Gangliocytic paraganglioma

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

• Extremely rare.
• May be associated with neurofibromatosis type 1.
• Classified a neuroendocrine tumour.
• Usually has a mix of the features seen in: neuroendocrine tumours, paragangliomas and ganglioneuromas.

Clinical - presentation:

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

Gross

• Classically in the duodenum ~90% of cases.

Microscopic

Features - three components:

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:

• Poorly differentiated carcinoma.
• Neuroendocrine tumour.
• Paraganglioma.

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:

• Epithelioid cells of a GP (wjso.com).
• Ganglion cell in a GP (wjso.com).
• Ganglion cells in a GP (pubcan.org).
• 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.