Gastrointestinal Intramucosal Nerve Sheath Neoplasm
Definition
- Gastrointestinal tract mesenchymal neoplasms located primarily in the lamina propria exhibiting features of nerve sheath differentiation, including:
- Note: This does not include gastrointestinal tract autonomic nerve sheath neoplasm (GANT), which is covered separately
Diagnostic Criteria
- All three are composed of bland S100 positive cells primarily located in the lamina propria
- When occurring as solitary lesions, none is related to familial syndromes such as:
- Cowden disease
- Multiple endocrine adenomatosis 2
- Neurofibromatosis
- All are benign
- All are overwhelmingly located in the colorectum
- It is not clear if or what is the relation between the three
- Select an individual diagnosis below for full description
- At least one of the microcystic/reticular schwannomas described by Liegl 2008 was composed of epithelioid cells confined to the lamina propria and thus may fall within the above spectrum
| Mucosal Benign Epithelioid Nerve Sheath Tumor | Mucosal Schwann Cell Hamartoma | GI Ganglioneuroma |
| Epithelioid | Spindled | Spindled |
| No axons | Very rare axons | Axons present |
| No ganglion cells | No ganglion cells | Ganglion cells present |
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting :November 29, 2009
