Duodenum

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The duodenum is the first part of the small bowel and receives food from the stomach. 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.
  • No organisms in lumen.

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DUODENUM, BIOPSY: 
- SMALL BOWEL MUCOSA AND BRUNNER'S GLANDS WITHIN NORMAL LIMITS.

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:

Rare:

Common stuffs

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 (IELs) - key feature.
    • Should be more pronounced at tips of villi.[7]
    • Criteria for number varies:
      • > 40 IELs / 100 enterocytes (epithelial cells).[8]
      • > 25 IELs / 100 enterocytes (epithelial cells).[9]
  • 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.[10]
      • Often look like a crescent moon (image of crescent moon) or semicircular[11] -- as the long axis of the organism is rarely in the plane of the (histologic) section.

DDx:

Images:

Acute duodenitis

  • Abbreviated AD.

General

DDx:

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;[12] thus, without chronic inflammation NSAIDs are unlikely.
      • Acute NSAID-related duodenitis reported.[13]

Peptic duodenitis

General

Microscopic

Features:[15]

DDx:

Stains

Foveolar metaplasia:

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DUODENUM, BIOPSY:
- SMALL BOWEL MUCOSA AND SUPERFICIAL SUBMUCOSA WITH BRUNNER'S GLAND HYPERPLASIA AND GASTRIC FOVEOLAR METAPLASIA.

COMMENT:
The findings are consistent with a chronic duodenitis.

Micro

The sections show small bowel mucosa and a small amount of submucosa. Brunner's glands are abundant and found focally in the lamina propria. Gastric foveolar-type epithelium is identified. Intraepithelial neutrophils are not identified.

The epithelium matures appropriately. There is no increase in intraepithelial lymphocytes.

Brunner's gland hyperplasia

Brunner's gland hamartoma redirects here.
  • Abbreviated BGH.

General

  • Benign.
  • Usually asymptomatic.[16]

Note:

  • The AFIP uses the term Brunner's gland hamartoma for lesions > 5 mm.[17]
    • Multiple lesions less than 5 mm are hyperplasia.

Gross

  • Nodularity of the duodenum.

Microscopic

Features:

  • Prominent Brunner's gland.
    • Tubular structures - formed by cells abundant cytoplasm that is clear with eosinophilic "cobwebs" and a round, small basal nucleus without a nucleolus.
    • Brunner's gland in the lamina propria.[citation needed]
  • +/-Pancreatic acini and ducts.[17]

DDx:

Image:


Sign out

DUODENUM, BIOPSY:
- CONSISTENT WITH BRUNNER'S GLAND HYPERPLASIA.
- NEGATIVE FOR DYSPLASIA.

Micro

The sections show small bowel mucosa and a small amount of submucosa. Brunner's glands are abundant and found focally in the lamina propria.

The epithelium matures appropriately. There is no increase in intraepithelial lymphocytes. No foveolar metaplasia of the epithelium is identified.

Weird stuff

Disaccharidases deficiency

General

  • Common among asians.
  • Includes: lactase, sucrase, and maltase.
    • Lactase changes seen with mild histomorphologic changes.[18]
    • Maltase and sucrase only affected in moderate and severe lesions.

Microscopic

Features:[18]

  • Decreased villous-crypt ratio (mild to severe).
  • +/-Inflammation (only in moderate and severe).

DDx:

Notes:

  • May have normal histomorphology.[18]

Whipple disease

General

Etiology:

  • Infection - caused by Tropheryma whipplei.[20]

Epidemiology:

  • Very rare.
  • Classically middle aged men.

Clinical

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

Treatment:

  • Antibiotics - for months and months.

Microscopic

Features:[21]

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

DDx:

Images:

Stains

  • PAS +ve organisms.
  • AFB stain -ve -- to r/o MAI.

Image:

Microvillous inclusion disease

This rare disease presents very shortly after birth.

Tufting enteropathy

  • AKA intestinal epithelial dysplasia.

