Ch12 Soft Tissue Tumors - Getting Nervous Flashcards by Austin Shackelford

What % range of schwannomas occur in the head and neck?

25-48%

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Bilateral schwannomas of the _________ nerve are a characteristic feature of the hereditary condition, neurofibromatosis type II (NF2).

auditory-vestibular (CN VIII)

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Bilateral ________ of the auditory-vestibular nerve are a characteristic feature of the hereditary condition, neurofibromatosis type II (NF2).

schwannomas

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Bilateral schwannomas of the auditory-vestibular nerve are a characteristic feature of the hereditary condition _____________.

neurofibromatosis type II (NF2)

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What is the genetic disorder where multiple schwannomas occur?

schwannomatosis

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Whats the alternate name for a schwannoma?

Neurilemoma

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As a schwannoma grows, it pushes the nerve aside..are they symptomatic?

not usually symptomatic

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Schwannoma: age range? most common site?

young and middle-aged adults…tongue

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Can an intraosseous schwannoma cause boney expansion? Where are they most common? Do they produce pain/paresthesia?

you bet your ass they can…posterior mandible…do not usually cause pain/paresthesia

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NF2 is what type of inheritance?

autosomal dominant

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NF2 is caused by a mutation of a WHAT tumor suppressor gene? What chromosome? What is the protein this gene is codes for?

The NF2 tumor supressor gene lol….chromosome 22….MERLIN protein

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Bilateral schwannomas AKA (“________”) of the vestibular nerve are signs of what condition?

acoustic neuromas…NF2

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NF2 patients also develop ________ of peripheral nerves, plus _______ and _______ of the central nervous system (CNS)

schwannomas…meningiomas and gliomas

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NF2: on occasion, ________ and _______ skin pigmentation may be observed.

neurofibromas…café au lait

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NF2 characteristic symptoms include progressive sensorineural ________, dizziness, and tinnitus

deafness

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Schwannomatosis is related to a mutation of the _________ gene on chromosome ___.

SMARCB1…22

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Which Antoni?: Streaming fascicles of spindle-shaped Schwann cells…. ALSO - are schwannomas encapsulated?

Antoni A….”usually” encapsulated

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Which Antoni?: These cells often form a palisaded arrangement around central acellular, eosinophilic areas

Antoni A

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In Antoni A schwannomas: cells often form a palisaded arrangement around central acellular, eosinophilic areas called ________

verocay bodies

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Verocay bodies consist of reduplicated ________ and ________ processes.

basement membrane and cytoplasmic

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Antoni ___ tissue is less CELLULAR and less ORGANIZED; the spindle cells are randomly arranged within a loose, myxomatous stroma

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The tumor cells in a schwannoma will show a diffuse, positive immunohistochemical reaction for ______

S-100 protein

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What is the term for a schwannoma that has undergone degenerative changes?

ANCIENT schwannoma

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What 5 features can be present in an ANCIENT schwannoma?

hemorrhage 2.hemosiderin 3.inflammation 4.fibrosis 5. nuclear atypia

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Which variant of schwannoma is characterized grossly and microscopically by a multinodular, plexiform growth pattern?

plexiform schwannoma (lol)

Which variant of schwannoma is occasionally associated with NF2 or Schwannomatosis?

Plexiform schwannoma

Plexiform schwannoma is occasionally associated with what two entities?

NF2 or schwannomatosis

Schwannoma: recurrence? malignant transformation?

"should not recur" and malignant transformation "does not occur" or is "extremely rare"

Vestibular schwannomas in patients with ___ are difficult to manage.

NF2

What is the most common type of peropheral nerve neoplasm?

neurofibroma

What are the two main cells that comprise a neurofibroma?

schwann cells (neuro), PERINEURAL fibroblasts (fibroma) (lol)

Solitary neurofibromas are most common in what age group?

young adults

What is the most common location overall for a neurofibroma? What about inside the oral cavity (2 sites)?

the skin...tongue and buccal mucosa

What is the radiographic appearance of a bony neurofibroma (yes, they occur)?

well to poorly demarcated uni or multilocular radiolucency (ha very specific)

Neurofibroma: composed of interlacing bundles of spindleshaped cells that often exhibit ____ nuclei..._____ cells tend to be numerous and can be a helpful diagnostic feature

wavy...MAST

Neurofibroma: Sparsely distributed small axons usually can be demonstrated within the tumor tissue by using ____ stains.

silver

Neurofibroma: Immunohistochemically, the tumor cells show a scattered, positive reaction for _______

S-100 protein

Neurofibroma: solitary recurrence following excision..what special consideration should be made? Malignant transformation?

RARE, evaluate clinically for neurofibromatosis..malignancy can occur, but risk is low esp compared to neurofibromatosis

NF1 accounts for __ - __ % of neurofibromatosis cases

85-97%

What is the tumor suppressor gene product of the NF1 gene? | What chromosome does the NF1 gene occur on?

neurofibromin.......17q11.2

Diagnostic criteria for NF1: How many of the 7 diagnostic criteria need to be met?

