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Endocrine tumor

ORPHA877
Synonym(s) APUDoma
Carcinoid tumor
Neuroendocrine tumor
Prevalence Unknown
Inheritance Unknown
Age of onset Adult
ICD-10 -
OMIM -
UMLS -
MeSH -
MedDRA -

Summary

Endocrine tumours, also referred to as neuroendocrine tumours (NETs), are defined by a common phenotype which is characterized by the expression of general markers (neuron specific enolase, chromogranin, synaptophysin) and hormone secretion products. These tumours may be localized in any part of the body and are generally discovered in non-specific situations, i.e. not immediately suggestive of NETs (tests for inherited predisposition to tumours or for a clinical syndrome caused by abnormal hormone secretion). Prevalence of these tumours is 1 in 100 000. Among endodermal NETs, the most common localisations are the lungs, ileus, appendix, or rectum. Neuroectodermal NETs include medullary thyroid cancer, pheochromocytoma, and paraganglioma. Diagnosis of a NET should include extensive investigation for hormonal secretions and for a possible association with an inherited syndrome of tumour predisposition (multiple endocrine neoplasy, von Lippel-Hindau disease, neurofibromatosis (see these terms)). This syndrome should be distinguished from a metastatic extension, a combination of sporadic tumours, or a non-inherited multiple form of NETs. Histological differentiation and tumoral stage are important factors to determine the survival prognosis, which varies widely. The localisation of the NET is closely linked to the biological activity and the association of a familial syndrome, but also constitutes a factor of prognosis. Clinical approach is based on antitumoral and antisecretory treatments, but also on a specialized and multidisciplinary management.

Expert reviewer(s)

  • Pr Michel DUCREUX

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Detailed information

Clinical practice guidelines
  • EN (2012)
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