Vermiform appendix

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The vermiform appendix, usually just appendix, is a little thingy that is attached to the cecum. Taking it out is the bread 'n butter of general surgery.

The appendix is a vestigial structure that is thought to have arisen from a larger cecum. Larger cecae are often seen in herbivores and thought to facilitate better digestion of plant matter.[1]

Normal

Normal vermiform appendix

General

Gross

  • Shiny serosal surface.
    • No exudate.
  • Small diameter.

Microscopic

Features:

  • +/-Lymphoid hyperplasia.

Negatives:

  • No neutrophils in the muscularis propria.
  • No lesion in appendiceal tip.
  • No serosal inflammation.
  • No organisms in the appendiceal lumen, e.g. Enterobius vermicularis.

Negative appendectomy

General

Gross

See normal vermiform appendix.

Microscopic

See normal vermiform appendix.

Notes:

Sign out

VERMIFORM APPENDIX, APPENDECTOMY:
- APPENDIX NEGATIVE FOR ACUTE APPENDICITIS AND NEGATIVE FOR ACUTE PERIAPPENDICITIS. 
VERMIFORM APPENDIX, APPENDECTOMY:
- APPENDIX WITH LYMPHOID HYPERPLASIA AND FOCAL MUCOSAL EROSIONS.
- NEGATIVE FOR ACUTE APPENDICITIS.
- NEGATIVE FOR ACUTE PERIAPPENDICITIS. 

Micro

The sections show appendiceal wall with focal mucosa erosions and several intraluminal neutrophil clusters. Lymphoid hyperplasia is present. Fecal material is present within the lumen of the appendix.

There are no neutrophils within the muscularis propria. There is no serositis. There is no distortion of the crypt architecture. No granulomas are identified. No cryptitis is identified.

Inflammatory pathologies

Acute appendicitis

General

  • Bread 'n butter of general surgery.
  • Interesting factoid: appendicitis is considered protective against ulcerative colitis.[2][3]

Short clinical DDx:

  • GI tract:
  • Gynecologic tract:
    • Ectopic pregnancy.
    • Ruptured ovarian cyst.
    • Ovarian torsion.
      • Pelvic inflammatory disease.

Gross

Features:

  • Serosal surface dull.
  • May be perforated (best determined on gross).
  • +/-Fibrinous exudate.

Note:

  • Normal diameter of appendix (based on CT): 6.6 +/- 1.5 mm.[4]
    • Similar numbers are found in another study.[5]

Microscopic

Features:

  • Neutrophils in the muscularis propria - key feature.
  • +/- Vascular thrombosis (and necrosis) - known as gangrenous appendicitis.[6]
  • +/- Findings suggestive of etiology - usu. absent:

Images:

DDx

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VERMIFORM APPENDIX, APPENDECTOMY:
- ACUTE APPENDICITIS.
- ACUTE PERIAPPENDICITIS. 
VERMIFORM APPENDIX, APPENDECTOMY:
- GANGRENOUS APPENDICITIS.
- ACUTE PERIAPPENDICITIS. 

Micro

Gangrenous appendicitis

The sections shows appendiceal wall with marked acute transmural inflammation and necrotic appendiceal wall with large collections of neutrophils. Several medium-sized blood vessels are thrombosed. A thick layer of neutrophils cover the serosa aspect.

Adenovirus appendicitis

General

  • Rare type of appendicitis in children.
  • Presents as run-of-the-mill acute appendicitis.
  • Caused by Adenovirus.

Microscopic

Features:[8]

  • Lymphoid hyperplasia - key feature.
  • +/-Adenovirus inclusions; "smudge cells".

Notes:

  • The classic finding of appendicitis (neutrophils infiltrating into the muscularis propria) may be absent.[8]

Image:

IHC

  • Adenovirus +ve = diagnostic.

Enterobius vermicularis

General

  • May be found in the appendix.
  • The incidence is higher in normal appendices than inflamed ones.[9][10]

Microscopic

Features:

Granulomatous appendicitis

General

Most common cause:

  • Yersinia appendicitis.[11]

DDx:[12]

  • Yersinia appendicitis.[11]
    • Yersinia = gram negative rod (red on Gram stain).
    • "Safety pin"-like appearance[13] - approximately 0.5 micrometers diameter x 2 micrometers length.
  • Other micro-organism (TB, fungus).
  • Crohn's disease.
  • Sarcoidosis.
  • Foreign body reaction.
  • Interval (delayed) appendectomy.
    • Approximately 60% of delayed appendectomies have granulomas.[14]

Microscopic

Features:

  • Granulomas.
  • +/-"Safety pin"-like organisms (Yersinia).

