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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Mucinous cystadenoma of the appendix -- intermed mag.jpg | |||
| Width = | |||
| Caption = Mucinous cystadenoma of the [[appendix]]. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = mucin, +/- nuclear atypia | |||
| Subtypes = see ''LMDDx'' | |||
| LMDDx = [[mucinous adenocarcinoma]], entrapped mucin, mucinous cystadenoma | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[appendix]] | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = dependent on stage and presence/absence of nuclear atypia | |||
| Other = | |||
| ClinDDx = other appendiceal tumours, metastatic tumours | |||
| Tx = right hemicolectomy or appendectomy (?) | |||
}} | |||
{{ Infobox external links | |||
| Name = Mucinous tumours of the appendix | |||
| EHVSC = 10183 | |||
| pathprotocols = | |||
| wikipedia = | |||
| pathoutlines = | |||
}} | |||
'''Mucinous tumours of the appendix''' are uncommon tumours of the [[vermiform appendix]] that can have a benign or [[malignant]] behaviour. | |||
'''Benign appendiceal [[mucocele]]''' and '''appendiceal mucocele''' redirect here. | |||
==General== | |||
*There are many classifications and they are controversial.<ref name=pmid21970481>{{Cite journal | last1 = Panarelli | first1 = NC. | last2 = Yantiss | first2 = RK. | title = Mucinous neoplasms of the appendix and peritoneum. | journal = Arch Pathol Lab Med | volume = 135 | issue = 10 | pages = 1261-8 | month = Oct | year = 2011 | doi = 10.5858/arpa.2011-0034-RA | PMID = 21970481 }}</ref> | |||
**The controversy centres on whether to call all mucinous tumours outside of the appendix ''adenocarcinoma'' - regardless of whether they have [[nuclear atypia|atypia]] & show invasion. | |||
*Many potential pathways for extra-appendiceal spread and peritoneal mucin deposition (pseudomyxoma peritonei) | |||
**A mucinous neoplasm ruptures the appendix due to expansion by excessive mucin | |||
***Could occur with either a benign or malignant neoplasm. | |||
**Malignant mucinous glands infiltrate through the appendiceal wall or enter the lymphatics and spread throughout the peritoneum. | |||
***Would require a malignant neoplasm. | |||
*''Panarelli and Yantiss'' created a nice summary table - that compares the classifications - see: [http://www.archivesofpathology.org/action/showFullPopup?id=i1543-2165-135-10-1261-t01&doi=10.5858%2Farpa.2011-0034-RA comparison of classifications (archivesofpathology.org)].<ref name=pmid21970481/> | |||
*In women - an ovarian primary must be excluded. | |||
**Concurrent ''bilateral'' ovarian tumours suggests the tumour originated from the appendix and spread to the ovaries. | |||
*The 2003 classification by Misdraji ''et al.''<ref name=pmid12883241>{{cite journal |author=Misdraji J, Yantiss RK, Graeme-Cook FM, Balis UJ, Young RH |title=Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases |journal=Am. J. Surg. Pathol. |volume=27 |issue=8 |pages=1089–103 |year=2003 |month=August |pmid=12883241 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=27&issue=8&spage=1089}}</ref> is a good starting point if one is unfamilar with the topic, as it is the least complicated. | |||
===Misdraji classification of 2003=== | |||
*Benign - ''low grade mucinous tumour''. | |||
*Borderline - ''mucinous tumour of uncertain malignant potential'' or ''borderline mucinous tumour''. | |||
*Malignant - ''[[mucinous adenocarcinoma]]''. | |||
