raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:458792

Mixed macro and microcystic lymphatic malformation

Synonyms: Mixed cystic lymphangioma

Type
Clinical subtype
Group
Subtype of disorder
Inheritance
Not applicable
Age of onset
Infancy, Neonatal
Status
active

A rare common cystic lymphatic malformation characterized by a benign cystic lesion composed of dilated lymphatic channels. Mixed cystic lesions consist of cysts both larger (macrocystic) and smaller (microcystic) 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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enMixed macro and microcystic lymphatic malformationMixed cystic lymphangioma
frMalformation lymphatique mixte macro et microkystiqueLymphangiome kystique mixte
deGemischte makro- und mikrozystische lymphatische FehlbildungLymphatische Malformation, gemischte zystische · Gemischtes zystisches Lymphangiom
esMalformación linfática mixta macro y microquísticaLinfangioma quístico mixto
itMalformazione linfatica mista macro- e microcisticaLinfangioma cistico misto
nlGemengde macro- en microcystische lymfatische malformatieGemengde macro- en microcysteuze lymfatische malformatie · Gemengd cysteus lymfangioom · Gemengd cystisch lymfangioom
ptMalformação quística linfática mista
plMieszana malformacja limfatyczna torbielowataMieszany naczyniak limfatyczny torbielowaty
csSmíšená cystická lymfatická malformaceSmíšený cystický lymfangiom
ukЗмішана кістозна лімфатична мальформаціяЗмішана кістозна лімфангіома
zh混合性囊性淋巴畸形混合性囊性淋巴管瘤
trKarma kistik lenfatik malformasyonKarma kistik lenfanjiyom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D18.1NTBTattributedvalidated
MONDO0018717Evalidated
UMLSC5680000Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

  • ORPHA:458833Isolated lymphatic malformation
    rare developmental anomalies during embryogenesis; rare genetic diseases; rare circulatory system diseases; rare otorhinolaryngological diseases; rare surgical maxillo-facial diseases

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card