MLchartDataset catalogue

nevoid basal cell carcinoma syndrome

Term · Oncology and biomedicine · MLC-T-ONC-005796

A rare, inherited disorder that affects many organs and tissues in the body. People with this disorder have a very high risk of developing basal cell skin cancer during adolescence or early adulthood. They are also at risk of developing medulloblastoma (a type of brain cancer) and other types of cancer. Nevoid basal cell carcinoma syndrome may also cause benign (not cancer) tumors in the jaw, heart, or ovaries. Other signs and symptoms include a large head and unusual facial features; small pits in the skin on the hands and feet; abnormalities of the spine, ribs, or skull; eye problems; and developmental problems. Nevoid basal cell carcinoma syndrome is caused by a mutation (change) in the PTCH1 gene. Also called basal cell nevus syndrome, BCNS, Gorlin syndrome, and NBCCS.

Table 1. Record
IdentifierMLC-T-ONC-005796
FieldOncology and biomedicine
Synonymsbasal cell nevus syndrome; BCNS; Gorlin syndrome; NBCCS
ReferencesNCI Dictionary of Cancer Terms
Record as JSON
{
  "id": "MLC-T-ONC-005796",
  "term": "nevoid basal cell carcinoma syndrome",
  "field": "Oncology and biomedicine",
  "definition": "A rare, inherited disorder that affects many organs and tissues in the body. People with this disorder have a very high risk of developing basal cell skin cancer during adolescence or early adulthood. They are also at risk of developing medulloblastoma (a type of brain cancer) and other types of cancer. Nevoid basal cell carcinoma syndrome may also cause benign (not cancer) tumors in the jaw, heart, or ovaries. Other signs and symptoms include a large head and unusual facial features; small pits in the skin on the hands and feet; abnormalities of the spine, ribs, or skull; eye problems; and developmental problems. Nevoid basal cell carcinoma syndrome is caused by a mutation (change) in the PTCH1 gene. Also called basal cell nevus syndrome, BCNS, Gorlin syndrome, and NBCCS.",
  "synonyms": [
    "basal cell nevus syndrome",
    "BCNS",
    "Gorlin syndrome",
    "NBCCS"
  ],
  "references": [
    "NCI Dictionary of Cancer Terms"
  ],
  "url": "https://mlchart.com/terminology/oncology/nevoid-basal-cell-carcinoma-syndrome/"
}

Record 11,783 of 17,721 in Oncology and biomedicine terminology (MLC-0111). Request the full dataset.