Ganglioneuroma of the Gastrointestinal Tract
Definition
- Gastrointestinal polyp composed of S100 positive spindled cells and ganglion cells
Note
- Ganglioneuroma of the GI tract has nothing to do with the lesion of the same name occurring in the adrenal and related tissues that is part of the neuroblastoma spectrum
Diagnostic Criteria
- Lamina propria expanded by an infiltrate of spindled cells and ganglion cells
- Indistinct borders
- Spindled cells in a fibrillar matrix surround crypts
- No atypia or mitotic activity
- Varying numbers of ganglion cells
- May be immature
- Nuclei smaller and nucleoli less prominent
- May range from rare to nodular collections
- Eosinophils may be frequent
- Extensively S100 positive spindled cells
- Neurofilament may highlight ganglion cells and multiple axons
- Surrounded crypts may be distorted and cystic
- May resemble juvenile polyps
- Plexiform pattern seen in some cases
- Involves mucosa and submucosa
- Nearly all occur in the colorectum
- Most endoscopically biopsied lesions are a few mm in size
- Can be as large as 3 cm
- When solitary:
- No relationship to familial syndromes
- Benign
- No recurrence
- Similar non-solitary lesions associated with syndromes
- Ganglioneuromatous polyposis
- Multiple ganglioneuromas similar to above
- Usually 20 to innumerable
- May be more polymorphous
- May have villous surface
- Frequently reported with associated peripheral and GI lipomas
- May be associated with PTEN Hamartomatous Tumor syndrome (unified syndrome including Cowden Disease and Bannayan-Ruvalcaba-Riley syndrome)
- Cutaneous lesions of PHTS other than lipoma have not been described, however
- Diffuse ganglioneuromatosis
- Poorly demarcated nodular or diffuse involvement of a bowel segment by a mixture of spindled and ganglion cells
- May form a sessile tumor mass
- Frequently transmural
- Most often involves region of terminal ileum, appendix and cecum
- Associated with PHTS, MEN2b and NF1
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/last update : 11/29/09, 3/7/16