General

  • Genetic disease[22] - related to abnormal enterocytes (development and/or differentiation).
    • Gene implicated: EPCAM.[23]

Microscopic

Features:[24]

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


Gangliocytic paraganglioma

  • Abbreviated GP.

General

Clinical - presentation:[28]

  • GI bleed ~ 45% of cases.
  • Abdominal pain ~ 43% of cases.
  • Anemia ~ 15% of cases.

Gross

  • Classically in the duodenum ~90% of cases.[28]

Microscopic

Features - three components:[29][30]

  1. Ganglion cells = large cells with:
    • Round large nucleus.
    • Prominent nucleolus.
    • Moderate or abundant cytoplasm.
  2. Epithelioid cells (neuroendocrine component):
    • Arranged in nests or cords.
    • Stippled chromatin.
  3. Spindle cells (schwannian component):
      1. Moderate or abundant cytoplasm.
      2. Nucleus spindle-shaped or ellipsoid.

DDx:[29]

Images:

IHC

  • Synaptophysin +ve.
  • CD56 +ve.
  • Chromogranin A +ve.
  • HU +ve in ganglion-like cells.
  • S100 +ve in spindle cells & sustentacular cells.

Pseudomelanosis duodeni

General

  • Rare.
  • Consists of iron and lipofuscin.[32]

Associations:[33]

  • Hypertension ~90% of cases.
  • Iron supplementation ~75% of cases.
  • End-stage renal disease ~60% of cases.

Note:

Gross/endoscopic

  • Dark spots ~35% of cases.[33]

Microscopic

Features:

  • Dark pigment in the lamina propria macrophages.

Images:

Stains

  • Prussian blue +ve ~80% of cases.[33]

Tumours

Lymphoma

Note:

Adenocarcinoma of the duodenum

  • AKA duodenal adenocarcinoma.
  • AKA duodenal carcinoma.

General

Risk factors:

Microscopic

Features:

DDx:

IHC

Duodenal neuroendocrine tumour

General

Associations:

Microscopic

Features:[38]

  • Usu. nests of cells - may be:
    • Trabecular.
    • Glandular - common in stomatostatin producing tumours.
  • Stippled chromatin - (AKA salt-and-pepper chromatin, coarse chromatin).
  • Classically subepithelial/mural.
  • +/-Psammoma bodies - suggestive of somatostatinoma and NF1.[39]

DDx:

Images:

Ampullary tumours

General

  • Individuals with high-grade dysplasia (on biopsy) are usually treated with a pancreaticoduodenectomy (Whipple procedure), as local resections have a very high recurrence rate.[40]

Microscopic

Features:

DDx:

Sign out

  • Ampullary carcinoma - has separate staging.

