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

PGY-2 DDx

  • Celiac.
    • 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.

Duodenal nodules DDX

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

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.

Celiac sprue

General

Etiology

  • Autoimmune.

Epidemiology

  • Associated with:
    • The skin condition dermatitis herpetiformis.[2]
      • Tx: dapsone.
    • IgA deficiency - 10-15X more common in celiac disease vs. healthy controls.[3]
    • Risk factor for gastrointestinal T cell lymphoma - known as: enteropathy-associated T cell lymphoma (EATL).

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:[4]

  • Enteritis.
    • Intraepithelial lymphocytes - key feature.
    • Plasma cells.
    • Macrophages.
  • Loss of villi - important feature.
    • Normal duodenal biopsy should have 3 good villi.
  • Mitosis increased (in the crypts).

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.

DDx:

  • Giardiasis.
    • Have giarrdia organisms.
    • Always consider Giardiasis and especially on exams.
  • Whipple's disease (very rare).
    • Abundant macrophages should make one suspicious.

Grading

Most pathologists do not grade celiac sprue.

The most common system is the modified Marsh system:[5][6]

Marsh 1 Marsh 3A Marsh 3C
Descriptors Well-formed villi Partial villous atrophy Total villous atrophy
Alternate descriptors Normal villous arch. Blunted villi Flattened mucosa

Giardiasis

Etiology

  • Flagellate protozoan Giardia lamblia.

Histology

  • 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.[7]
      • Often look like a crescent moon (image of crescent moon) or semicircular[8] -- 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:

Treatment

  • Antibiotics, e.g. metronidazole (Flagyl).

Whipple's disease

Epidemiology

  • Very rare.
  • Classically middle aged men.

Clinical

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

Etiology

  • Infection - caused by Tropheryma whipplei.[9]

Histology

Features:[10]

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

Treatment

  • Antibiotics - for months and months.

Micrograph: Whipple's disease - wikipedia.org.

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.[11][12][13]

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. ↑ TN 2007 D22
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ Wahab PJ, Meijer JW, Dumitra D, Goerres MS, Mulder CJ (June 2002). "Coeliac disease: more than villous atrophy". Rom J Gastroenterol 11 (2): 121–7. PMID 12145668.
  6. ↑ Ciaccio EJ, Bhagat G, Tennyson CA, Lewis SK, Hernandez L, Green PH (September 2010). "Quantitative Assessment of Endoscopic Images for Degree of Villous Atrophy in Celiac Disease". Dig Dis Sci. doi:10.1007/s10620-010-1371-6. PMID 20844959.
  7. ↑ http://www.water-research.net/Giardia.htm
  8. ↑ http://en.wikipedia.org/wiki/Semicircle
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ Chetty, R. (Apr 2008). "Requiem for the term 'carcinoid tumour' in the gastrointestinal tract?". Can J Gastroenterol 22 (4): 357-8. PMID 18414708.
  12. ↑ 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.
  13. ↑ 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.
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