raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:247762

Lipoblastoma

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Childhood
Status
active

A rare soft tissue tumor characterized by a lobulated, localized (lipoblastoma) or diffuse (lipoblastomatosis) lesion resembling fetal adipose tissue, composed of mature and immature adipocytes. It is most commonly found during the first years of life and presents as a slowly growing, well circumscribed mass, which may compress adjacent structures, depending on the location. Malignant transformation or metastasis does not occur, while recurrences are described especially in lipoblastomatosis.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enLipoblastoma
frLipoblastome
deLipoblastom
esLipoblastoma
itLipoblastoma
nlLipoblastoom
ptLipoblastoma
plLipoblastoma
csLipoblastom
ukЛіпобластома
zh成脂细胞瘤
trLipoblastom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D17.9NTBTattributedvalidated
ICD-112E80.1Especificvalidated
MONDO0016611Evalidated
MeSHD062689Evalidated
UMLSC1260965Evalidated
GARD12015Evalidated
MedDRA10074547Evalidated

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):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

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

7

Sources for this card