Pathology - S2B4 - Neuro 4

Front 1

Neurofibroma

You would need to resect that part of the nerve which would probably cause some deficits

Back 1

What type of tumor does this exemplify? What kind of treatment would be needed?

Front 2

Schwannoma

Tumor could probably be teased off from the nerve, leaving it intact

Back 2

What type of tumor does this exemplify? What type of treatment would this need?

Front 3

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)

Back 3

Neurofibromas

Front 4

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

Back 4

Plexiform

Malignant Peripheral Nerve Sheath Tumor (MPNST)

Front 5

Neurofibromas

Back 5

What are seen in these pictures?

Front 6

A moderately firm, usually benign, unencapsulated, slow-growing heterogeneous tumors of the nervous system arising from the supporting cells (Schwann cells) of peripheral nerves

Back 6

Neurofibroma

Front 7

How are Schwannoma's & Neurofibromas different?

Back 7

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

Front 8

What syndrome are Schwannomas associated with?

Back 8

Neurofibromatosis-2

Front 9

Where do Schwannomas typically occur?

Back 9

1. CN VIII = Vestibular branch = Acoustic neuroma
2. CN V (Trigeminal)
3. Spinal nerve roots
4. Peripheral nerves

Front 10

What is the pathology seen in Schwannomas?

Back 10

Encapsulated

Micro:
-Antoni A = interlacing bundles of elongated cells with palisading nuclei
-Antoni B = looser, less cellular pattern

Front 11

Schwannoma

Back 11

What is this seen in?

Front 12

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

Back 12

What is this showing?

Front 13

Autosomal dominant disorder that is caused by a mutation of a tumor suppressor gene located on chr. 17

Back 13

Neurofibromatosis-1

Front 14

What are the clinical features of Neurofibromatosis-1?

Back 14

1. Neurofibromas
2. Gliomas of the Optic nerve
3. Lisch nodules = pigmented nodules of the iris
4. Cafe-au-lait spots

Front 15

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?

Back 15

Neurofibromatosis-1

Front 16

Neurofibroma in a NF-1 patient

Back 16

What is this showing?

Front 17

Malignant peripheral nerve sheath tumors (MPNST) that arose from from a Neurofibroma

Back 17

What is this showing?

Front 18

Syndrome in which the normal product is Neurofibromin, a tumor suppressor gene....but is mutated

Back 18

NF-1

Front 19

What are the clinical manifestations of NF-2?

Back 19

1. Bilateral Acoustic Schwannomas
2. Multiple Meningiomas
3. Gliomas
4. Hamartomas

Front 20

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

Back 20

NF-2

Front 21

Schwannoma in NF-2

Back 21

What is seen here?

Front 22

Autosomal dominant disorder that is due to either a mutation of the Hamartin gene on chr. 9 or the Tuberin gene on chr. 16

Back 22

Tuberous Sclerosis

Front 23

Disorders that has Hamartomas in the following areas:
-Cysts = liver, kidneys, pancreas
-Myomas = heart, lungs
-Cutaneous = angiofibromas, shagreen patches, ash-leaf spots, subungal fibromas

Back 23

Tuberous Sclerosis

Front 24

Disorder that has Hamartomas in the CNS that include Cortical Tubers (neurons & cells of intermediate phenotype) & Subependymal Nodules (candle guttering)

Back 24

Tuberous Sclerosis

Front 25

Tuberous Sclerosis

Back 25

What disorder is this?

Front 26

Tuberous Sclerosis
-subependymal nodules = hamartomas

Back 26

What disorder is this?

Front 27

Disorder that has a neoplasm called a Subependymal Giant Cell Astrocytoma

Back 27

Tuberous Sclerosis

Front 28

Tuberous Sclerosis

Back 28

What disorder is this?

Front 29

Mental retardation + Hamartomas in the brain & kidneys

Back 29

Tuberous Sclerosis

Front 30

What symptoms does Tuberous Sclerosis cause?

Back 30

Mental Retardation & Seizures

Hypopigmented skin lesions = "ash leaf" lesions

Angiolipomas in the kidneys

Front 31

Autosomal dominant syndrome that has a mutation of a tumor suppressor gene on chr. 3

Back 31

von Hippel-Lindau

Front 32

Renal cell CA + Cavernous Hemangiomas + Adenomas = ?

Back 32

von Hippel-Lindau

Front 33

Where do Hemangioblastomas occur in von Hippel-Lindau syndrome?

Back 33

1. Cerebellum
2. Retina
3. Brain stem
4. Spinal Cord

Front 34

Hemangioblastoma of the Cerebellum with Retinal Angiomas = ?

Back 34

von Hippel-Lindau

Front 35

What can Hemangioblastoma's in von Hippel-Lindau syndrome produce?

Back 35

EPO -> polycythemia

Front 36

Hemangioblastoma in von Hippel-Lindau syndrome

Back 36

What is seen here?

Front 37

Hemangioblastoma in von Hippel-Lindau
-foamy cells & high vascularity

Back 37

What syndrome is this seen in?

Front 38

Neurofibromatosis-1
-Lisch nodules

Neurofibromas
Gliomas of the Optic Nerve
Cafe au lait spots

Back 38

What syndrome is this seen in?

What other clinical manifestations does this syndrome have?