Orphanet: Macrocystic lymphatic malformation

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

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.


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: -
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.