Duodenum

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The duodenum is the first part of the small bowel. It is accessible by EGD (esophagogastroduodenoscopy) and frequently biopsied.

An introduction to gastrointestinal pathology is in the gastrointestinal pathology article.

The clinical history is often: r/o celiac or r/o giardia.

Getting started

Normal duodenum

  • Three tall villi.
  • Few intraepithelial lymphocytes; < 1 lymphocyte / 4 epithelial cells.
  • No (pink) subepithelial collagen band.
  • Predominant lamina propria cell: plasma cells.
    • Lack of plasma cells suggests common variable immunodeficiency (CVID).[1]
  • No organisms in lumen.

Basic DDx

  • Celiac sprue.
    • Intraepithelial lymphocytes - key feature.
    • Loss of villi.
  • Giarrdia.
    • Like celiac... but giarrdia organisms.
  • Adenomas.
    • Too much blue - similar to colonic adenomas.
  • Cancer.
    • Too much blue and epithelium in the wrong place.

More

  • H. pylori only in areas of gastric metaplasia.[2]

Duodenal nodules DDX

 
 
 
 
 
 
 
 
 
 
 
 
Duodenal
nodule
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Benign
(common)
 
 
 
 
 
 
 
 
 
 
 
 
 
Neoplastic
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Brunner's
gland
 
Heterotopic
gastric mucosa
 
Lymphoid
nodule
 
Adenoma
 
NET
 
Paraganglioma
 
Prolapsed
gastric polyp
 
Metastasis
 
 
 
 

Infections of the duodenum[3]

Common:

  • Giardia

Rare:

  • Cryptosporidia.
  • Microsporidia.
  • Isospora belli.
  • Cyclospora.
  • MAI (Mycobacterium avium intracellulare).
  • CMV (cytomegalovirus).
  • Cryptococcus neoformans.

Celiac sprue

Main article: Celiac sprue

General

  • Etiology: autoimmune.

Epidemiology

  • Associated with:
    • The skin condition dermatitis herpetiformis.[4]
    • IgA deficiency - 10-15X more common in celiac disease vs. healthy controls.[5]
    • Risk factor for gastrointestinal T cell lymphoma - known as: enteropathy-associated T cell lymphoma (EATL).

Clinical

Treatment:

  • Gluten free diet.
    • Mnemonic: BROW = barley, rye, oats, wheat.

Serologic testing:

  • Anti-transglutaminase antibody.
    • Alternative test: anti-endomysial antibody.
  • IgA -- assoc. with celiac sprue.

Microscopic

Features:[6]

  • Intraepithelial lymphocytes - key feature.
    • Should be more pronounced at tips of villi.[7]
  • Loss of villi - important feature.
    • Normal duodenal biopsy should have 3 good villi.
  • Plasma cells - abundant (weak feature).
  • Macrophages.
  • Mitosis increased (in the crypts).
  • +/-Collagen band (pink material in mucosa) - "Collagenous sprue"; must encompass ~25% of mucosa.

Image:

Notes:

  • If you see acute inflammatory cells, i.e. neutrophils, consider Giardiasis and other infectious etiologies.
  • Biopsy should consist of 2-3 sites. In children it is important to sample the duodenal cap, as it is the only affected site in ~10% of cases.
  • Flat lesions without IELs are unlikely to be celiac sprue.
  • Mucosa erosions are rare in celiac sprue; should prompt consideration of an alternate diagnosis (infection, medications, Crohn's disease).

Grading

Rarely done - see celiac sprue article.

Giardiasis

General

  • Etiology:
    • Flagellate protozoan Giardia lamblia.
  • Treatment
    • Antibiotics, e.g. metronidazole (Flagyl).

Microscopic

Features:

  • +/-Loss of villi.
  • Intraepithelial lymphocytes.
    • +Other inflammatory cells, especially PMNs, close to the luminal surface.
  • Flagellate protozoa -- diagnostic feature.
    • Organisms often at site of bad inflammation.
    • Pale/translucent on H&E.
    • Size: 12-15 micrometers (long axis) x 6-10 micrometers (short axis) -- if seen completely.[8]
      • Often look like a crescent moon (image of crescent moon) or semicircular[9] -- as the long axis of the organism is rarely in the plane of the (histologic) section.

Notes:

  • Giardiasis can look (histologically) a lot like celiac disease.

Images:

Acute duodenitis

Etiology (DDx)

  • Infection.
    • Helicobactor organisms in the stomach.
  • Medications (NSAIDs).
  • Crohn's disease (usually focal/patchy).
  • Celiac sprue.

Microscopic

Features:

  • Intraepithelial lymphocytes.
  • Neutrophils - "found without searching" - key feature.
  • Eosinophils - "found without searching" - key feature.
  • Plasma cells (increased).

Notes:

  • One needs stomach concurrent biopsies to r/o Helicobactor.
  • Erosions make celiac sprue much less likely.
  • Presence of chronic inflammation useful for NSAIDS vs. Helicobacter organisms:
    • NSAIDs not commonly assoc. with acute inflammation;[10] thus, without chronic inflammation NSAIDs are unlikely.
      • Acute NSAID-related duodenitis reported.[11]

Whipple's disease

General

Epidemiology

  • Very rare.
  • Classically middle aged men.

Clinical

  • Malabsorption (diarrhea), arthritis + others.
    • Symptoms are non-specific.

Treatment:

  • Antibiotics - for months and months.

Etiology

  • Infection - caused by Tropheryma whipplei.[12]

Microscopic

Features:[13]

  • Infectious microorganism typically found in macrophages.
    • Macrophages usually abundant - key feature that should raise Dx in DDx.
    • Organisms periodic acid-Schiff (PAS) positive.

