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von Hippel-Lindau syndrome

Term · Oncology and biomedicine · MLC-T-ONC-009313

A rare, inherited disorder that causes tumors and cysts to grow in certain parts of the body, including the brain, spinal cord, eyes, inner ear, adrenal glands, pancreas, kidney, and reproductive tract. The tumors are usually benign (not cancer), but some may be malignant (cancer). Patients with von Hippel-Lindau syndrome have an increased risk of certain types of cancer, especially kidney cancer and pancreatic cancer. The signs and symptoms of von Hippel-Lindau syndrome usually do not appear until young adulthood. It is caused by a mutation (change) in the VHL gene. Also called VHL disease, VHL syndrome, and von Hippel-Lindau disease.

Table 1. Record
IdentifierMLC-T-ONC-009313
FieldOncology and biomedicine
SynonymsVHL disease; VHL syndrome; von Hippel-Lindau disease
ReferencesNCI Dictionary of Cancer Terms
Record as JSON
{
  "id": "MLC-T-ONC-009313",
  "term": "von Hippel-Lindau syndrome",
  "field": "Oncology and biomedicine",
  "definition": "A rare, inherited disorder that causes tumors and cysts to grow in certain parts of the body, including the brain, spinal cord, eyes, inner ear, adrenal glands, pancreas, kidney, and reproductive tract. The tumors are usually benign (not cancer), but some may be malignant (cancer). Patients with von Hippel-Lindau syndrome have an increased risk of certain types of cancer, especially kidney cancer and pancreatic cancer. The signs and symptoms of von Hippel-Lindau syndrome usually do not appear until young adulthood. It is caused by a mutation (change) in the VHL gene. Also called VHL disease, VHL syndrome, and von Hippel-Lindau disease.",
  "synonyms": [
    "VHL disease",
    "VHL syndrome",
    "von Hippel-Lindau disease"
  ],
  "references": [
    "NCI Dictionary of Cancer Terms"
  ],
  "url": "https://mlchart.com/terminology/oncology/von-hippel-lindau-syndrome/"
}

Record 17,334 of 17,721 in Oncology and biomedicine terminology (MLC-0111). Request the full dataset.