Difference between revisions of "Gangliocytic paraganglioma"

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#redirect [[Duodenum#Gangliocytic_paraganglioma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Gangliocytic_paraganglioma_-_intermed_mag.jpg
| Width      =
| Caption    = Gangliocytic paraganglioma. [[H&E stain]].
| Synonyms  =
| Micro      =
| Subtypes  =
| LMDDx      =
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[duodenum]] usually
| Assdx      =
| Syndromes  = [[neurofibromatosis type 1]]
| Clinicalhx =
| Signs      = +/-GI bleed
| Symptoms  = +/-abdominal pain
| Prevalence = extremely rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Gangliocytic paraganglioma''', abbreviated '''GP''', is an extremely rare tumour most often found in the [[duodenum]].


==General==
*Extremely rare.<ref name=pmid22340577>{{Cite journal  | last1 = Wu | first1 = GC. | last2 = Wang | first2 = KL. | last3 = Zhang | first3 = ZT. | title = Gangliocytic paraganglioma of the duodenum: a case report. | journal = Chin Med J (Engl) | volume = 125 | issue = 2 | pages = 388-9 | month = Jan | year = 2012 | doi =  | PMID = 22340577 }}</ref>
*May be associated with [[neurofibromatosis type 1]].<ref name=pmid12754392>{{Cite journal  | last1 = Castoldi | first1 = L. | last2 = De Rai | first2 = P. | last3 = Marini | first3 = A. | last4 = Ferrero | first4 = S. | last5 = De Luca | first5 = V. | last6 = Tiberio | first6 = G. | title = Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction. | journal = Int J Gastrointest Cancer | volume = 29 | issue = 2 | pages = 93-98 | month =  | year = 2001 | doi =  | PMID = 12754392 }}</ref>
*Classified a [[neuroendocrine tumour]].<ref>URL: [http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SmallbowelNET_11protocol.pdf http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SmallbowelNET_11protocol.pdf]. Accessed on: 29 March 2012.</ref>
*Usually has a mix of the features seen in: [[neuroendocrine tumour]]s, [[paraganglioma]]s and [[ganglioneuroma]]s.
Clinical - presentation:<ref name=pmid21599949/>
*GI bleed ~ 45% of cases.
*Abdominal pain ~ 43% of cases.
*[[Anemia]] ~ 15% of cases.
==Gross==
*Classically in the duodenum ~90% of cases.<ref name=pmid21599949>{{Cite journal  | last1 = Okubo | first1 = Y. | last2 = Wakayama | first2 = M. | last3 = Nemoto | first3 = T. | last4 = Kitahara | first4 = K. | last5 = Nakayama | first5 = H. | last6 = Shibuya | first6 = K. | last7 = Yokose | first7 = T. | last8 = Yamada | first8 = M. | last9 = Shimodaira | first9 = K. | title = Literature survey on epidemiology and pathology of gangliocytic paraganglioma. | journal = BMC Cancer | volume = 11 | issue =  | pages = 187 | month =  | year = 2011 | doi = 10.1186/1471-2407-11-187 | PMID = 21599949 }}</ref>
==Microscopic==
Features - three components:<ref name=pmid15740625>{{Cite journal  | last1 = Wong | first1 = A. | last2 = Miller | first2 = AR. | last3 = Metter | first3 = J. | last4 = Thomas | first4 = CR. | title = Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature. | journal = World J Surg Oncol | volume = 3 | issue = 1 | pages = 15 | month = Mar | year = 2005 | doi = 10.1186/1477-7819-3-15 | PMID = 15740625 }}</ref><ref>URL: [http://surgpathcriteria.stanford.edu/gitumors/gangliocytic-paraganglioma/printable.html http://surgpathcriteria.stanford.edu/gitumors/gangliocytic-paraganglioma/printable.html]. Accessed on: 31 May 2012.</ref>
#Ganglion cells = large cells with:
#*Round large nucleus.
#*Prominent [[nucleolus]].
#*Moderate or abundant cytoplasm.
#Epithelioid cells (neuroendocrine component):
#*Arranged in nests or cords.
#*Stippled chromatin.
#Spindle cells ([[Schwannoma|schwannian]] component):
#*Moderate or abundant cytoplasm.
#*Nucleus spindle-shaped or ellipsoid.
DDx:<ref name=pmid15740625/>
*Poorly differentiated carcinoma.
*[[Neuroendocrine tumour]].
*[[Paraganglioma]].
===Images===
<gallery>
Image:Gangliocytic_paraganglioma_-_intermed_mag.jpg | GP - intermed. mag. (WC)
Image:Gangliocytic_paraganglioma_-_high_mag.jpg | GP - high mag. (WC)
Image:Gangliocytic_paraganglioma_-_very_high_mag.jpg | GP - very high mag. (WC)
Image:Gangliocytic_paraganglioma_-_2_-_intermed_mag.jpg | GP - 2 - intermed. mag. (WC)
Image:Gangliocytic_paraganglioma_-_2_-_high_mag.jpg | GP - 2 - high mag. (WC)
</gallery>
www:
*[http://www.wjso.com/content/3/1/15/figure/F2 Epithelioid cells of a GP (wjso.com)].
*[http://www.wjso.com/content/3/1/15/figure/F4 Ganglion cell in a GP (wjso.com)].
*[http://www.pubcan.org/images/large/Fig_5-17_A.jpg Ganglion cells in a GP (pubcan.org)].<ref>URL: [http://www.pubcan.org/printicdotopo.php?id=5028 http://www.pubcan.org/printicdotopo.php?id=5028]. Accessed on: 15 April 2012.</ref>
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802175012135 GP (surgicalpathologyatlas.com)].
==IHC==
*Synaptophysin +ve.
*CD56 +ve.
*Chromogranin A +ve.
*HU +ve in ganglion-like cells.
*S100 +ve in spindle cells & sustentacular cells.
==See also==
*[[Duodenum]].
==References==
{{Reflist|2}}
[[Category:Duodenum]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Latest revision as of 19:14, 12 April 2014

