Small intestine leiomyosarcoma

Summary

Small intestine leiomyosarcoma (MONDO:0003360) is a disease and 5 clinical trials. Top therapeutic interventions include irinotecan hydrochloride, vismodegib, and ucn-01. A subtype of small intestinal sarcoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 5

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	small intestine leiomyosarcoma
Mondo ID	MONDO:0003360
Orphanet	104076
DOID	DOID:5271
ICD-11	389419378
NCIT	C7085
SNOMED CT	716651004
UMLS	C0920305
MedGen	183086
GARD	0019852
Anatomy (UBERON)	UBERON:0002108
Is cancer (heuristic)	no

Also known as: leiomyosarcoma of small bowel · leiomyosarcoma of small intestine · leiomyosarcoma of the small bowel · leiomyosarcoma of the small intestine · small bowel leiomyosarcoma · small intestinal leiomyosarcoma · small intestine leiomyosarcoma · smooth muscle connective tissue tumour

Disease family

This is a subtype of small intestinal sarcoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › digestive system disorder › intestinal disorder › intestinal neoplasm › small intestine neoplasm › small intestine cancer › small intestinal sarcoma › small intestine leiomyosarcoma

Related subtypes (1): small intestinal fibrosarcoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 5.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1	2
Not specified	2
PHASE1/PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01154452	PHASE1/PHASE2	COMPLETED	Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma
NCT00031681	PHASE1	COMPLETED	7-Hydroxystaurosporine and Irinotecan Hydrochloride in Treating Patients With Metastatic or Unresectable Solid Tumors or Triple Negative Breast Cancer (Currently Accruing Only Triple-negative Breast Cancer Patients Since 6/8/2007)
NCT00397384	PHASE1	COMPLETED	Erlotinib Hydrochloride and Cetuximab in Treating Patients With Advanced Gastrointestinal Cancer, Head and Neck Cancer, Non-Small Cell Lung Cancer, or Colorectal Cancer
NCT01793168	Not specified	RECRUITING	Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford
NCT00087191	Not specified	TERMINATED	EF5 and Motexafin Lutetium in Detecting Tumor Cells in Patients With Abdominal or Non-Small Cell Lung Cancer

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
IRINOTECAN HYDROCHLORIDE	4	1
VISMODEGIB	4	1
UCN-01	2	1
CHEMBL1236539	0	1
MOTEXAFIN LUTETIUM	-1	1