raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:104076

Leiomyosarcoma of small intestine

Type
Disease
Group
Disorder
Status
active

Small bowel leiomyosarcoma is a rare type of small bowel malignancy, originating in the smooth muscle cells within the muscularis propria or the muscularis mucosa, most often found in the jejunum, and presenting with gastrointestinal bleeding and anemia and sometimes with other non-specific symptoms such as vomiting, nausea, abdominal pain and weakness and spreading to regional lymph nodes in 14% of cases.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enLeiomyosarcoma of small intestine
frLéiomyosarcome de l'instestin grêle
deLeiomyosarkom des Dünndarms
esLeiomiosarcoma de intestino delgado
itLeiomiosarcoma dell'intestino tenue
nlLeiomyosarcoom van dunne darm
ptLeiomiossarcoma do intestino curto
plMięsak gładkokomórkowy jelita cienkiego
csLeiomyosarkom tenkého střeva
ukЛейоміосаркома тонкої кишки
zh小肠平滑肌肉瘤
trİnce bağırsak leiomyosarkomu

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C17.0BTNTattributedvalidated
ICD-10C17.1BTNTattributedvalidated
ICD-10C17.2BTNTattributedvalidated
ICD-10C17.3BTNTattributedvalidated
ICD-10C17.8BTNTattributedvalidated
ICD-112B58.YNTBTindexvalidated
MONDO0003360Evalidated
UMLSC0920305Evalidated
GARD19852Evalidated
MedDRA10041127Evalidated

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

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