Difference between revisions of "Neuroendocrine tumour of the appendix"

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'''Neuroendocrine tumour of the appendix''' is a common tumour of the [[vermiform appendix]].
'''Neuroendocrine tumour of the appendix''' is a common tumour of the [[vermiform appendix]]. It is also known as '''appendiceal neuroendocrine tumour''', abbreviated '''appendiceal NET'''.
 
It was previously known as '''appendiceal [[carcinoid]]'''.


==General==
==General==
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*<1.0 cm - do not metastasize.
*<1.0 cm - do not metastasize.
*1.0-2.0 cm - rarely metastasize.
*1.0-2.0 cm - rarely metastasize.
Management:
*Simple resection, e.g. appendectomy, sufficient for most tumours <2 cm.<ref name=pmid25840530>{{Cite journal  | last1 = Nussbaum | first1 = DP. | last2 = Speicher | first2 = PJ. | last3 = Gulack | first3 = BC. | last4 = Keenan | first4 = JE. | last5 = Ganapathi | first5 = AM. | last6 = Englum | first6 = BR. | last7 = Tyler | first7 = DS. | last8 = Blazer | first8 = DG. | title = Management of 1- to 2-cm Carcinoid Tumors of the Appendix: Using the National Cancer Data Base to Address Controversies in General Surgery. | journal = J Am Coll Surg | volume = 220 | issue = 5 | pages = 894-903 | month = May | year = 2015 | doi = 10.1016/j.jamcollsurg.2015.01.005 | PMID = 25840530 }}</ref><ref name=pmid18338494>{{Cite journal  | last1 = Fornaro | first1 = R. | last2 = Frascio | first2 = M. | last3 = Sticchi | first3 = C. | last4 = De Salvo | first4 = L. | last5 = Stabilini | first5 = C. | last6 = Mandolfino | first6 = F. | last7 = Ricci | first7 = B. | last8 = Gianetta | first8 = E. | title = Appendectomy or right hemicolectomy in the treatment of appendiceal carcinoid tumors? | journal = Tumori | volume = 93 | issue = 6 | pages = 587-90 | month =  | year =  | doi =  | PMID = 18338494 }}</ref>


==Gross==
==Gross==
*Classically found in the tip of the appendix.
*Classically found in the tip of the appendix.
*Characteristic yellow cut surface (when fixed)
*Characteristic yellow cut surface post-[[fixation]].
*Circumscribed but not encapsulated
*Circumscribed but not encapsulated.
*Firm (due to desmoplasia)
*Firm (due to desmoplasia).
*Centred in the submucosa
*Centred in the submucosa.
*Nodules that do not usually cause erosion of the overlying mucosa.
*Nodules that do not usually cause erosion of the overlying mucosa.


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*Classically subepithelial/mural.  
*Classically subepithelial/mural.  
*Various growth patterns:
*Various growth patterns:
**Nested (insular)
**Nested (insular).
**Trabecular
**Trabecular.
**Palisading  
**Palisading.
**Ribbons, rosettes
**Ribbons, [[rosette]]s.
*Fibrous stroma in between cell groups.
*Fibrous stroma in between cell groups.
*Cytomorphology
*Cytomorphology:
**Monotonous appearance with scanty mitoses.
**Monotonous appearance with scanty mitoses.
**Round central nuclei
**Round central nuclei.
**Stippled chromatin [[AKA]] salt-and-pepper chromatin, coarse chromatin.
**Stippled chromatin ([[AKA]] ''salt-and-pepper chromatin'' and ''coarse chromatin'').
**Eosinophilic granular cytoplasm
**Eosinophilic granular cytoplasm.
 
DDx:
*[[Colorectal adenocarcinoma]].
*Adenocarcinoid.
*[[Crypt cell carcinoma]], also known as ''goblet cell carcinoid''.
*[[Metastasis|Metastatic]] [[adenocarcinoma]].
*Normal ganglion cells in the Meissner plexus (submucosa) and Auerbach plexus (located between the inner and outer layers of the muscularis propria).


===Special Types===
===Special Types===
*Tubular carcinoid  
*Tubular carcinoid.
**Neuroendocrine cells forming tubules (no cell nests)
**Neuroendocrine cells forming tubules (no cell nests).
**Some tubules can contain mucin  
**Some tubules can contain mucin.
**Can be confused with adenocarcinoma  
**Can be confused with adenocarcinoma.
**Features suggesting tubular carcinoid (over adenocarcinoma):
**Features suggesting tubular carcinoid (over adenocarcinoma):
***Arises from base of crypts, with no disruption of surface epithelium.
***Arises from base of crypts, with no disruption of surface epithelium.
***No associated epithelial precursor (no adenomatous change).
***No associated epithelial precursor (no adenomatous change).
***Neuroendocrine cytologic features, without prominent atypia  
***Neuroendocrine cytologic features, without prominent atypia.
***IHC (NE markers +ve)  
***IHC (NE markers +ve).
 
*Goblet cell carcinoid - dealt with in the article ''[[crypt cell carcinoma]]''.
*Goblet cell carcinoid aka [[Crypt cell carcinoma]]
*Signet-ring cells forming glandular structures.
*Signet-ring cells forming glandular structures,
*Possibly also with extra-cellular mucin.{{fact}}
*Possibly also with extra-cellular mucin  
 
DDx:
*[[Colorectal adenocarcinoma]].
*Adenocarcinoid.
*[[Crypt cell carcinoma]] (goblet cell carcinoid).
*Metastatic adenocarcinoma.
*Normal ganglion cells in the Meissner plexus (submucosa) and Auerbach plexus (located between the inner and outer layers of the muscularis propria).  


===Images===
===Images===
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*Chromogranin A -ve/+ve.
*Chromogranin A -ve/+ve.
*Synaptophysin +ve.
*Synaptophysin +ve.
*Keratin positive, but CK7/CK20 negative
*Keratin positive.{{fact}}
*S100 positive for appendix  
*S100 positive for appendix.{{fact}}
 
Others:
*CK7 and CK20 variable.<ref name=pmid22461652>{{Cite journal  | last1 = Matsukuma | first1 = KE. | last2 = Montgomery | first2 = EA. | title = Tubular carcinoids of the appendix: the CK7/CK20 immunophenotype can be a diagnostic pitfall. | journal = J Clin Pathol | volume = 65 | issue = 7 | pages = 666-8 | month = Jul | year = 2012 | doi = 10.1136/jclinpath-2011-200639 | PMID = 22461652 }}</ref>
 
==Sign out==
<pre>
Vermiform Appendix, Appendectomy:
    - Low-grade neuroendocrine tumour (carcinoid tumour), see comment.
    -- Margins clear.
    -- Please see synoptic report.
    - Perforated acute appendicitis with periappendicitis.
 
Comment:
The tumour stains as follows:
POSITIVE: AE1/AE3, chromogranin A, synaptophysin, CD56.
NEGATIVE: CK7, CK20, S100.
PROLIFERATION (Ki-67): <3%.
 
The low-grade neuroendocrine tumour (in the planes of section) is in the tip and
separate from the appendiceal perforation site/acute appendicitis.


See: ''''.
The specimen was submitted in total.
</pre>


==See also==
==See also==
*[[Vermiform appendix]].
*[[Vermiform appendix]].
*[[[[neuroendocrine tumours]].
*[[Neuroendocrine tumours]].


==References==
==References==
48,466

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