Ganglioneuroma

Epidemiology:

• 40-60y

Common sites:

• left colon

Gross features:

• solitary or multiple if part of MEN IIb or FAP or NF-1
• GI tract:
• exophytic polyp (FAP)
• poorly demarcated transmural (diffuse ganglioneuromatosis) (MEN IIb / NF-1)

Histologic features:

• GI tract:
• polypoid ganglioneuromas:
• expanded lamina propria with disturbed crypt architecture
1. spindle cells within a fibrillary matrix
2. irregular nests and groups of ganglion cells
• may extend into submucosa
• diffuse ganglioneuromatosis:
• proliferation centred around the myenteric plexus
• intramural or transmural
• fusiform expansions or confluent transmural proliferations

Immunophenotype:

Marker:	Sensitivity:	Specificity:
S-100
NSE (ganglion cells)
Synaptophysin (ganglion cells)
Neurofilament protein (ganglion cells)

Molecular features:

Other features:

• diffuse ganglioneuromatosis in virtually all patients with MEN IIb

References:

• Gastrointestinal and Liver Pathology2005