Macrocystic lymphatic malformation

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Macrocystic lymphatic malformation

ORPHA:79489Clinical subtype

Also called Cavernous lymphangioma · Cavernous lymphatic malformation · Macrocystic lymphangioma

What it is

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.

Key facts

Age of onset
Infancy, Neonatal
Inheritance
Not applicable
Classified as
Clinical subtype

ICD-10 codes

D18.1filed under a broader ICD-10 category — shared with 6 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

GARD 6010MONDO 0019328UMLS C0205828

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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