Gangliocytic paraganglioma

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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

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.