Image(s):

Inflammatory bowel disease

See Inflammatory bowel disease.

Periappendicitis

General

Definition: inflammation of tissues around the (vermiform) appendix.[15]

  • May be seen in association of appendicitis or alone.
    • With appendicitis it is suggestive of perforation.
    • Without concurrent appendicitis it is suggestive of another abdominal pathology.[16][17]

Microscopic

Features:

  • Acute inflammation of the serosa.

Tumours of the appendix

Adenocarcinoma

Mucinous tumours of the appendix

Benign appendiceal mucocele redirects here.

General

  • Classification is controversial.
    • The controversy centres on whether to call all mucinous tumours outside of the appendix adenocarcinoma - regardless of whether they have atypia & show invasion.
  • In women - an ovarian primary must be excluded.
    • Concurrent bilateral ovarian tumours suggests the tumour originated from the appendix and spread to the ovaries.

Classification:[18]

  • 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):[18]

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.

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).
  • No marked nuclear atypia.

Note:

  • May be deceptively bland appearing from a cytologic perspective.

Images:

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.

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LAMN

VERMIFORM APPENDIX, APPENDECTOMY:
- LOW-GRADE APPENDICEAL MUCINOUS NEOPLASM.
- ACUTE APPENDICITIS.
- ACUTE PERIAPPENDICITIS. 

Goblet cell carcinoid

General

  • Rare appendiceal tumour that typically has an aggressive course vis-a-vis other appendiceal carcinoids.[20]
  • Mixed (biphasic) tumour with endocrine and exocrine features.

Microscopic

Features:[21]

  • Mixed neuroendocrine-nonneuroendocrine tumour;[22] features of both carcinoid and adenocarcinoma.[21]
    • Archictecture: cells arranged in nests or clusters without a lumen.
    • Location: deep to the intestinal crypts (crypts of Lieberkühn); usually do not involve the mucosa.
    • Cytoplasm distended with mucin.
    • DNA: crescentic nucleus (similar to in signet-ring cells).
      • +/-Multinucleation.
      • +/-High mitotic rate.
      • Usually minimal nuclear atypia.

Images:

Stains

  • Mucin stains +ve:
    • Mucicarmine, perodic acid-Schiff diastase (PAS-D), alician blue.

IHC

  • Classic neuroendocrine markers:
    • Synaptophysin +ve.
    • Chromogranin +ve.
  • S100 +ve.
  • NSE +ve.
  • Serotonin +ve.

Keratins:

  • Usually CK20 +ve > CK7 +ve.
  • CEA +ve (membrane).

Notes:

  • Nice review of stains in Pahlavan and Kanthan.[21]

Neuroendocrine tumour of the appendix

  • Previously known as appendiceal carcinoid.
  • AKA appendiceal neuroendocrine tumour, abbreviated appendiceal NET.

General

  • Most common tumour of the appendix.[23]

Size matters in appendiceal NETs:[24]

  • <1.0 cm - do not metastasize.
  • 1.0-2.0 cm - rarely metastasize.

Microscopic

Features:

IHC

Features:

  • Chromogranin A -ve/+ve.
  • Synaptophysin +ve.

See: neuroendocrine tumours.