Five year survival (in a series of 107 cases) as per Misdraji classification:<ref name=pmid12883241/> | |||
{| class="wikitable sortable" | |||
!Tumour | |||
!Five year survival | |||
|- | |||
|LAMN | |||
|100% | |||
|- | |||
|LAMN extra-appendiceal spread | |||
|86% | |||
|- | |||
|MACA | |||
|44% | |||
|- | |||
|} | |||
*LAMN = low-grade appendiceal mucinous neoplasm. | |||
*LAMN extra-appendiceal = low-grade appendiceal mucinous neoplasm with extra-appendiceal spread. | |||
*MACA = mucinous adenocarcinoma of the appendix. | |||
===World Health Organization classification=== | |||
WHO classification: | |||
*Adenoma with low-grade dysplasia. | |||
*Adenoma with high-grade dysplasia. | |||
*Low-grade invasive mucinous adenocarcinoma | |||
**Confined to the appendiceal wall. | |||
**Outside of the appendix. | |||
*High-grade invasive mucinous adenocarcinoma. | |||
===Comparison between Misdraji and WHO classification=== | |||
Adapted from ''Panarelli and Yantiss'':<ref name=pmid21970481>{{Cite journal | last1 = Panarelli | first1 = NC. | last2 = Yantiss | first2 = RK. | title = Mucinous neoplasms of the appendix and peritoneum. | journal = Arch Pathol Lab Med | volume = 135 | issue = 10 | pages = 1261-8 | month = Oct | year = 2011 | doi = 10.5858/arpa.2011-0034-RA | PMID = 21970481 }}</ref> | |||
{| class="wikitable sortable" | |||
! Stage | |||
! Cytologic dysplasia | |||
! Misdraji | |||
! World Health Organization | |||
|- | |||
| Confined to the mucosa | |||
| low-grade | |||
| low-grade appendiceal mucinous neoplasm (LAMN) | |||
| mucinous adenoma, negative for high-grade dysplasia | |||
|- | |||
| Confined to the mucosa | |||
| high-grade | |||
| non-invasive mucinous cystadenocarcinoma of the appendix | |||
| mucinous adenoma with high-grade dysplasia | |||
|- | |||
| At least into the submucosa, confined to the appendix | |||
| low-grade | |||
| low-grade appendiceal mucinous neoplasm (LAMN) | |||
| invasive mucinous adenocarcinoma, low-grade | |||
|- | |||
| At least into the submucosa, confined to the appendix | |||
| high-grade | |||
| mucinous adenocarcinoma of the appendix (MACA) | |||
| invasive mucinous adenocarcinoma, high-grade | |||
|- | |||
| Extra-appendiceal spread | |||
| low-grade | |||
| low-grade appendiceal mucinous neoplasm (LAMN) | |||
| invasive mucinous adenocarcinoma, low-grade | |||
|- | |||
| Extra-appendiceal spread | |||
| high-grade | |||
| mucinous adenocarcinoma of the appendix (MACA) | |||
| invasive mucinous adenocarcinoma, high-grade | |||
|} | |||
==Microscopic== | |||
===Low-grade appendiceal mucinous neoplasm=== | |||
*[[AKA]] ''benign mucinous tumour of the appendix''. | |||
Microscopic: | |||
*Single layer of epithelium with ''tufts''. | |||
**Vaguely resemble ''serrations'', i.e. the ''saw-tooth'' pattern in hyperplastic polyps of the colon. | |||
*Mucin contained (inside appendix only). | |||
** mucin extravasation into the appendiceal wall does not upgrade the tumour. | |||
*No marked nuclear atypia. | |||
Note: | |||
*May be deceptively bland appearing from a cytologic perspective. | |||
====Images==== | |||
www: | |||
*[http://www.nature.com/modpathol/journal/v17/n12/fig_tab/3800212f1.html LAMN - low mag. (nature.com)].<ref name=pmid15354187/> | |||
*[http://www.nature.com/modpathol/journal/v17/n12/fig_tab/3800212f3.html LAMN - high mag. (nature.com)].<ref name=pmid15354187>{{Cite journal | last1 = Misdraji | first1 = J. | last2 = Burgart | first2 = LJ. | last3 = Lauwers | first3 = GY. | title = Defective mismatch repair in the pathogenesis of low-grade appendiceal mucinous neoplasms and adenocarcinomas. | journal = Mod Pathol | volume = 17 | issue = 12 | pages = 1447-54 | month = Dec | year = 2004 | doi = 10.1038/modpathol.3800212 | PMID = 15354187 }}</ref> | |||