Micrograph: Whipple's disease - wikipedia.org.

DDx:

  • Mycobacterium avium intracellulare (MAI).

Weird stuff

Microvillous inclusion disease

General

  • AKA Davidson's disease.
  • Autosomal recessive inherited condition - due to mutation in MYO5B.[14]

Microscopic

Features:

  • Flat mucosa; no villi.

IHC:

  • Carcinoembryonic antigen (CEA) +ve.[15]

EM:

Notes:

  • Appearance similar to celiac sprue; however, usually lacks the intraepithelial lymphocytic infiltration characteristic of celiac sprue.

Tufting enteropathy

General

  • AKA intestinal epithelial dysplasia.
  • Genetic disease[17] - related to abnormal enterocytes (development and/or differentiation).

Microscopic

Features:[18]

  • Villous atrophy
  • Mononuclear cell infiltration of the lamina propria
  • Abnormal surface enterocytes:
    • Focal crowding -- resembling tufts.

Tumours

Lymphoma

Main article: Lymphoma

Note:

Adenocarcinoma

  • Similar to large bowel adenocarcinomas (see colorectal tumours article).
  • Duodenum - most common site in small bowel.

Risk factors:

Neuroendocrine tumours

General

  • Like neuroendocrine tumours elsewhere.
  • Use of the term carcinoid is discouraged.[19][20][21]

Microscopic

Features:

  • Nests of cells.
  • Stippled chromatin - AKA: salt-and-pepper chromatin, coarse chromatin.
  • Classically subepithelial/mural.

Images:

Ampullary tumours

  • Ampullary carcinoma - has separate staging.
  • Intraductal papillary mucinous tumour (IPMT) - a pancreatic tumour, see pancreas article.

See also

References

  1. ↑ Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L (July 2010). "Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease". Inflamm Bowel Dis. doi:10.1002/ibd.21376. PMID 20629103.
  2. ↑ El-Zimaity. 18 October 2010.
  3. ↑ Serra S, Jani PA (November 2006). "An approach to duodenal biopsies". J. Clin. Pathol. 59 (11): 1133–50. doi:10.1136/jcp.2005.031260. PMC 1860495. PMID 16679353. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860495/?tool=pubmed.
  4. ↑ TN 2007 D22
  5. ↑ Kumar, V.; Jarzabek-Chorzelska, M.; Sulej, J.; Karnewska, K.; Farrell, T.; Jablonska, S. (Nov 2002). "Celiac disease and immunoglobulin a deficiency: how effective are the serological methods of diagnosis?". Clin Diagn Lab Immunol 9 (6): 1295-300. PMID 12414763.
  6. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 843. ISBN 0-7216-0187-1.
  7. ↑ Biagi F, Luinetti O, Campanella J, et al. (August 2004). "Intraepithelial lymphocytes in the villous tip: do they indicate potential coeliac disease?". J. Clin. Pathol. 57 (8): 835–9. doi:10.1136/jcp.2003.013607. PMC 1770380. PMID 15280404. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770380/.
  8. ↑ http://www.water-research.net/Giardia.htm
  9. ↑ http://en.wikipedia.org/wiki/Semicircle
  10. ↑ Taha AS, Dahill S, Nakshabendi I, Lee FD, Sturrock RD, Russell RI (September 1993). "Duodenal histology, ulceration, and Helicobacter pylori in the presence or absence of non-steroidal anti-inflammatory drugs". Gut 34 (9): 1162–6. PMC 1375446. PMID 8406146. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1375446/.
  11. ↑ Hashash JG, Atweh LA, Saliba T, et al. (November 2007). "Acute NSAID-related transmural duodenitis and extensive duodenal ulceration". Clin Ther 29 (11): 2448–52. doi:10.1016/j.clinthera.2007.11.012. PMID 18158085.
  12. ↑ Liang Z, La Scola B, Raoult D (January 2002). "Monoclonal antibodies to immunodominant epitope of Tropheryma whipplei". Clin. Diagn. Lab. Immunol. 9 (1): 156?9. PMC 119894. PMID 11777846. http://cvi.asm.org/cgi/pmidlookup?view=long&pmid=11777846.
  13. ↑ Bai J, Mazure R, Vazquez H, Niveloni S, Smecuol E, Pedreira S, Mauriño E (2004). "Whipple's disease". Clin Gastroenterol Hepatol 2 (10): 849?60. doi:10.1016/S1542-3565(04)00387-8. PMID 15476147.
  14. ↑ Müller T, Hess MW, Schiefermeier N, et al. (October 2008). "MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity". Nat. Genet. 40 (10): 1163–5. doi:10.1038/ng.225. PMID 18724368.
  15. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. ISBN 978-0781740517.
  16. ↑ Kennea N, Norbury R, Anderson G, Tekay A (2001). "Congenital microvillous inclusion disease presenting as antenatal bowel obstruction". Ultrasound Obstet Gynecol 17 (2): 172–4. doi:10.1046/j.1469-0705.2001.00211.x. PMID 11251929.
  17. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/185535. Accessed on: 21 September 2010.
  18. ↑ Goulet O, Salomon J, Ruemmele F, de Serres NP, Brousse N (2007). "Intestinal epithelial dysplasia (tufting enteropathy)". Orphanet J Rare Dis 2: 20. doi:10.1186/1750-1172-2-20. PMC 1878471. PMID 17448233. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1878471/.
  19. ↑ Chetty, R. (Apr 2008). "Requiem for the term 'carcinoid tumour' in the gastrointestinal tract?". Can J Gastroenterol 22 (4): 357-8. PMID 18414708.
  20. ↑ 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.
  21. ↑ 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.

External links

Review article(s)

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