Nevoid Basal Cell Carcinoma Syndrome (also known as Basal Cell Nevus Syndrome) is a rare genetic disorder characterized by the presence of multiple basal cell carcinomas (BCCs) on the skin, as well as other physical abnormalities. It is caused by a mutation in the PTCH1 gene, which is responsible for controlling the growth of cells in the skin. People with this syndrome are at an increased risk of developing BCCs, as well as other types of skin cancer. Other physical abnormalities associated with this syndrome include skeletal abnormalities, cysts, and benign tumors.

The most common symptoms of Nevoid Basal Cell Carcinoma Syndrome (Basal Cell Nevus Syndrome) include:

-Multiple basal cell carcinomas (BCCs)
-Multiple keratocystic odontogenic tumors (KCOTs)
-Palmar and/or plantar pits
-Facial skeletal abnormalities, such as macrocephaly, frontal bossing, and mandibular prognathism
-Ocular abnormalities, such as hypertelorism, strabismus, and cataracts
-Neurological abnormalities, such as seizures, mental retardation, and hydrocephalus
-Skeletal abnormalities, such as scoliosis, spina bifida, and rib anomalies
-Cardiac abnormalities, such as ventricular septal defects and patent ductus arteriosus

The exact cause of Nevoid Basal Cell Carcinoma Syndrome (Basal Cell Nevus Syndrome) is unknown. However, it is believed to be caused by a mutation in the PTCH1 gene, which is responsible for controlling the development of certain tissues in the body. This mutation is usually inherited from a parent, although it can also occur spontaneously.

The primary treatment for Nevoid Basal Cell Carcinoma Syndrome (Basal Cell Nevus Syndrome) is surgical removal of the affected areas. This may include excision of the nevi, or removal of the entire affected area. In some cases, radiation therapy may be used to treat the affected areas. In addition, topical medications such as imiquimod or 5-fluorouracil may be used to reduce the size of the nevi. In some cases, chemotherapy may be used to treat the underlying cause of the syndrome. Finally, regular skin examinations and sun protection are important to help prevent the development of skin cancer.

1. Exposure to ultraviolet (UV) radiation from the sun or tanning beds
2. Fair skin, light hair, and blue eyes
3. Family history of Nevoid Basal Cell Carcinoma Syndrome
4. History of multiple basal cell carcinomas
5. History of multiple keratoses
6. History of multiple cysts
7. History of multiple skeletal abnormalities
8. History of multiple jaw cysts
9. History of multiple palmar and/or plantar pits
10. History of multiple calcifications of the falx cerebri

There is no cure for Nevoid Basal Cell Carcinoma Syndrome (Basal Cell Nevus Syndrome). Treatment is focused on managing the symptoms and preventing the development of skin cancer. This may include regular skin exams, sun protection, and medications such as retinoids and immunomodulators. Surgery may be necessary to remove any tumors or lesions that develop.