*[http://pathlabmed.typepad.com/surgical_pathology_and_la/2012/02/digital-case-simple-mucocele-of-the-appendix.html Appendiceal mucocele (pathlabmed.typepad.com)]. | |||
<gallery> | |||
Image: Mucinous cystadenoma of the appendix -- low mag.jpg | Mucinous cystadenoma - low mag. (WC) | |||
Image: Mucinous cystadenoma of the appendix -- intermed mag.jpg | Mucinous cystadenoma - intermed. mag. (WC) | |||
Image: Mucinous cystadenoma of the appendix -- high mag.jpg | Mucinous cystadenoma - high mag. (WC) | |||
Image: Mucinous cystadenoma of the appendix - alt -- high mag.jpg | Mucinous cystadenoma - high mag. (WC) | |||
Image: Mucinous cystadenoma of the appendix -- very high mag.jpg | Mucinous cystadenoma - very high mag. (WC) | |||
Image: Appendix LowGradeMucinousNeoplasm MucinousNeoplasm LowGrade LP PA.JPG|Low Grade Appendiceal Mucinous Neoplasm - low power (SKB) | |||
Image: Appendix LowGradeMucinousNeoplasm LP-2 PA.JPG|Low Grade Appendiceal Mucinous Neoplasm - mucin extravasation into the appendiceal wall- low power (SKB) | |||
Image: Appendix LowGradeMucinousNeoplasm LP - Copy PA.JPG|Low Grade Appendiceal Mucinous Neoplasm - mucin extravasation into the appendiceal wall - low power (SKB) | |||
Image: Appendix LowGradeMucinousNeoplasm MucinousNeoplasm LowGrade HP (2) PA.JPG|Low Grade Appendiceal Mucinous Neoplasm - medium power (SKB) | |||
Image: Appendix LowGradeMucinousNeoplasm MucinousNeoplasm LowGrade HP PA.JPG|Low Grade Appendiceal Mucinous Neoplasm - high power (SKB) | |||
Image: Appendix LowGradeMucinousNeoplasm LP 14BR***.jpg|Low Grade Appendiceal Mucinous Neoplasm - low power (SKB) | |||
Image: Appendix LowGradeMucinousNeoplasm MP 14BR***.jpg|Low Grade Appendiceal Mucinous Neoplasm - medium power (SKB) | |||
</gallery> | |||
===Low-grade appendiceal mucinous neoplasm with extra-appendiceal spread=== | |||
*[[AKA]] ''mucinous borderline tumour of the appendix''. | |||
Microscopic: | |||
*Same as LAMN but mucin outside of the appendix. | |||
*Cells in mucin, i.e. cellular mucin. | |||
===Mucinous adenocarcinoma of the appendix=== | |||
*[[AKA]] ''malignant mucinous tumour of the appendix''. | |||
Microscopic: | |||
*Marked nuclear pleomorphism. | |||
*Invasion into the appendiceal wall. | |||
==Sign out== | |||
===LAMN=== | |||
<pre> | |||
VERMIFORM APPENDIX, APPENDECTOMY: | |||
- LOW-GRADE APPENDICEAL MUCINOUS NEOPLASM. | |||
-- NEGATIVE FOR EXTRA-APPENDICEAL MUCIN. | |||
-- MARGINS NEGATIVE FOR MUCINOUS NEOPLASM. | |||
- ACUTE APPENDICITIS. | |||
- ACUTE PERIAPPENDICITIS. | |||
</pre> | |||
<pre> | |||
VERMIFORM APPENDIX AND CECUM, APPENDECTOMY WITH CECAL CUFF: | |||
- LOW-GRADE APPENDICEAL MUCINOUS NEOPLASM (MUCINOUS CYSTADENOMA). | |||
- MARGINS NEGATIVE FOR MUCINOUS NEOPLASM. | |||
COMMENT: | |||
No extra-appendiceal mucin is identified. There is no invasion into the appendiceal wall. | |||
</pre> | |||
===WHO=== | |||
<pre> | |||
APPENDIX, APPENDECTOMY: | |||
- APPENDICEAL MUCINOUS CYSTADENOMA WITH EXTENSIVE CALCIFICATION. | |||
-- NEGATIVE FOR HIGH-GRADE DYSPLASIA. | |||
- NEGATIVE FOR MALIGNANCY. | |||
</pre> | |||
==See also== | |||
*[[Vermiform appendix]]. | |||
*[[Mucinous carcinoma]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Vermiform appendix]] |
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