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Appendix Enterochromaffin Cell Serotonin-Producing Neuroendocrine Tumor

MedGen UID:
474402
Concept ID:
C3272769
Neoplastic Process
Synonyms: Appendix EC Cell Serotonin-Producing Carcinoid Tumor; Appendix EC Cell Serotonin-Producing NET; Appendix EC-Cell Serotonin-Producing Neuroendocrine Tumor; Appendix Enterochromaffin Cell Serotonin-Producing Carcinoid Tumor

Definition

A well differentiated neuroendocrine tumor arising from the wall of the appendix. It is characterized by the presence of neoplastic cells forming round solid nests and occasionally glandular structures. The majority of the cases are asymptomatic, and they are found incidentally in appendectomy specimens. The majority of the tumors are located in the distal end of the appendix and they are enterochromaffin-cell carcinoid tumors producing serotonin. Most cases show morphologic evidence of appendiceal wall and lymphatic vessel invasion by tumor cells. Despite the morphologic evidence of invasion, appendiceal carcinoid tumors only infrequently produce lymph node or distant metastases. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAppendix Enterochromaffin Cell Serotonin-Producing Neuroendocrine Tumor

Recent clinical studies

Etiology

Levy AD, Sobin LH
Radiographics 2007 Jan-Feb;27(1):237-57. doi: 10.1148/rg.271065169. PMID: 17235010

Diagnosis

Levy AD, Sobin LH
Radiographics 2007 Jan-Feb;27(1):237-57. doi: 10.1148/rg.271065169. PMID: 17235010

Prognosis

Rindi G, Inzani F, Solcia E
Endocrinol Metab Clin North Am 2010 Dec;39(4):713-27. doi: 10.1016/j.ecl.2010.08.009. PMID: 21095540
Levy AD, Sobin LH
Radiographics 2007 Jan-Feb;27(1):237-57. doi: 10.1148/rg.271065169. PMID: 17235010

Clinical prediction guides

Rindi G, Inzani F, Solcia E
Endocrinol Metab Clin North Am 2010 Dec;39(4):713-27. doi: 10.1016/j.ecl.2010.08.009. PMID: 21095540

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