2 or more

Diagnostic criteria for NF1: ____ or more ______ macules more than __ mm in greatest diameter in prepubertal persons and more than __ mm in greatest diameter in postpubertal persons

6...café au lait...5..15

Diagnostic criteria for NF1: ___ or more neurofibromas of any type or ONE ______ neurofibroma

2...PLEXIFORM

Diagnostic criteria for NF1: Freckling in the _______ OR _______ regions

axillary or inguinal

Diagnostic criteria for NF1: Optic _____

glioma

Diagnostic criteria for NF1: Two or more _____ nodules (iris hamartomas)

Lisch

Diagnostic criteria for NF1: A distinctive ________ lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis

osseous

Diagnostic criteria for NF1: A _____-degree relative (parent, sibling, or offspring) with NF1, based on the previously mentioned criteria

first

The clinical appearance of neurofibromatosis can vary from small papules to larger soft nodules to massive baggy, pendulous masses called _________

elephantiasis neuromatosa

The ________ variant of neurofibroma, which feels | like a “________,” is considered PATHOGNOMONIC for NF1.

plexiform....bag of worms

When do NF1 tumors typically begin to manifest?

puberty

What fraction of NF1 pts have mild disease? What life situation can cause more rapid growth?

2/3 mild disease...pregnancy

In NF1, this pigmentation typically has an edge (“coast of _______”), in contrast to the border of the café au lait spots that may occur with polyostotic fibrous dysplasia resemble the coast of _____

smooth edge: California....Maine

Coast of California

NF1

Coast of Maine

Polyostotic fibrous dysplasia

What is the clinical term for freckling of the axilla?

Crowe sign

What translucent brown-pigmented spots on the iris are found in NF1 pts? How many of NF1 pts have these?

Lisch nodules: NEARLY ALL NF1 pts

What is the most common general medical problem in NF1 patients? What is this condition often secondary to (3 possibilities)?

HTN...secondary to coarctation of the aorta, pheochromocytoma, or renal artery stenosis

NF1 presents in the oral cavity in __-__% of patients...what is the most common finding in the oral cavity? What % of NF1 patients have this finding?

72-92% of patients...enlarged fungiform papillae...up to 50% have it

What % (range) of NF1 patients develop NFs in the mouth?

25-37%

Radiographic findings in NF1: enlargement of the mandibular _______, enlargement or branching of the mandibular _____ , _______ bone density, concavity of the ______ surface of the ramus, and increase in dimension of the _______ notch. Cephalometric analysis often shows a ______ length of the mandible, maxilla, and cranial base.

foramen..canal...increased...medial...coronoid...short

Odd clinical variants of NF1: unilateral enlargement that mimics ________. In addition, several patients with NF1 have been described with associated _______ syndrome or with _________ of the jaw.

hemifacial hyperplasia...Noonan....central giant cell granulomas

What is the most common malignancy in NF1 patients? (main name and 2 alternate names) What % of patients have been reported? WHERE are they most commonly seen?

Malignant peripheral nerve sheath tumor (neurofibrosarcoma, malignant schwannoma)...5%...trunk and extremities

Besides a malignant peripheral nerve sheath tumor, what 5 malignancies have been associated with NF1?

1.CNS tumors 2.pheochromocytoma 3.leukemia 4.rhabdomyosarcoma 5.wilms tumor

What is the life exectancy of an NF1 pt compared to the general population?

8-15 years shorter due to vascular disease and malignant neoplasms

Genetic council is extremely important for NF1 pts so they learn that they DONT have Proteus syndrome...AKA the _______ syndrome

elephant man

What are the 4 places affected by MEN1?

parathyroid, pancreatic islets, anterior pituitary, and adrenal cortex

What are the three disorders associated with MEN2? What do they all have in common?

MEN2A, MTC, MEN2B...they all lead to development of MTC (medullary thyroid carcinoma)

The MEN2 disorders are caused by mutations at various sites of the ____ proto-oncogene on chromosome ___

RET...10

Patients with familial MTC syndrome develop MTC but are not at increased risk for _______.

other neuroendocrine tumors

Patients with MEN type 2A are at increased risk for ____ (over 95% of patients), __________(50% of patients), and ___________ (20% to 30% of patients).

MTC, adrenal pheochromocytomas, primary hyperparathyroidism

Which MEN2 disorder has oral manifestations? What are the oral manifestations?

mucosal neuromas

Patients with MEN type 2B usually have a ________ body build

marfanoid

Are neurofibromas encapsulated?

No, often well-circumscribed though (esp in the perineurium, when outside the perineurium they can be less demarcated)

What is the alternate name for NF1?

von Recklinghausen disease of the skin

How many types of neurofibromatosis are there?

at least 8 forms

What % of patients with NF1 have NO family history and represent new mutations?

50%