Neuroendocrine neoplasms

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Neuroendocrine neoplasms, also neuroendocrine tumours, are a group of tumours whose behaviour varies from benign to malignant and are found in may different sites.

Carcinoid is a term whose use is discouraged.

These tumours arise from epithelium; thus, malignant tumours in this group are referred to as neuroendocrine carcinomas.

Use of the term carcinoid

Use of the term carcinoid is discouraged (in pathology reports).[1][2][3]

Carcinoid syndrome

Cause:

  • Neuroendocrine tumour -> hormone secretion (5-hydroxytryptamine + others).[4]

Classic pathologic finding:[4]

  • Fibrosis of cardiac valves (tricuspid and pulmonary).
    • Result TIPS = tricuspid insufficiency pulmonary stenosis.

Clinical features:[4]

  • Diarrhea, flushing and wheezing.

Microscopic - generic

Features:

  • Nests of cells.
  • Stippled chromatin AKA salt-and-pepper chromatin, coarse chromatin.
  • Classically subepithelial/mural.

Images:

Site specific

Lung

GI tract

WHO classification

Subtypes:[6]

  1. Neuroendocrine tumour G1.
  2. Neuroendocrine tumor G2.
  3. Neuroendocrine carcinoma, small cell type.
  4. Neuroendocrine carcinoma, large cell type.
  5. Mixed adenoneuroendocrine carcinoma.


Further reading:
Volante, M.; Righi, L.; Berruti, A.; Rindi, G.; Papotti, M. (Apr 2011). "The pathological diagnosis of neuroendocrine tumors: common questions and tentative answers.". Virchows Arch 458 (4): 393-402. doi:10.1007/s00428-011-1060-7. PMID 21344263.

Pancreatic

See also

References

  1. Chetty, R. (Apr 2008). "Requiem for the term 'carcinoid tumour' in the gastrointestinal tract?". Can J Gastroenterol 22 (4): 357-8. PMID 18414708.
  2. Klöppel, G.; Perren, A.; Heitz, PU. (Apr 2004). "The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification.". Ann N Y Acad Sci 1014: 13-27. PMID 15153416.
  3. Klöppel G (July 2003). "[Neuroendocrine tumors of the gastrointestinal tract]" (in German). Pathologe 24 (4): 287–96. doi:10.1007/s00292-003-0636-7. PMID 14513276.
  4. 4.0 4.1 4.2 Druce, M.; Rockall, A.; Grossman, AB. (May 2009). "Fibrosis and carcinoid syndrome: from causation to future therapy.". Nat Rev Endocrinol 5 (5): 276-83. doi:10.1038/nrendo.2009.51. PMID 19444261.
  5. URL: [http://path.upmc.edu/cases/case7.html http://path.upmc.edu/cases/case7.html}. Accessed on: 17 December 2011.
  6. Scoazec, JY.; Couvelard, A. (Apr 2011). "[The new WHO classification of digestive neuroendocrine tumors].". Ann Pathol 31 (2): 88-92. doi:10.1016/j.annpat.2011.01.001. PMID 21601112.