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Skin cancer syndromes

Basal Cell Nevus Syndrome (BCNS) or Gorlin Syndrome, is a rare hereditary cancer syndrome associated with

  • Skin cancer called basal cell carcinoma (BCC) seen either at an early age or being diagnosed with multiple BCCs over a lifetime
  • Jaw cysts
  • Non-cancerous fibromas or dermoid tumors of the ovary
  • Oftentimes individuals who have NBCCS have macrocephaly (large heads), bone changes seen on x-rays (lamellar calcification of the falx) and other skeletal changes

Inherited Melanoma

Melanoma is a skin cancer that is sometimes inherited or seen in families. Melanoma can occur in more than one family member because families may have inherited fair skin or have the same exposure to sun. Some families have an inherited melanoma caused by a specific gene change.

The most common gene associated with hereditary melanoma is CDKN2A. This gene has multiple names (MTS1, INK4, MLM) and is commonly called by the name of its protein, p16. The risk for melanoma due to CDKN2A mutations differ widely, and may be influenced by sun exposure. Some CDKN2A mutation carrier families are at risk for both:

  • Melanoma
  • Pancreatic cancer

Birt-Hogg-Dubé (BHDS) is a rare disorder that affects the skin and lungs. The condition is characterized by

  • Multiple noncancerous (benign) skin tumors, particularly on the face, neck, and upper chest. These growths typically first appear in a person's twenties or thirties and become larger and more numerous over time
  • Cysts in the lungs and an abnormal accumulation of air in the chest cavity (pneumothorax) that may result in the collapse of a lung
  • Cancerous or noncancerous kidney tumors