Difference between revisions of "Mucinous tumours of the appendix"

From Libre Pathology
Jump to navigation Jump to search
(split out)
m (→‎General: fix typo)
 
(16 intermediate revisions by 3 users not shown)
Line 1: Line 1:
{{ 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
{{ Infobox external links
| Name          = Mucinous tumours of the appendix
| Name          = Mucinous tumours of the appendix
Line 11: Line 42:
==General==
==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>
*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 atypia & show invasion.
**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.
*''Panarelli and Yantiss'' created a nice summary table - that compare 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/>
*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.
*In women - an ovarian primary must be excluded.
**Concurrent ''bilateral'' ovarian tumours suggests the tumour originated from the appendix and spread to the ovaries.
**Concurrent ''bilateral'' ovarian tumours suggests the tumour originated from the appendix and spread to the ovaries.
*[[Onlinepathology]] prefers the classification of Misdraji,<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> as it is the least complicated
*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===
===Misdraji classification of 2003===
*Benign - ''low grade mucinous tumour''.
*Benign - ''low grade mucinous tumour''.
*Borderline - ''mucinous tumour of uncertain malignant potential'' or ''borderline mucinous tumour''.
*Borderline - ''mucinous tumour of uncertain malignant potential'' or ''borderline mucinous tumour''.
Line 97: Line 133:
**Vaguely resemble ''serrations'', i.e. the ''saw-tooth'' pattern in hyperplastic polyps of the colon.
**Vaguely resemble ''serrations'', i.e. the ''saw-tooth'' pattern in hyperplastic polyps of the colon.
*Mucin contained (inside appendix only).
*Mucin contained (inside appendix only).
** mucin extravasation into the appendiceal wall does not upgrade the tumour.
*No marked nuclear atypia.
*No marked nuclear atypia.


Line 102: Line 139:
*May be deceptively bland appearing from a cytologic perspective.
*May be deceptively bland appearing from a cytologic perspective.


Images:
====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/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://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)].
*[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===
===Low-grade appendiceal mucinous neoplasm with extra-appendiceal spread===
Line 126: Line 179:
VERMIFORM APPENDIX, APPENDECTOMY:
VERMIFORM APPENDIX, APPENDECTOMY:
- LOW-GRADE APPENDICEAL MUCINOUS NEOPLASM.
- LOW-GRADE APPENDICEAL MUCINOUS NEOPLASM.
-- NEGATIVE FOR EXTRA-APPENDICEAL MUCIN.
-- MARGINS NEGATIVE FOR MUCINOUS NEOPLASM.
- ACUTE APPENDICITIS.
- ACUTE APPENDICITIS.
- ACUTE PERIAPPENDICITIS.  
- ACUTE PERIAPPENDICITIS.  
Line 139: Line 194:
</pre>
</pre>


===WHO===
<pre>
<pre>
APPENDIX, APPENDECTOMY:
APPENDIX, APPENDECTOMY:

Latest revision as of 22:10, 8 June 2015

Mucinous tumours of the appendix
Diagnosis in short

Mucinous cystadenoma of the appendix. H&E stain.
LM mucin, +/- nuclear atypia
Subtypes see LMDDx
LM DDx mucinous adenocarcinoma, entrapped mucin, mucinous cystadenoma
Site appendix
Prevalence uncommon
Prognosis dependent on stage and presence/absence of nuclear atypia
Clin. DDx other appendiceal tumours, metastatic tumours
Treatment right hemicolectomy or appendectomy (?)
Mucinous tumours of the appendix
External resources
EHVSC 10183

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.[1]
    • The controversy centres on whether to call all mucinous tumours outside of the appendix adenocarcinoma - regardless of whether they have 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: comparison of classifications (archivesofpathology.org).[1]
  • 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.[2] 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:[2]

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:[1]

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:

  • Mucinous cystadenoma - low mag. (WC)

  • Mucinous cystadenoma - intermed. mag. (WC)

  • Mucinous cystadenoma - high mag. (WC)

  • Mucinous cystadenoma - high mag. (WC)

  • Mucinous cystadenoma - very high mag. (WC)

  • Low Grade Appendiceal Mucinous Neoplasm - low power (SKB)

  • Low Grade Appendiceal Mucinous Neoplasm - mucin extravasation into the appendiceal wall- low power (SKB)

  • Low Grade Appendiceal Mucinous Neoplasm - mucin extravasation into the appendiceal wall - low power (SKB)

  • Low Grade Appendiceal Mucinous Neoplasm - medium power (SKB)

  • Low Grade Appendiceal Mucinous Neoplasm - high power (SKB)

  • Low Grade Appendiceal Mucinous Neoplasm - low power (SKB)

  • Low Grade Appendiceal Mucinous Neoplasm - medium power (SKB)

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

VERMIFORM APPENDIX, APPENDECTOMY:
- LOW-GRADE APPENDICEAL MUCINOUS NEOPLASM.
-- NEGATIVE FOR EXTRA-APPENDICEAL MUCIN.
-- MARGINS NEGATIVE FOR MUCINOUS NEOPLASM.
- ACUTE APPENDICITIS.
- ACUTE PERIAPPENDICITIS. 

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.

WHO

APPENDIX, APPENDECTOMY:
- APPENDICEAL MUCINOUS CYSTADENOMA WITH EXTENSIVE CALCIFICATION.
-- NEGATIVE FOR HIGH-GRADE DYSPLASIA.
- NEGATIVE FOR MALIGNANCY.

See also

References

  1. ↑ 1.0 1.1 1.2 Panarelli, NC.; Yantiss, RK. (Oct 2011). "Mucinous neoplasms of the appendix and peritoneum.". Arch Pathol Lab Med 135 (10): 1261-8. doi:10.5858/arpa.2011-0034-RA. PMID 21970481.
  2. ↑ 2.0 2.1 Misdraji J, Yantiss RK, Graeme-Cook FM, Balis UJ, Young RH (August 2003). "Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases". Am. J. Surg. Pathol. 27 (8): 1089–103. PMID 12883241. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=27&issue=8&spage=1089.
  3. ↑ 3.0 3.1 Misdraji, J.; Burgart, LJ.; Lauwers, GY. (Dec 2004). "Defective mismatch repair in the pathogenesis of low-grade appendiceal mucinous neoplasms and adenocarcinomas.". Mod Pathol 17 (12): 1447-54. doi:10.1038/modpathol.3800212. PMID 15354187.
Retrieved from "https://librepathology.org/w/index.php?title=Mucinous_tumours_of_the_appendix&oldid=38445"