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xi
Contents
1 The Peripheral Nerve and Cutaneous Neural Tumors:
Introduction, Definitions, and Classification ................................. 1
Additional Reading ............................................................................ 6
2 Special Techniques for the Study
of Cutaneous Neural Tumors .......................................................... 7
Additional Reading .......................................................................... 14
3 Nonneoplastic and Hamartomatous Lesions
of the Cutaneous Peripheral Nerve .............................................. 15
Palisaded Encapsulated Neuroma .................................................... 15
Definition, Classification, and Histogenesis................................ 15
Clinical Findings ......................................................................... 15
Histopathology ............................................................................ 15
Differential Diagnosis ................................................................. 21
Mucosal (Mucocutaneous) Neuroma ............................................... 21
Definition, Classification, and Histogenesis................................ 21
Clinical ........................................................................................ 21
Histopathology ............................................................................ 22
Differential Diagnosis ................................................................. 25
Fibrolipomatous Hamartoma ........................................................... 25
Definition, Classification, and Histogenesis................................ 25
Clinical ........................................................................................ 26
Histopathology ............................................................................ 26
Differential Diagnosis ................................................................. 26
Traumatic Neuroma ......................................................................... 26
Definition, Classification, and Histogenesis................................ 26
Clinical ........................................................................................ 26
Histopathology ............................................................................ 28
Differential Diagnosis ................................................................. 28
Morton’s Neuroma (Interdigital Neuritis) ........................................ 31
Definition, Classification, and Histogenesis................................ 31
Clinical ........................................................................................ 31
Histopathology ............................................................................ 31
Differential Diagnosis ................................................................. 31
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Reparative Perineurial Hyperplasia ................................................. 33
Definition, Classification, and Histogenesis................................ 33
Clinical ........................................................................................ 33
Histopathology ............................................................................ 33
Differential Diagnosis ................................................................. 33
Epithelial Sheath Neuroma .............................................................. 33
Definition, Classification, and Histogenesis................................ 33
Clinical ........................................................................................ 33
Histopathology ............................................................................ 34
Differential Diagnosis ................................................................. 34
Additional Reading .......................................................................... 35
Palisaded Encapsulated Neuroma ............................................... 35
Mucosal Neuroma ....................................................................... 35
Fibrolipomatous Hamartoma of Nerve ....................................... 36
Reparative Perineurial Hyperplasia ............................................. 36
Epithelial Sheath Neuroma ......................................................... 36
4 Benign Cutaneous Neoplasms with Peripheral
Nerve Differentiation ..................................................................... 37
Neurofibroma ................................................................................... 37
Definition, Classification, and Histogenesis................................ 37
Clinical Findings ......................................................................... 37
Histopathology ............................................................................ 37
Differential Diagnosis ................................................................. 50
Schwannoma .................................................................................... 52
Definition, Classification, and Histogenesis................................ 52
Clinical ........................................................................................ 53
Histopathology ............................................................................ 53
Differential Diagnosis ................................................................. 68
Granular Cell Tumor ........................................................................ 68
Definition, Classification, and Histogenesis................................ 68
Clinical ........................................................................................ 68
Histopathology ............................................................................ 68
Differential Diagnosis ................................................................. 70
Nerve Sheath Myxoma .................................................................... 71
Definition, Classification, and Histogenesis................................ 71
Clinical ........................................................................................ 71
Histopathology ............................................................................ 72
Differential Diagnosis ................................................................. 74
Perineurioma .................................................................................... 76
Definition, Classification, and Histogenesis................................ 76
Clinical ........................................................................................ 76
Histopathology ............................................................................ 76
Differential Diagnosis ................................................................. 81
Lipoblastic Nerve Sheath Tumors .................................................... 81
Additional Reading .......................................................................... 81
Neurofibroma .............................................................................. 81
Schwannoma ............................................................................... 82
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Granular Cell Tumor ................................................................. 82
Nerve Sheath Myxoma .............................................................. 82
Perineurioma ............................................................................. 82
Lipoblastic Nerve Sheath Tumors ............................................. 82
5 Malignant Cutaneous Neoplasms with Peripheral
Nerve Differentiation ................................................................... 83
Malignant Peripheral Nerve Sheath Tumor ................................... 83
Definition, Classification, and Histogenesis.............................. 83
Clinical Findings ....................................................................... 83
Histopathology .......................................................................... 83
Differential Diagnosis ............................................................... 90
Malignant Granular Cell Tumor..................................................... 90
Definition, Classification, and Histogenesis.............................. 90
Clinical ...................................................................................... 90
Histopathology .......................................................................... 90
Differential Diagnosis ............................................................... 90
Additional Reading ........................................................................ 91
Malignant Peripheral Nerve Sheath Tumor ............................... 91
6 Cutaneous Proliferations with Putative
Neural Differentiation.................................................................. 93
Neurothekeoma .............................................................................. 93
Definition, Classification, and Histogenesis.............................. 93
Clinical Findings ....................................................................... 93
Histopathology .......................................................................... 93
Differential Diagnosis ............................................................... 101
Primary Cutaneous Neuroendocrine Carcinoma
(Merkel Cell Carcinoma) ............................................................... 103
Definition, Classification, and Histogenesis.............................. 103
Clinical ...................................................................................... 103
Histopathology .......................................................................... 104
Differential Diagnosis ............................................................... 108
Neurofollicular Hamartoma ........................................................... 109
Definition, Classification, and Histogenesis.............................. 109
Clinical ...................................................................................... 109
Histopathology .......................................................................... 109
Differential Diagnosis ............................................................... 109
Additional Reading ........................................................................ 111
Neurothekeoma ......................................................................... 111
Primary Cutaneous Neuroendocrine Carcinoma ....................... 111
Neurofollicular Hamartoma ...................................................... 111
7 Cutaneous Neuroblastic and Ganglion Cell Proliferations ...... 113
Neuroblastoma ............................................................................... 113
Definition, Classification, and Histogenesis.............................. 113
Clinical Findings ....................................................................... 113
Histologic Features.................................................................... 113
Differential Diagnosis ............................................................... 115
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Ganglioneuroma ............................................................................. 115
Definition, Classification, and Histogenesis.............................. 115
Clinical ...................................................................................... 115
Histopathology .......................................................................... 115
Differential Diagnosis ............................................................... 118
Ganglion Cell Choristoma ............................................................. 118
Definition, Classification, and Histogenesis.............................. 118
Clinical ...................................................................................... 118
Histopathology .......................................................................... 118
Differential Diagnosis ............................................................... 118
Additional Reading ........................................................................ 118
8 Cutaneous Glial and Meningothelial Proliferations ................. 119
Nasal Glioma (Heterotopic Glial Tissue) ....................................... 119
Definition, Classification, and Histogenesis.............................. 119
Clinical Findings ....................................................................... 119
Histopathology .......................................................................... 119
Differential Diagnosis ............................................................... 120
Cutaneous Meningioma ................................................................. 121
Definition, Classification, and Histogenesis.............................. 121
Clinical ...................................................................................... 121
Histopathology .......................................................................... 122
Differential Diagnosis ............................................................... 123
Meningocele ................................................................................... 123
Definition, Classification, and Histogenesis.............................. 123
Clinical ...................................................................................... 124
Histopathology .......................................................................... 124
Differential Diagnosis ............................................................... 126
Additional Reading ........................................................................ 126
Cutaneous Meningioma ............................................................ 126
Appendix: Practical Approach to Neural Tumors –
Flowcharts and Differential Diagnostic Tables ................................. 127
Index ...................................................................................................... 133
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