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Dermatology
Neural and Neuroendocrine Neoplasms (Other Than Neurofibromatosis)
Built from Dermatology, 5th Edition
What’s inside
12 sections · 143 slides
Overview
- What this topic covers
- Two families of cutaneous neural tumors
Classification and Normal Nerve Anatomy
What a peripheral nerve is built from, and how tumors recapitulate that structure
- Classification of cutaneous neural and neuroendocrine tumors
- Structure of the normal peripheral nerve
- How a peripheral nerve is built, layer by layer
- Cells that build neural tumors
- Immunohistochemistry as a diagnostic tool
- Immunohistochemical staining patterns of neural neoplasms
Neuromas
Traumatic neuroma and solitary circumscribed neuroma
- Neuromas: two distinct benign forms
- Traumatic neuroma: epidemiology and cause
- Traumatic neuroma: clinical features
- Rudimentary supernumerary digit
- Traumatic neuroma: what the tissue shows
- Traumatic neuroma: nerve fascicles in a fibrous stroma
- Amputation neuroma of a digit
- Traumatic neuroma: diagnosis and treatment
- Solitary circumscribed neuroma (SCN): overview
- SCN: clinical features
- SCN and multiple mucosal neuroma syndromes
- SCN: what the tissue shows
- SCN: nerve fascicles positive for S100 protein
- Epithelial sheath neuroma
- SCN: diagnosis and treatment
- Clinical features of benign neural neoplasms: neuromas, schwannoma, neurofibroma
- Clinical features of benign neural neoplasms: neuromas, schwannoma, neurofibroma (continued)
- Clinical features of benign neural neoplasms: neuromas, schwannoma, neurofibroma (continued)
- Clinical features of benign neural neoplasms: myxoma, neurothekeoma, granular cell tumor
- Clinical features of benign neural neoplasms: myxoma, neurothekeoma, granular cell tumor (continued)
- Clinical features of benign neural neoplasms: myxoma, neurothekeoma, granular cell tumor (continued)
- Histologic differential diagnosis: traumatic neuroma vs. SCN
- Histologic differential diagnosis: traumatic neuroma vs. SCN (continued)
- Histologic differential diagnosis: schwannoma
- Histologic differential diagnosis: schwannoma (continued)
- Histologic differential diagnosis: neurofibroma (localized cutaneous variant)
- Histologic differential diagnosis: neurofibroma (localized cutaneous variant) (continued)
Schwannoma
A fully encapsulated tumor made almost entirely of Schwann cells
- Schwannoma: overview
- Schwannoma: genetics
- Psammomatous melanotic schwannoma
- Schwannoma: clinical features
- Schwannoma: pathology - Antoni A and B
- Schwannoma: Verocay bodies and SOX10 staining
- Schwannoma subtypes
- Schwannoma: diagnosis and treatment
Neurofibroma
A mixed-cell tumor, with a plexiform form that is a hallmark of NF1
- Neurofibroma: overview
- Mosaic (segmental) neurofibromatosis type 1
- Neurofibroma: pathogenesis
- Solitary neurofibroma: clinical features
- Plexiform neurofibroma: the “bag of worms”
- Plexiform neurofibromas: soft, sagging bag-like plaques
- Plexiform neurofibroma in a child with NF1
- Neurofibroma: pathology of the common type
- Neurofibroma: spindle cells and S100 expression
- Diffuse, intraneural, and plexiform variants
- Plexiform neurofibroma: expanded, twisted nerve fascicles
- When neurofibroma turns malignant
- Steps toward malignant transformation in NF1
- Neurofibroma: diagnosis and treatment
Nerve Sheath Myxoma and Cellular Neurothekeoma
Two distinct entities that used to share a single name
- Two entities, one old name
- Splitting one old diagnosis into two real entities
- Who gets each tumor
- Nerve sheath myxoma: clinical features
- Nerve sheath myxoma: lobular pattern in a myxoid stroma
- Nerve sheath myxoma: what the tissue shows
- Cellular neurothekeoma: clinical features
- Cellular neurothekeoma: epithelioid nests in a hyalinized stroma
