Brooke-Spiegler Syndrome, Multiple Familial Trichoepithelioma, and Familial CylindromatosisBrooke-Spiegler Syndrome (BSS), familial cylindromatosis, and multiple familial trichoepithelioma (MFT) are all autosomal dominant syndromes with overlapping clinical characteristics with allelic variance. Features of BSS include multiple skin appendage tumors such as cylindromas (tumors arising in the hair follicle stem cells), trichoepitheliomas (tumors arising in the hair follicle), and spiradenomas (benign tumors arising in the sweat gland). MFT is characterized by nonmalignant skin tumors, primarily trichoepitheliomas, and familial cylindromatosis manifests predominantly as cutaneous cylindromas. Onset of tumors for these syndromes is typically in late childhood or early adolescence, suggesting a hormonal influence. There is some evidence of greater severity in females than in males. UV radiation appears to be a major initiating factor for cylindromas. Typical tumor sites for
Eye-sparing radiation therapy, either by plaque brachytherapy or external beam, is the preferred option for most patients with medium-sized choroidal melanoma. Enucleation remains the standard therapy for large, choroidal melanomas and melanomas that cause severe glaucoma or invade the optic nerve. Standard treatment options:Tumor growth pattern is a factor in the therapeutic decision. If there is a diffuse melanoma or if there is extraocular extension, enucleation should be considered, but radiation therapy can be employed for less extensive disease. Medium-sized choroidal melanomasPlaque radiation therapy.[1,2,3,4]External-beam, charged-particle radiation therapy: This approach is offered at specialized referral centers. It requires careful patient cooperation, with voluntary fixation of gaze.[5,6,7]Local eye-wall resection.[8,9]Combined therapy, with ablative laser coagulation or transpupillary thermotherapy to supplement plaque treatment.[10,11]Enucleation. This approach is
There are different types of treatments for patients with intraocular melanoma. Different types of treatments are available for patients with intraocular melanoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.Five types of standard treatment are used:SurgerySurgery is the most common treatment for intraocular melanoma. The following types of surgery may be used:Resection: Surgery to remove the tumor and a small amount of healthy tissue around it.Enucleation: Surgery to remove the eye and part of
The most common causes of melanoma are: Exposure to ultraviolet radiation from the sun between 10:00 a.m. and 3:00 p.m. and exposure at high altitudes. Damage to the DNA of melanocytes from exposure to the sun and its UV light radiation is the single most
Skin cancer is often or usually caused by years of too much sun exposure. More than 90% of all skin cancers are found on body parts that get the most sun most of the time. The face,neck,ears,hands,and arms are common body parts that get skin cancer. Skin cancer can often be prevented by avoiding overexposure to the sun's ultraviolet rays (UV rays). UV rays from artificial sources,such as ...