Mucosal Benign Epithelioid Nerve Sheath Tumor
Differential Diagnosis
| 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 |
| GI Schwannoma | GI Mucosal Benign Epithelioid Nerve Sheath Tumor |
| Most cases intramural | Most cases confined to lamina propria |
| Peripheral lymphoid cuff common | Lacks lymphoid cuff |
| Frequent pleomorphic hyperchromatic cells | No pleomorphism |
| Spindled cells | Epithelioid cells |
| GI Mucosal Benign Epithelioid Nerve Sheath Tumor | GI Neurofibroma |
| Axons absent | Axons scattered throughout |
| Very uniform infiltrate | More heterogeneity in infiltrate |
| Restricted to colon | Most are in stomach and small intestine |
| Epithelioid cells | Spindled cells |
| GI Mucosal Benign Epithelioid Nerve Sheath Tumor | GI Perineurioma |
| Abundant cytoplasm | Scant cytoplasm |
| No association with hyperplastic crypts | Frequently associated with hyperplastic crypts |
| Perineurial markers negative | Perineurial markers positive |
| S100 positive | S100 negative |
| Epithelioid cells | Spindled cells |
| GI Mucosal Benign Epithelioid Nerve Sheath Tumor | GI Leiomyoma (Epithelioid) |
| Most in lamina propria | Most colonic lesions centered on muscularis mucosae |
| S100 positive | S100 negative |
| Smooth muscle actin negative | Smooth muscle actin positive |
| Restricted to colon | Very rare in intestines |
| GI Mucosal Benign Epithelioid Nerve Sheath Tumor | GIST (Epithelioid) |
| Centered in lamina propria or submucosa | Most centered on muscularis propria |
| S100 positive | S100 negative |
| CD117 negative | CD117 74-95% |
| CD34 negative | CD34 70% |
| Restricted to colon | Most common in stomach |
