Surgical Pathology Criteria

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
It is possible that Mucosal Benign Epithelioid Nerve Sheath Tumor and Mucosal Schwann Cell Hamartoma are related

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
Most epithelioid leiomyomas reported in the past are now considered GIST

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

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