)
In anatomy, the orbit is the cavity or socket of the skull in which the eye and its appendages are situated. "Orbit" can refer to the bony socket, or it can also be used to imply the contents...
dermoid cyst
Dermoid cyst
A dermoid cyst is a cystic teratoma that contains developmentally mature skin complete with hair follicles and sweat glands, sometimes clumps of long hair, and often pockets of sebum, blood, fat, bone, nails, teeth, eyes, cartilage, and thyroid tissue. Because it contains mature tissue, a dermoid...
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 have 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.
- Nevus - Are benign, freckle in the eye. These should be checked out and regular checks on the eye done to ensure it hasn't turned into a melanoma.
- Iris and conjuctival tumors (melanomas) - Presents 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. Please check photographs of your child or children, 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 checked out. Photographs of a child/children are very good clues as they can save the eyes, vision and lives of children, the other object is the ophthalmoscope.
Treatment
- Laser therapy
- Plaque therapy
- Radiotherapy
- 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 someone called an ocularist and is made to look like their 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.
- IridectomyIridectomyAn iridectomy, also known as a surgical iridectomy or corectomy, is the surgical removal of part of the iris. These procedures are most frequently performed in the treatment of closed-angle glaucoma and iris melanoma....
- Removal of the affected piece of the iris - Choroidectomy - Removal of the choroid layer (the sandwich 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.
- ChemotherapyChemotherapyChemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
Choroidal tumors
- Choroidal hemangiomaHemangiomaA hemangioma of infancy is a benign self-involuting tumor of endothelial cells, the cells that line blood vessels. It usually appears during the first weeks of life and sometimes resolves by age 10. In more severe case hemangioma may have permanency, if not treated by a physician...
- Choroidal melanomaMelanomaMelanoma is a malignant tumor of melanocytes. Melanocytes are cells that produce the dark pigment, melanin, which is responsible for the color of skin. They predominantly occur in skin, but are also found in other parts of the body, including the bowel and the eye...
- Choroidal metastasisMetastasisMetastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
- Choroidal nevusNevusNevus is the medical term for sharply-circumscribed and chronic lesions of the skin. These lesions are commonly named birthmarks and moles. Nevi are benign by definition...
- Choroidal osteomaOsteomaAn osteoma 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 tissue it is called "heteroplastic osteoma".Osteoma represents the most common...
- Ciliary body melanomaMelanomaMelanoma is a malignant tumor of melanocytes. Melanocytes are cells that produce the dark pigment, melanin, which is responsible for the color of skin. They predominantly occur in skin, but are also found in other parts of the body, including the bowel and the eye...
- The nevus of OtaNevus of OtaNevus of Ota is a blue hyperpigmentation that occurs on the face. It was first reported by Dr. M. T. Ota of Japan in 1939.Nevus of Ota is caused by the entrapment of melanocytes in the upper third of the dermis...
Conjunctival tumors
- Conjunctival Kaposi's sarcomaKaposi's sarcomaKaposi's sarcoma is a tumor caused by Human herpesvirus 8 , also known as Kaposi's sarcoma-associated herpesvirus . It was originally described by Moritz Kaposi , a Hungarian dermatologist practicing at the University of Vienna in 1872. It became more widely known as one of the AIDS defining...
- Epibulbar dermoid
- MalignantMalignantMalignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
conjunctival tumors
- LymphomaLymphomaLymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
of the conjunctiva
- MelanomaMelanomaMelanoma is a malignant tumor of melanocytes. Melanocytes are cells that produce the dark pigment, melanin, which is responsible for the color of skin. They predominantly occur in skin, but are also found in other parts of the body, including the bowel and the eye...
and PAM with atypiaAtypiaAtypia is a clinical term for abnormality in a cell. The term is medical jargon for an atypical cell. Atypia: Etymology: Gk, a + typos, without type; a condition of being irregular or nonstandard....
- Pigmented conjunctival tumors
- Pingueculum
- PterygiumPterygiumPterygium most often refers to a benign growth of the conjunctiva. A pterygium commonly grows from the nasal side of the sclera. It is usually present in the palpebral fissure. It is associated with, and thought to be caused by ultraviolet-light exposure , low humidity, and dust...
- Squamous carcinoma and intraepithelial neoplasiaIntraepithelial neoplasiaIntraepithelial neoplasia can refer to:* Vulvar intraepithelial neoplasia* Prostatic intraepithelial neoplasia* Cervical intraepithelial neoplasia* Endometrial intraepithelial neoplasia* Vaginal intraepithelial neoplasia...
of the conjunctiva
Ocular oncology
Ocular oncologyOncology
Oncology is a branch of medicine that deals with cancer...
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 and a multidisciplinary team of support staff and nurses.
External links
- The Tumori Foundation
- The See A Cure Foundation. A grassroots, patient-based, eye cancer nonprofit
- The Ocular Oncology Service
- The Eye Cancer Network
- Cancer.Net: Eye Cancer
- Eye Cancer - Medline Plus
- Ocular Oncology - Bascom Palmer Eye Institute
Specific types
- Cancer.Net: Eyelid Cancer
- DeAngelis, D; Hurwitz, J. "Lacrimal Gland Tumors." eMedicine.com. June 8, 2005.
- Cancer.Net: Lacrimal Gland Tumor
- Cancer.Net: Retinoblastoma, Childhood