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Macrocystic lymphatic malformation

Disease definition

A rare common cystic lymphatic malformation characterized by a benign cystic lesion composed of dilated lymphatic channels. Macrocystic lesions consist of cysts larger than 1 cm in diameter. They usually present at birth or during the first years of life and most often occur in the head and neck region but may affect any site. Symptoms depend on the location and extent of the lesion. Infection, trauma, or intracystic hemorrhage can lead to lesional expansion. Malignant transformation does not occur.

ORPHA:79489

Classification level: Disorder
  • Synonym(s):
    • Cavernous lymphangioma
    • Cavernous lymphatic malformation
    • Macrocystic lymphangioma
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Neonatal
  • ICD-10: D18.1
  • OMIM: -
  • UMLS: C0205828
  • MeSH: -
  • GARD: 6010
  • MedDRA: -
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