Gangliocytic paraganglioma
Diagnosis in short

Gangliocytic paraganglioma. H&E stain.
Site duodenum usually
Syndromes neurofibromatosis type 1
Signs +/-GI bleed
Symptoms +/-abdominal pain
Prevalence extremely rare

Gangliocytic paraganglioma, abbreviated GP, is an extremely rare tumour most often found in the duodenum.

General

Clinical - presentation:[4]

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

Gross

  • Classically in the duodenum ~90% of cases.[4]

Microscopic

Features - three components:[5][6]

  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):
    • Moderate or abundant cytoplasm.
    • Nucleus spindle-shaped or ellipsoid.

DDx:[5]

Images

  • GP - intermed. mag. (WC)

  • GP - high mag. (WC)

  • GP - very high mag. (WC)

  • GP - 2 - intermed. mag. (WC)

  • GP - 2 - high mag. (WC)

www:

IHC

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

See also

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ URL: http://www.cap.org/apps/docs/committees/cancer/cancer_protocols/2011/SmallbowelNET_11protocol.pdf. Accessed on: 29 March 2012.
  4. ↑ 4.0 4.1 Okubo, Y.; Wakayama, M.; Nemoto, T.; Kitahara, K.; Nakayama, H.; Shibuya, K.; Yokose, T.; Yamada, M. et al. (2011). "Literature survey on epidemiology and pathology of gangliocytic paraganglioma.". BMC Cancer 11: 187. doi:10.1186/1471-2407-11-187. PMID 21599949.
  5. ↑ 5.0 5.1 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.
  6. ↑ URL: http://surgpathcriteria.stanford.edu/gitumors/gangliocytic-paraganglioma/printable.html. Accessed on: 31 May 2012.
  7. ↑ URL: http://www.pubcan.org/printicdotopo.php?id=5028. Accessed on: 15 April 2012.
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