Peutz-Jeghers polyp

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Peutz-Jeghers polyp
Diagnosis in short

Peutz-Jeghers polyp. H&E stain.

LM polyp with branching or thickened muscularis mucosae, benign epithelium, and lamina propria
Molecular STK11 mutation
Site colon, small bowel, stomach, others

Syndromes Peutz-Jeghers syndrome

Clinical history family history of cancer - esp. gastrointestinal and breast
Prognosis benign lesion; suggests increased malignancy risk
Clin. DDx other gastrointestinal polyps
Peutz-Jeghers polyp
External resources
EHVSC 10180

Peutz-Jeghers polyp, abbreviated PJP, is an uncommon hamartomous gastrointestinal polyp. It is usually associated with Peutz-Jeghers syndrome.

General

Epidemiology

Features:[1][2]

Clinical

Features:[3]

  • Melanocytic macules.
    • Lips, buccal mucosa, and digits.
    • Multiple Peutz-Jeghers polyps.

Increased risk of various neoplasms - primarily:

Microscopic

Features:[1][2]

  • Frond-like polyp with all three components of mucosa:
    1. Muscosal epithelium (melanotic mucosa, goblet cells).
    2. Lamina propria.
    3. M. mucosae.

Notes:

  • Frond = leaflike expansion.[6]
    • The key is "thick" smooth muscle bundles - if one is lucky one sees branching.[7]
      • "Thick" ~= thickness of muscularis mucosae.

DDx:

Images

IHC

  • Chromogranin A - increased staining in relation to background; increased enterochromaffin cells.[8]

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Duodenum

POLYPS, DUODENUM, EXCISION:
- PEUTZ-JEGHERS POLYPS (x2) WITH BRUNNER'S GLANDS.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

Colon

 POLYP, COLON (40 CM), EXCISION:
- PEUTZ-JEGHERS POLYP.
- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.

See also

References

  1. 1.0 1.1 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. 859. ISBN 0-7216-0187-1.
  2. 2.0 2.1 Bronner, MP. (Apr 2003). "Gastrointestinal inherited polyposis syndromes.". Mod Pathol 16 (4): 359-65. doi:10.1097/01.MP.0000062992.54036.E4. PMID 12692201. http://www.nature.com/modpathol/journal/v16/n4/full/3880773a.html.
  3. URL: http://www.ncbi.nlm.nih.gov/omim/175200. Accessed on: 13 July 2010.
  4. Beggs AD, Latchford AR, Vasen HF, et al. (July 2010). "Peutz-Jeghers syndrome: a systematic review and recommendations for management". Gut 59 (7): 975–86. doi:10.1136/gut.2009.198499. PMID 20581245.
  5. URL: http://www.ncbi.nlm.nih.gov/omim/175200. Accessed on: 22 December 2010.
  6. URL: http://dictionary.reference.com/browse/frond. Accessed on: 26 July 2011.
  7. C. Streutker. 26 July 2011.
  8. Krstić, M.; Katić, V.; Stojnev, S.; Mihailović, D.; Mojsilović, M.; Radovanović, Z.; Zivković, V.. "Peutz-Jeghers syndrome: quantitative study on enterochromaffin cells in hamartomatous intestine polyps.". Srp Arh Celok Lek 141 (9-10): 602-7. PMID 24364221.