Neuroendocrine tumor of the appendix, well differentiated, low or intermediate grade

Summary

Neuroendocrine tumor of the appendix, well differentiated, low or intermediate grade (MONDO:0015066) is a cancer and 1 clinical trial. A subtype of neuroendocrine tumor of the colon, well differentiated, low or intermediate grade tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Phenotypes (HPO): 30
• Clinical trials: 1

Clinical features

Signs & symptoms

Clinical features (HPO)

30 HPO clinical features (Orphanet curated; top 30 by frequency):

HPO ID	Term	Frequency
HP:0006723	Intestinal carcinoid	Obligate (100%)
HP:0002017	Nausea and vomiting	Frequent (30-79%)
HP:0002574	Episodic abdominal pain	Frequent (30-79%)
HP:0004396	Poor appetite	Frequent (30-79%)
HP:0005249	Functional intestinal obstruction	Frequent (30-79%)
HP:0010676	Mechanical ileus	Frequent (30-79%)
HP:0011848	Abdominal colic	Frequent (30-79%)
HP:0012701	Bowel urgency	Frequent (30-79%)
HP:0030142	Abnormal bowel sounds	Frequent (30-79%)
HP:0030144	Hypoactive bowel sounds	Frequent (30-79%)
HP:0002019	Constipation	Occasional (5-29%)
HP:0002039	Anorexia	Occasional (5-29%)
HP:0002240	Hepatomegaly	Occasional (5-29%)
HP:0002730	Chronic noninfectious lymphadenopathy	Occasional (5-29%)
HP:0002910	Elevated circulating hepatic transaminase concentration	Occasional (5-29%)
HP:0003148	Elevated serum acid phosphatase	Occasional (5-29%)
HP:0004385	Protracted diarrhea	Occasional (5-29%)
HP:0030412	Ileal adenocarcinoma	Occasional (5-29%)
HP:0040276	Adenocarcinoma of the colon	Occasional (5-29%)
HP:0001579	Primary hypercortisolism	Very rare (<1-4%)
HP:0001962	Palpitations	Very rare (<1-4%)
HP:0002099	Asthma	Very rare (<1-4%)
HP:0002615	Hypotension	Very rare (<1-4%)
HP:0003144	Increased serum serotonin	Very rare (<1-4%)
HP:0005211	Midgut malrotation	Very rare (<1-4%)
HP:0010446	Tricuspid stenosis	Very rare (<1-4%)
HP:0011749	Adrenocorticotropic hormone excess	Very rare (<1-4%)
HP:0030148	Heart murmur	Very rare (<1-4%)
HP:0031499	Appendiceal mucinous neoplasm	Very rare (<1-4%)
HP:0100615	Ovarian neoplasm	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	neuroendocrine tumor of the appendix, well differentiated, low or intermediate grade
Mondo ID	MONDO:0015066
Orphanet	100079
ICD-11	1590340268
NCIT	C96422
SNOMED CT	725167001
UMLS	C3272767
MedGen	474400
GARD	0019754
Anatomy (UBERON)	UBERON:0001154
Is cancer (heuristic)	yes

Also known as: appendiceal NEN · appendiceal neuroendocrine neoplasm · appendiceal neuroendocrine tumor · appendiceal neuroendocrine tumour · appendix NET · appendix neuroendocrine tumor · appendix neuroendocrine tumour · appendix well differentiated endocrine tumor · appendix well differentiated endocrine tumor/carcinoma · appendix well differentiated endocrine tumour · NEN of appendix · well-differentiated neuroendocrine tumor of the appendix · well-differentiated neuroendocrine tumour of the appendix

Disease family

This is a subtype of neuroendocrine tumor of the colon, well differentiated, low or intermediate grade tumor. 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 › digestive system neuroendocrine neoplasm › digestive system neuroendocrine tumor, grade 1/2 › neuroendocrine tumor of the colon, well differentiated, low or intermediate grade tumor › neuroendocrine tumor of the appendix, well differentiated, low or intermediate grade

Related subtypes (2): colonic L-cell glucagon-like peptide producing tumor, colon neuroendocrine tumor G1

Subtypes (4): appendix neuroendocrine tumor G1, classic neuroendocrine tumor of appendix, goblet cell carcinoma, appendix L-cell glucagon-like peptide-producing neuroendocrine tumor

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: 1.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT05734430	Not specified	RECRUITING	Genetics of Appendix Cancer Study