Neuroendocrine neoplasms
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), in the context of gastrointestinal pathology.[1][2][3]
The term is still used for neuroendocrine tumours of the lung - see carcinoid tumours.
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:
- WC:
- www:
Site specific
Lung
GI tract
WHO classification
Subtypes:[6]
- Neuroendocrine tumour G1.
- Neuroendocrine tumour G2.
- Neuroendocrine carcinoma, small cell type.
- Neuroendocrine carcinoma, large cell type.
- Mixed adenoneuroendocrine carcinoma.
Grading
Grading of NETs:[7]
- G1: < 2 mitoses/2 mm^2 or < 3% Ki-67.
- G2: > 2 mitoses/2 mm^2 or > 3% Ki-67 and < 20 mitoses/2 mm^2 or < 20% Ki-67.
- G3: > 20 mitoses/2 mm^2 or > 20% Ki-67.
Notes:
- Neuroendocrine carcinomas are G3 by definition.
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
- ↑ Chetty, R. (Apr 2008). "Requiem for the term 'carcinoid tumour' in the gastrointestinal tract?". Can J Gastroenterol 22 (4): 357-8. PMID 18414708.
- ↑ 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.
- ↑ 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.
- ↑ 4.0 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.
- ↑ URL: [http://path.upmc.edu/cases/case7.html http://path.upmc.edu/cases/case7.html}. Accessed on: 17 December 2011.
- ↑ 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.
- ↑ 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.