Difference between revisions of "Peutz-Jeghers polyp"

Peutz-Jeghers polyp
	External resources
EHVSC	10180

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

Latest revision as of 18:22, 25 April 2018

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

General

Epidemiology

Features:^[1]^[2]

Peutz-Jeghers syndrome is autosomal dominant.
Altered gene: STK11.

Clinical

Features:^[3]

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

Increased risk of various neoplasms - primarily:

Breast and gastrointestinal cancer.^[4]
Others tumours:^[5]
- Granulosa cell tumour.
- Sertoli cell tumour - esp. with calcification.

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:

Hyperplastic polyp of the stomach - should not have thickened muscle.
- May be confused with PJP as branching may not be apparent.

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.

References

↑ ^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.
↑ ^2.0 ^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.
↑ URL: http://www.ncbi.nlm.nih.gov/omim/175200. Accessed on: 13 July 2010.
↑ 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.
↑ URL: http://www.ncbi.nlm.nih.gov/omim/175200. Accessed on: 22 December 2010.
↑ URL: http://dictionary.reference.com/browse/frond. Accessed on: 26 July 2011.
↑ C. Streutker. 26 July 2011.
↑ 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.

[Ref_PBoD859-1] 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.

[pmid12692201-2] 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.

[pmid20581245-4] 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.

[pmid24364221-8] 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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@@ Line 1: / Line 1: @@
-#redirect [[Gastrointestinal_tract_polyps#Peutz-Jeghers_polyp]]
+{{ Infobox diagnosis
+| Name       = {{PAGENAME}}
+| Image      = Peutz-Jeghers_syndrome_polyp.jpg
+| Width      =
+| Caption    = Peutz-Jeghers polyp. [[H&E stain]].
+| Micro      = polyp with branching ''or'' thickened muscularis mucosae, benign epithelium, and lamina propria
+| Subtypes   =
+| LMDDx      =
+| Stains     =
+| IHC        =
+| EM         =
+| Molecular  = STK11 mutation
+| IF         =
+| Gross      =
+| Grossing   =
+| Site       = [[colon]], [[small bowel]], [[stomach]], others
+| Assdx      =
+| Syndromes  = [[Peutz-Jeghers syndrome]]
+| Clinicalhx = family history of cancer - esp. gastrointestinal and [[breast cancer|breast]]
+| Signs      =
+| Symptoms   =
+| Prevalence =
+| Bloodwork  =
+| Rads       =
+| Endoscopy  =
+| Prognosis  = benign lesion; suggests increased malignancy risk
+| Other      =
+| ClinDDx    = other [[gastrointestinal polyps]]
+}}
+{{ Infobox external links
+| Name           = Peutz-Jeghers polyp
+| EHVSC          = 10180
+| pathprotocols  =
+| wikipedia      =
+| pathoutlines   =
+}}
+'''Peutz-Jeghers polyp''', abbreviated '''PJP''', is an uncommon [[hamartoma|hamartomous]] [[gastrointestinal polyp]].  It is usually associated with [[Peutz-Jeghers syndrome]].
+==General==
+===Epidemiology===
+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.
+*Altered gene: STK11.
+===Clinical===
+Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/175200 http://www.ncbi.nlm.nih.gov/omim/175200]. Accessed on: 13 July 2010.</ref>
+*Melanocytic macules.
+**Lips, buccal mucosa, and digits.
+**Multiple Peutz-Jeghers polyps.
+Increased risk of various neoplasms - primarily:
+*Breast and gastrointestinal cancer.<ref name=pmid20581245>{{cite journal |author=Beggs AD, Latchford AR, Vasen HF, ''et al.'' |title=Peutz-Jeghers syndrome: a systematic review and recommendations for management |journal=Gut |volume=59 |issue=7 |pages=975–86 |year=2010 |month=July |pmid=20581245 |doi=10.1136/gut.2009.198499 |url=}}</ref>
+*Others tumours:<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/175200 http://www.ncbi.nlm.nih.gov/omim/175200]. Accessed on: 22 December 2010.</ref>
+**[[Granulosa cell tumour]].
+**[[Sertoli cell tumour]] - esp. with calcification.
+==Microscopic==
+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>
+*Frond-like polyp with all three components of mucosa:
+*# Muscosal epithelium (melanotic mucosa, goblet cells).
+*# Lamina propria.
+*# M. mucosae.
+Notes:
+*''Frond'' = leaflike expansion.<ref>URL: [http://dictionary.reference.com/browse/frond http://dictionary.reference.com/browse/frond]. Accessed on: 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.
+DDx:
+*[[Hyperplastic polyp of the stomach]] - should ''not'' have thickened muscle.
+**May be confused with PJP as branching may not be apparent.
+===Images===
+<gallery>
+Image:Peutz-Jeghers_syndrome_polyp.jpg | Peutz-Jeghers polyp - intestine (WC/Nephron)
+</gallery>
+<gallery>
+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>
+==Sign out==
+===Duodenum===
+<pre>
+POLYPS, DUODENUM, EXCISION:
+- PEUTZ-JEGHERS POLYPS (x2) WITH BRUNNER'S GLANDS.
+- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
+</pre>
+===Colon===
+<pre>
+ POLYP, COLON (40 CM), EXCISION:
+- PEUTZ-JEGHERS POLYP.
+- NEGATIVE FOR DYSPLASIA AND NEGATIVE FOR MALIGNANCY.
+</pre>
+==See also==
+*[[Gastrointestinal tract polyps]].
+==References==
+{{Reflist|2}}
+[[Category:Diagnosis]]