Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Neuroendocrine neoplasm
Synonyms
Definitions

A group of rare tumors characterized by predominantly neuroendocrine differentiation, potentially arising in most organs of the body, including the central nervous system, respiratory tract, larynx, gastrointestinal tract, thyroid, skin, breast, and urogenital system. The gastrointestinal tract and lungs are the most common primary tumor sites. Based on clinical behavior, histology, and proliferation rate, the tumors may be categorized as well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinomas. They may or may not be associated with clinical hormone hypersecretion syndromes.

ID

http://www.orpha.net/ORDO/Orphanet_877

definition

A group of rare tumors characterized by predominantly neuroendocrine differentiation, potentially arising in most organs of the body, including the central nervous system, respiratory tract, larynx, gastrointestinal tract, thyroid, skin, breast, and urogenital system. The gastrointestinal tract and lungs are the most common primary tumor sites. Based on clinical behavior, histology, and proliferation rate, the tumors may be categorized as well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinomas. They may or may not be associated with clinical hormone hypersecretion syndromes.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=877

has_age_of_onset

Adult

has_inheritance

Unknown

hasDbXref

UMLS:C0206754

MeSH:D018358

MedDRA:10052399

label

Neuroendocrine neoplasm

notation

Category

ORPHA:877

prefixIRI

ORDO:Orphanet_877

prefLabel

Neuroendocrine neoplasm

present_in

United States AND has_point_prevalence_average_value : 35.0 AND has_point_prevalence_range : 1-5 / 10 000

Norway AND has_annual_incidence_average_value : 3.2 AND has_annual_incidence_range : 1-9 / 100 000

United States AND has_annual_incidence_average_value : 5.25 AND has_annual_incidence_range : 1-9 / 100 000

Europe AND has_annual_incidence_average_value : 2.53 AND has_annual_incidence_range : 1-9 / 100 000

Worldwide AND has_point_prevalence_range : Unknown

subClassOf

http://www.orpha.net/ORDO/Orphanet_557492

http://www.orpha.net/ORDO/Orphanet_97978

http://www.orpha.net/ORDO/Orphanet_182130

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