Difference between revisions of "Peutz-Jeghers polyp"

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| Width      =
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| Caption    = Peutz-Jeghers polyp. [[H&E stain]].  
| Caption    = Peutz-Jeghers polyp. [[H&E stain]].  
| Micro      = polyp with: (1) muscosal epithelium, (2) lamina propria and (3) muscularis mucosae - esp. branching ''or'' thickened
| Micro      = polyp with branching ''or'' thickened muscularis mucosae, benign epithelium, and lamina propria
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      =
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| Assdx      =
| Assdx      =
| Syndromes  = [[Peutz-Jeghers syndrome]]
| Syndromes  = [[Peutz-Jeghers syndrome]]
| Clinicalhx = family history of cancer - esp. [[CRC]]
| Clinicalhx = family history of cancer - esp. gastrointestinal and [[breast cancer|breast]]
| Signs      =
| Signs      =
| Symptoms  =
| Symptoms  =
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| Rads      =
| Rads      =
| Endoscopy  =
| Endoscopy  =
| Prognosis  = benign lesion; suggests increase malignancy risk
| Prognosis  = benign lesion; suggests increased malignancy risk
| Other      =
| Other      =
| ClinDDx    = other [[gastrointestinal polyps]]
| ClinDDx    = other [[gastrointestinal polyps]]
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| pathoutlines  =
| pathoutlines  =
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'''Peutz-Jeghers polyp''' is an uncommon gastrointestinal polyp.  It is usually associated with [[Peutz-Jeghers syndrome]].
'''Peutz-Jeghers polyp''', abbreviated '''PJP''', is an uncommon [[hamartoma|hamartomous]] [[gastrointestinal polyp]].  It is usually associated with [[Peutz-Jeghers syndrome]].


==General==
==General==
===Epidemiology===
===Epidemiology===
Features:<ref name=Ref_PBoD859/><ref name=pmid12692201>{{Cite journal  | last1 = Bronner | first1 = MP. | title = Gastrointestinal inherited polyposis syndromes. | journal = Mod Pathol | volume = 16 | issue = 4 | pages = 359-65 | month = Apr | year = 2003 | doi = 10.1097/01.MP.0000062992.54036.E4 | PMID = 12692201 | url = http://www.nature.com/modpathol/journal/v16/n4/full/3880773a.html }}</ref>
Features:<ref name=Ref_PBoD859>{{Ref PBoD|859}}</ref><ref name=pmid12692201>{{Cite journal  | last1 = Bronner | first1 = MP. | title = Gastrointestinal inherited polyposis syndromes. | journal = Mod Pathol | volume = 16 | issue = 4 | pages = 359-65 | month = Apr | year = 2003 | doi = 10.1097/01.MP.0000062992.54036.E4 | PMID = 12692201 | url = http://www.nature.com/modpathol/journal/v16/n4/full/3880773a.html }}</ref>
*[[Peutz-Jeghers syndrome]] is autosomal dominant.
*[[Peutz-Jeghers syndrome]] is autosomal dominant.
*Altered gene: STK11.
*Altered gene: STK11.
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**The '''key''' is "thick" smooth muscle bundles - if one is lucky one sees branching.<ref>C. Streutker. 26 July 2011.</ref>  
**The '''key''' is "thick" smooth muscle bundles - if one is lucky one sees branching.<ref>C. Streutker. 26 July 2011.</ref>  
***"Thick" ~= thickness of muscularis mucosae.
***"Thick" ~= thickness of muscularis mucosae.
DDx:
*[[Hyperplastic polyp of the stomach]] - should ''not'' have thickened muscle.
**May be confused with PJP as branching may not be apparent.


===Images===
===Images===
<gallery>
<gallery>
Image:Peutz-Jeghers_syndrome_polyp.jpg | Peutz-Jeghers polyp - intestine (WC/Nephron)
Image:Peutz-Jeghers_syndrome_polyp.jpg | Peutz-Jeghers polyp - intestine (WC/Nephron)
Image:Gastric_Peutz-Jeghers_polyp_-_very_low_mag.jpg | Peutz-Jeghers polyp - stomach (WC/Nephron)
</gallery>
</gallery>
www:
<gallery>
*[http://www.nature.com/modpathol/journal/v16/n4/fig_tab/3880773f3.html Peutz-Jeghers polyp (nature.com)].
Image:Gastric_Peutz-Jeghers_polyp_-_very_low_mag.jpg | Peutz-Jeghers polyp - stomach - very low mag. (WC/Nephron)
Image: Gastric Peutz-Jeghers polyp - low mag.jpg | Peutz-Jeghers polyp - stomach - low mag. (WC/Nephron)
Image: Gastric Peutz-Jeghers polyp - intermed mag.jpg | Peutz-Jeghers polyp - stomach - intermed. mag. (WC/Nephron)
</gallery>
<gallery>
Image:Colon histology with Peutz-Jeghers polyp.jpg | Peutz-Jeghers (colonic) polyp (WC)
</gallery>
 
==IHC==
*[[Chromogranin A]] - increased staining in relation to background; increased enterochromaffin cells.<ref name=pmid24364221>{{Cite journal  | last1 = Krstić | first1 = M. | last2 = Katić | first2 = V. | last3 = Stojnev | first3 = S. | last4 = Mihailović | first4 = D. | last5 = Mojsilović | first5 = M. | last6 = Radovanović | first6 = Z. | last7 = Zivković | first7 = V. | title = Peutz-Jeghers syndrome: quantitative study on enterochromaffin cells in hamartomatous intestine polyps. | journal = Srp Arh Celok Lek | volume = 141 | issue = 9-10 | pages = 602-7 | month =  | year =  | doi =  | PMID = 24364221 }}</ref>


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Latest revision as of 18:22, 25 April 2018

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

  • Peutz-Jeghers polyp - intestine (WC/Nephron)

  • Peutz-Jeghers polyp - stomach - very low mag. (WC/Nephron)

  • Peutz-Jeghers polyp - stomach - low mag. (WC/Nephron)

  • Peutz-Jeghers polyp - stomach - intermed. mag. (WC/Nephron)

  • Peutz-Jeghers (colonic) polyp (WC)

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.
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