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38 Cards in this Set
- Front
- Back
Neurofibroma
You would need to resect that part of the nerve which would probably cause some deficits |
What type of tumor does this exemplify? What kind of treatment would be needed?
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Schwannoma
Tumor could probably be teased off from the nerve, leaving it intact |
What type of tumor does this exemplify? What type of treatment would this need?
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Unencapsulated, but well-circumscribed masses of spindle cells, which occur in the dermis (cutaneous), in the peripheral nerve (solitary), or in a large nerve trunk (plexiform)
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Neurofibromas
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What type of Neurofibroma is associated with NF-1? What may this transform into?
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Plexiform
Malignant Peripheral Nerve Sheath Tumor (MPNST) |
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Neurofibromas
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What are seen in these pictures?
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A moderately firm, usually benign, unencapsulated, slow-growing heterogeneous tumors of the nervous system arising from the supporting cells (Schwann cells) of peripheral nerves
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Neurofibroma
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How are Schwannoma's & Neurofibromas different?
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In contrast to Schwannomas - another type of tumor arising from Schwan cells - Neurofibromas incorporate all sorts of cells and structural elements in addition to the Schwann cells
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What syndrome are Schwannomas associated with?
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Neurofibromatosis-2
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Where do Schwannomas typically occur?
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1. CN VIII = Vestibular branch = Acoustic neuroma
2. CN V (Trigeminal) 3. Spinal nerve roots 4. Peripheral nerves |
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What is the pathology seen in Schwannomas?
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Encapsulated
Micro: -Antoni A = interlacing bundles of elongated cells with palisading nuclei -Antoni B = looser, less cellular pattern |
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Schwannoma
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What is this seen in?
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These are the classic microscopic appearances of a schwannoma, which is benign. Note the more cellular "Antoni A" pattern on the left with palisading nuclei surrounding pink areas (Verocay bodies). On the right is the "Antoni B" pattern with a looser stroma, fewer cells, and myxoid change
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What is this showing?
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Autosomal dominant disorder that is caused by a mutation of a tumor suppressor gene located on chr. 17
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Neurofibromatosis-1
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What are the clinical features of Neurofibromatosis-1?
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1. Neurofibromas
2. Gliomas of the Optic nerve 3. Lisch nodules = pigmented nodules of the iris 4. Cafe-au-lait spots |
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A 14-year-old girl presents to your clinic complaining of multiple nodules on her skin. She tells you that her mother suffers from a similar condition. Upon examination, you find multiple coffee-colored macules on her torso & limbs & pigmented nodules on her irises. You suspect her condition is due to an Autosomal Dominant genetic disorder. Dx?
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Neurofibromatosis-1
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Neurofibroma in a NF-1 patient
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What is this showing?
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Malignant peripheral nerve sheath tumors (MPNST) that arose from from a Neurofibroma
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What is this showing?
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Syndrome in which the normal product is Neurofibromin, a tumor suppressor gene....but is mutated
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NF-1
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What are the clinical manifestations of NF-2?
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1. Bilateral Acoustic Schwannomas
2. Multiple Meningiomas 3. Gliomas 4. Hamartomas |
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Rare (1/40,000) Autosomal Dominant disorder that is due to a mutation on chr. 22 in which a tumor suppressor gene, Merlin, is mutated
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NF-2
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Schwannoma in NF-2
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What is seen here?
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Autosomal dominant disorder that is due to either a mutation of the Hamartin gene on chr. 9 or the Tuberin gene on chr. 16
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Tuberous Sclerosis
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Disorders that has Hamartomas in the following areas:
-Cysts = liver, kidneys, pancreas -Myomas = heart, lungs -Cutaneous = angiofibromas, shagreen patches, ash-leaf spots, subungal fibromas |
Tuberous Sclerosis
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Disorder that has Hamartomas in the CNS that include Cortical Tubers (neurons & cells of intermediate phenotype) & Subependymal Nodules (candle guttering)
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Tuberous Sclerosis
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Tuberous Sclerosis
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What disorder is this?
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Tuberous Sclerosis
-subependymal nodules = hamartomas |
What disorder is this?
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Disorder that has a neoplasm called a Subependymal Giant Cell Astrocytoma
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Tuberous Sclerosis
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Tuberous Sclerosis
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What disorder is this?
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Mental retardation + Hamartomas in the brain & kidneys
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Tuberous Sclerosis
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What symptoms does Tuberous Sclerosis cause?
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Mental Retardation & Seizures
Hypopigmented skin lesions = "ash leaf" lesions Angiolipomas in the kidneys |
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Autosomal dominant syndrome that has a mutation of a tumor suppressor gene on chr. 3
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von Hippel-Lindau
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Renal cell CA + Cavernous Hemangiomas + Adenomas = ?
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von Hippel-Lindau
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Where do Hemangioblastomas occur in von Hippel-Lindau syndrome?
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1. Cerebellum
2. Retina 3. Brain stem 4. Spinal Cord |
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Hemangioblastoma of the Cerebellum with Retinal Angiomas = ?
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von Hippel-Lindau
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What can Hemangioblastoma's in von Hippel-Lindau syndrome produce?
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EPO -> polycythemia
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Hemangioblastoma in von Hippel-Lindau syndrome
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What is seen here?
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Hemangioblastoma in von Hippel-Lindau
-foamy cells & high vascularity |
What syndrome is this seen in?
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Neurofibromatosis-1
-Lisch nodules Neurofibromas Gliomas of the Optic Nerve Cafe au lait spots |
What syndrome is this seen in?
What other clinical manifestations does this syndrome have? |