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38 Cards in this Set

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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?
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?
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)
Neurofibromas
What type of Neurofibroma is associated with NF-1? What may this transform into?
Plexiform

Malignant Peripheral Nerve Sheath Tumor (MPNST)
Neurofibromas
What are seen in these pictures?
A moderately firm, usually benign, unencapsulated, slow-growing heterogeneous tumors of the nervous system arising from the supporting cells (Schwann cells) of peripheral nerves
Neurofibroma
How are Schwannoma's & Neurofibromas different?
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
What syndrome are Schwannomas associated with?
Neurofibromatosis-2
Where do Schwannomas typically occur?
1. CN VIII = Vestibular branch = Acoustic neuroma
2. CN V (Trigeminal)
3. Spinal nerve roots
4. Peripheral nerves
What is the pathology seen in Schwannomas?
Encapsulated

Micro:
-Antoni A = interlacing bundles of elongated cells with palisading nuclei
-Antoni B = looser, less cellular pattern
Schwannoma
What is this seen in?
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
What is this showing?
Autosomal dominant disorder that is caused by a mutation of a tumor suppressor gene located on chr. 17
Neurofibromatosis-1
What are the clinical features of Neurofibromatosis-1?
1. Neurofibromas
2. Gliomas of the Optic nerve
3. Lisch nodules = pigmented nodules of the iris
4. Cafe-au-lait spots
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?
Neurofibromatosis-1
Neurofibroma in a NF-1 patient
What is this showing?
Malignant peripheral nerve sheath tumors (MPNST) that arose from from a Neurofibroma
What is this showing?
Syndrome in which the normal product is Neurofibromin, a tumor suppressor gene....but is mutated
NF-1
What are the clinical manifestations of NF-2?
1. Bilateral Acoustic Schwannomas
2. Multiple Meningiomas
3. Gliomas
4. Hamartomas
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
NF-2
Schwannoma in NF-2
What is seen here?
Autosomal dominant disorder that is due to either a mutation of the Hamartin gene on chr. 9 or the Tuberin gene on chr. 16
Tuberous Sclerosis
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
Disorder that has Hamartomas in the CNS that include Cortical Tubers (neurons & cells of intermediate phenotype) & Subependymal Nodules (candle guttering)
Tuberous Sclerosis
Tuberous Sclerosis
What disorder is this?
Tuberous Sclerosis
-subependymal nodules = hamartomas
What disorder is this?
Disorder that has a neoplasm called a Subependymal Giant Cell Astrocytoma
Tuberous Sclerosis
Tuberous Sclerosis
What disorder is this?
Mental retardation + Hamartomas in the brain & kidneys
Tuberous Sclerosis
What symptoms does Tuberous Sclerosis cause?
Mental Retardation & Seizures

Hypopigmented skin lesions = "ash leaf" lesions

Angiolipomas in the kidneys
Autosomal dominant syndrome that has a mutation of a tumor suppressor gene on chr. 3
von Hippel-Lindau
Renal cell CA + Cavernous Hemangiomas + Adenomas = ?
von Hippel-Lindau
Where do Hemangioblastomas occur in von Hippel-Lindau syndrome?
1. Cerebellum
2. Retina
3. Brain stem
4. Spinal Cord
Hemangioblastoma of the Cerebellum with Retinal Angiomas = ?
von Hippel-Lindau
What can Hemangioblastoma's in von Hippel-Lindau syndrome produce?
EPO -> polycythemia
Hemangioblastoma in von Hippel-Lindau syndrome
What is seen here?
Hemangioblastoma in von Hippel-Lindau
-foamy cells & high vascularity
What syndrome is this seen in?
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?