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. Oberhuber G, Granditsch G, Vogelsang H (October 1999). "The histopathology of coeliac disease: time for a standardized report scheme for pathologists". Eur J Gastroenterol Hepatol 11 (10): 1185–94. PMID 10524652.
  9. Corazza GR, Villanacci V, Zambelli C, et al. (July 2007). "Comparison of the interobserver reproducibility with different histologic criteria used in celiac disease". Clin. Gastroenterol. Hepatol. 5 (7): 838–43. doi:10.1016/j.cgh.2007.03.019. PMID 17544877.
  10. http://www.water-research.net/Giardia.htm
  11. http://en.wikipedia.org/wiki/Semicircle
  12. 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/.
  13. 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.
  14. Genta, RM.; Kinsey, RS.; Singhal, A.; Suterwala, S. (Nov 2010). "Gastric foveolar metaplasia and gastric heterotopia in the duodenum: no evidence of an etiologic role for Helicobacter pylori.". Hum Pathol 41 (11): 1593-600. doi:10.1016/j.humpath.2010.04.010. PMID 20656325.
  15. 15.0 15.1 Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 145. ISBN 978-0443066573.
  16. 16.0 16.1 Lee, WC.; Yang, HW.; Lee, YJ.; Jung, SH.; Choi, GY.; Go, H.; Kim, A.; Cha, SW. (Jun 2008). "Brunner's gland hyperplasia: treatment of severe diffuse nodular hyperplasia mimicking a malignancy on pancreatic-duodenal area.". J Korean Med Sci 23 (3): 540-3. doi:10.3346/jkms.2008.23.3.540. PMID 18583897.
  17. 17.0 17.1 17.2 Patel, ND.; Levy, AD.; Mehrotra, AK.; Sobin, LH. (Sep 2006). "Brunner's gland hyperplasia and hamartoma: imaging features with clinicopathologic correlation.". AJR Am J Roentgenol 187 (3): 715-22. doi:10.2214/AJR.05.0564. PMID 16928936.
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  21. 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.
  22. Online 'Mendelian Inheritance in Man' (OMIM) 613217
  23. Online 'Mendelian Inheritance in Man' (OMIM) 185535
  24. 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/.
  25. Wu, GC.; Wang, KL.; Zhang, ZT. (Jan 2012). "Gangliocytic paraganglioma of the duodenum: a case report.". Chin Med J (Engl) 125 (2): 388-9. PMID 22340577.
  26. Castoldi, L.; De Rai, P.; Marini, A.; Ferrero, S.; De Luca, V.; Tiberio, G. (2001). "Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction.". Int J Gastrointest Cancer 29 (2): 93-98. PMID 12754392.
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  29. 29.0 29.1 Wong, A.; Miller, AR.; Metter, J.; Thomas, CR. (Mar 2005). "Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature.". World J Surg Oncol 3 (1): 15. doi:10.1186/1477-7819-3-15. PMID 15740625.
  30. URL: http://surgpathcriteria.stanford.edu/gitumors/gangliocytic-paraganglioma/printable.html. Accessed on: 31 May 2012.
  31. URL: http://www.pubcan.org/printicdotopo.php?id=5028. Accessed on: 15 April 2012.
  32. Lin, HJ.; Tsay, SH.; Chiang, H.; Tsai, YT.; Lee, SD.; Yeh, YS.; Lo, GH. (Apr 1988). "Pseudomelanosis duodeni. Case report and review of literature.". J Clin Gastroenterol 10 (2): 155-9. PMID 2458404.
  33. 33.0 33.1 33.2 Giusto, D.; Jakate, S. (Feb 2008). "Pseudomelanosis duodeni: associated with multiple clinical conditions and unpredictable iron stainability - a case series.". Endoscopy 40 (2): 165-7. doi:10.1055/s-2007-995472. PMID 18253910.
  34. Bläker, H.; Aulmann, S.; Helmchen, B.; Otto, HF.; Rieker, RJ.; Penzel, R. (2004). "Loss of SMAD4 function in small intestinal adenocarcinomas: comparison of genetic and immunohistochemical findings.". Pathol Res Pract 200 (1): 1-7. PMID 15157044.
  35. Chetty, R. (Apr 2008). "Requiem for the term 'carcinoid tumour' in the gastrointestinal tract?". Can J Gastroenterol 22 (4): 357-8. PMID 18414708.
  36. 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.
  37. 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.
  38. URL: http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SmallbowelNET_11protocol.pdf. Accessed on: 29 March 2012.
  39. Kim, JA.; Choi, WH.; Kim, CN.; Moon, YS.; Chang, SH.; Lee, HR. (Mar 2011). "Duodenal somatostatinoma: a case report and review.". Korean J Intern Med 26 (1): 103-7. doi:10.3904/kjim.2011.26.1.103. PMID 21437171.
  40. Meneghetti, AT.; Safadi, B.; Stewart, L.; Way, LW. (Dec 2005). "Local resection of ampullary tumors.". J Gastrointest Surg 9 (9): 1300-6. doi:10.1016/j.gassur.2005.08.031. PMID 16332486.

External links

Review article(s)