Solitary circumscribed neuroma (“palisaded encapsulated neuroma”) represents spontaneous proliferation of peripheral nerve fibers
Multiple mucosal neuromas associated with multiple endocrine neoplasia syndrome (type 2B) represents rare autosomal dominant condition
Traumatic neuroma represents posttraumatic proliferation of peripheral nerve fibers
Morton neuroma represents degenerative neural change with reactive fibrosis on foot
Pacinian neuroma represents painful hyperplasia of Pacinian bodies on finger
Epithelial sheath neuroma represents proliferation of enlarged dermal nerves ensheathed by squamous epithelium
Clinical Issues
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Solitary circumscribed neuroma most common on face
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Morton neuroma occurs in distal parts of peripheral nerves of 3rd and 4th metatarsals
Microscopic
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Spindled Schwann cells &/or peripheral nerve fibers
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Clefts in solitary circumscribed neuroma
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Perineural fibrosis in traumatic neuroma
Ancillary Tests
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Strong S100(+) in Schwann cells and nerve fibers
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Negative for SMA, desmin, melanocytic markers
Top Differential Diagnoses
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Benign nerve sheath tumors
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Superficial (pilar) leiomyoma
TERMINOLOGY
Definitions
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Proliferation of peripheral nerve fibers in which ratio of axons to Schwann cell fascicles is in nearly equal proportions
Solitary circumscribed neuroma [“palisaded encapsulated neuroma” (PEN)] is spontaneous proliferation of peripheral nerve fibers
Multiple mucosal neuromas associated with multiple endocrine neoplasia (MEN) syndrome (type 2B), rare autosomal dominant condition
Traumatic neuroma represents posttraumatic proliferation of peripheral nerve fibers
Morton neuroma is due to degenerative neural changes with reactive fibrosis on foot
Pacinian neuroma represents painful hyperplasia of pacinian bodies on finger
Epithelial sheath neuroma is proliferation of nerves ensheathed by squamous epithelium
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
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Multiple mucosal neuromas in MEN 2B syndrome
Represents rare autosomal dominant condition
Also includes medullary carcinoma of thyroid, pheochromocytoma, and somatic abnormalities
Mucosal neuromas have been rarely reported without any other systemic features of syndrome
Environmental Exposure
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Traumatic neuroma
Amputation neuroma is painful, reactive proliferation of nerve fibers after amputation
Proliferation of nonencapsulated nerve fibers after intrauterine or perinatal amputation of supernumerary digits
Can occur on penis after circumcision
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Morton neuroma
Degenerative damage of peripheral nerves
CLINICAL ISSUES
Epidemiology
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Age
Solitary circumscribed neuroma most common in 5th and 7th decades
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Sex
Solitary circumscribed neuroma occurs in equal ratio in both genders
Site
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Solitary circumscribed neuroma
Majority (90%) located on face
Rare in other anatomic locations
Mucosal involvement has been reported rarely
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Mucosal neuroma
Lips, tongue, eyelids
Upper GI tract
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Morton neuroma
Sole of foot
Usually in distal parts of peripheral nerves of 3rd and 4th metatarsals
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Pacinian neuroma
Usually on fingers