- Cellular neurothekeoma: what the tissue shows
- Nerve sheath myxoma and cellular neurothekeoma: diagnosis and treatment
Granular Cell Tumor
Abrikossoff's tumor - a schwannian tumor with lysosome-packed cytoplasm
- Granular cell tumor: overview
- Granular cell tumor: who gets it, and why
- Granular cell tumor: clinical features
- Granular cell tumor: granular cytoplasm and S100 staining
- Granular cell tumor: what the tissue shows
- Granular cell tumor: diagnosis and treatment
Perineurioma
A proliferation of the fascicle-wrapping perineurial cell
- Perineurioma: overview
- Soft tissue perineurioma: storiform spindle-cell pattern
- Key features of soft tissue (extraneural) perineuriomas
- Key features of soft tissue (extraneural) perineuriomas (continued)
Malignant Peripheral Nerve Sheath Tumor
MPNST: rare in the skin, but a real risk from NF1-associated plexiform neurofibromas
- Malignant peripheral nerve sheath tumor (MPNST): overview
- MPNST: pathology
- MPNST: molecular drivers
Merkel Cell Carcinoma
An aggressive, often virus-driven neuroendocrine skin cancer
- Merkel cell carcinoma: overview
- Merkel cell carcinoma: naming and history
- Merkel cell carcinoma: who gets it
- Merkel cell polyomavirus: the 2008 discovery
- How a common skin virus can cause Merkel cell carcinoma
- Virus-positive vs. virus-negative MCC
- Merkel cell carcinoma: what it looks like
- Merkel cell carcinoma: clinical presentations
- Merkel cell carcinoma: additional presentations on the toe and cheek
- Merkel cell carcinoma: nodule on the finger
- Merkel cell carcinoma: stage at diagnosis and outlook
- Merkel cell carcinoma staging (AJCC 8th edition)
- Merkel cell carcinoma: paraneoplastic syndromes
- Merkel cell carcinoma: pathology
- Merkel cell carcinoma: histology and CK20 staining pattern
- Merkel cell carcinoma: immunohistochemistry
- Merkel cell carcinoma: differential diagnosis
- Merkel cell carcinoma: surgery and staging workup
- Merkel cell carcinoma: systemic treatment
- Merkel cell carcinoma: surveillance after treatment
Heterotopic Neural Tissue
Neural tissue misplaced in the skin during embryonic development
- Heterotopic neural tissue: overview
- Nasal glial heterotopia (“nasal glioma”): overview
- Nasal glial heterotopia: clinical features
- Nasal glial heterotopia: pathology
- Nasal glial heterotopia: treatment
- Heterotopic meningeal tissue (rudimentary meningocele): overview
- Rudimentary meningocele: clinical and pathologic features
- Cutaneous meningioma: types II and III
- Heterotopic meningeal tissue: treatment
- Ectopic and heterotopic neural tissue: nasal glial heterotopia vs. rudimentary meningocele
- Ectopic and heterotopic neural tissue: nasal glial heterotopia vs. rudimentary meningocele (continued)
- Ectopic and heterotopic neural tissue: nasal glial heterotopia vs. rudimentary meningocele (continued)
- Ectopic and heterotopic neural tissue: cutaneous meningioma vs. peripheral neuroblastoma
- Ectopic and heterotopic neural tissue: cutaneous meningioma vs. peripheral neuroblastoma (continued)
- Ectopic and heterotopic neural tissue: cutaneous meningioma vs. peripheral neuroblastoma (continued)
Primitive Neuroectodermal Tumors
A spectrum from primitive neuroblastoma to mature ganglioneuroma
- Primitive neuroectodermal tumors: overview
- Primitive neuroectodermal tumors: pathogenesis
- Cutaneous metastases of neuroblastoma in children
- Primary cutaneous PNET in adults
- Neuroblastoma: pathology
- Cutaneous neuroblastoma: Homer Wright-type rosettes
- Toward maturity: ganglioneuroma
- PNET and neuroblastoma: markers and diagnosis
- Primitive neuroectodermal tumors: treatment
- Putting it together
- How to approach a cutaneous neural tumor
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- Dermatology, 5th Edition (2-Volume Set)