See also

References

  1. Dawkins, R. (2009). The Greatest Show on Earth: The Evidence for Evolution (1st ed.). Free Press. pp. 115. ISBN 978-1416594789.
  2. Beaugerie, L.; Sokol, H. (Aug 2009). "Appendicitis, not appendectomy, is protective against ulcerative colitis, both in the general population and first-degree relatives of patients with IBD.". Inflamm Bowel Dis. doi:10.1002/ibd.21064. PMID 19685454.
  3. Timmer, A.; Obermeier, F. (2009). "Reduced risk of ulcerative colitis after appendicectomy.". BMJ 338: b225. PMID 19273505.
  4. Charoensak, A.; Pongpornsup, S.; Suthikeeree, W. (Dec 2010). "Wall thickness and outer diameter of the normal appendix in adults using 64 slices multidetector CT.". J Med Assoc Thai 93 (12): 1437-42. PMID 21344807.
  5. Huwart, L.; El Khoury, M.; Lesavre, A.; Phan, C.; Rangheard, AS.; Bessoud, B.; Menu, Y. (Mar 2007). "[What is the thickness of the normal appendix on MDCT?].". J Radiol 88 (3 Pt 1): 385-9. PMID 17457270.
  6. URL: http://emedicine.medscape.com/article/363818-overview. Accessed on: 21 June 2010.
  7. Stangl, PC.; Herbst, F.; Birner, P.; Oberhuber, G. (Apr 2002). "Crohn's disease of the appendix.". Virchows Arch 440 (4): 397-403. doi:10.1007/s004280100532. PMID 11956821.
  8. 8.0 8.1 Grynspan D, Rabah R (2008). "Adenoviral appendicitis presenting clinically as acute appendicitis". Pediatr. Dev. Pathol. 11 (2): 138–41. doi:10.2350/07-06-0299.1. PMID 17990936.
  9. 9.0 9.1 Wiebe, BM. (Mar 1991). "Appendicitis and Enterobius vermicularis.". Scand J Gastroenterol 26 (3): 336-8. PMID 1853157.
  10. 10.0 10.1 Dahlstrom, JE.; Macarthur, EB. (Oct 1994). "Enterobius vermicularis: a possible cause of symptoms resembling appendicitis.". Aust N Z J Surg 64 (10): 692-4. PMID 7945067.
  11. 11.0 11.1 Lamps LW, Madhusudhan KT, Greenson JK, et al. (April 2001). "The role of Yersinia enterocolitica and Yersinia pseudotuberculosis in granulomatous appendicitis: a histologic and molecular study". Am. J. Surg. Pathol. 25 (4): 508–15. PMID 11257626.
  12. http://granuloma.homestead.com/appendicitis.html
  13. URL: http://www.cdc.gov/ncidod/dvbid/plague/p1.htm. Accessed on: 30 June 2011.
  14. Guo, G.; Greenson, JK. (Aug 2003). "Histopathology of interval (delayed) appendectomy specimens: strong association with granulomatous and xanthogranulomatous appendicitis.". Am J Surg Pathol 27 (8): 1147-51. PMID 12883248.
  15. URL: http://www.medilexicon.com/medicaldictionary.php?t=66889. Accessed on: 1 June 2011.
  16. Fink, AS.; Kosakowski, CA.; Hiatt, JR.; Cochran, AJ. (Jun 1990). "Periappendicitis is a significant clinical finding.". Am J Surg 159 (6): 564-8. PMID 2349982.
  17. O'Neil, MB.; Moore, DB. (Sep 1977). "Periappendicitis: Clinical reality or pathologic curiosity?". Am J Surg 134 (3): 356-7. PMID 900337.
  18. 18.0 18.1 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.
  19. 19.0 19.1 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.
  20. 20.0 20.1 van Eeden S, Offerhaus GJ, Hart AA, et al. (December 2007). "Goblet cell carcinoid of the appendix: a specific type of carcinoma". Histopathology 51 (6): 763–73. doi:10.1111/j.1365-2559.2007.02883.x. PMID 18042066.
  21. 21.0 21.1 21.2 Pahlavan PS, Kanthan R (June 2005). "Goblet cell carcinoid of the appendix". World J Surg Oncol 3: 36. doi:10.1186/1477-7819-3-36. PMC 1182398. PMID 15967038. http://wjso.com/content/3/1/36. Cite error: Invalid <ref> tag; name "pmid15967038" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid15967038" defined multiple times with different content
  22. Volante M, Righi L, Asioli S, Bussolati G, Papotti M (August 2007). "Goblet cell carcinoids and other mixed neuroendocrine/nonneuroendocrine neoplasms". Virchows Arch. 451 Suppl 1: S61–9. doi:10.1007/s00428-007-0447-y. PMID 17684764.
  23. Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 435. ISBN 978-1416054542.
  24. Modlin, IM.; Lye, KD.; Kidd, M. (Feb 2003). "A 5-decade analysis of 13,715 carcinoid tumors.". Cancer 97 (4): 934-59. doi:10.1002/cncr.11105. PMID 12569593.