Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neville and Damm > Ch12 Soft Tissue Tumors - Getting Nervous > Flashcards

Ch12 Soft Tissue Tumors - Getting Nervous Flashcards

1
Q

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

A

25-48%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

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

A

auditory-vestibular (CN VIII)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

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

A

schwannomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A

neurofibromatosis type II (NF2)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the genetic disorder where multiple schwannomas occur?

A

schwannomatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Whats the alternate name for a schwannoma?

A

Neurilemoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

not usually symptomatic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Schwannoma: age range? most common site?

A

young and middle-aged adults…tongue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

NF2 is what type of inheritance?

A

autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

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

A

acoustic neuromas…NF2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

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

A

schwannomas…meningiomas and gliomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A

neurofibromas…café au lait

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A

deafness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A

SMARCB1…22

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

Antoni A….”usually” encapsulated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

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

A

Antoni A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

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

A

verocay bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Verocay bodies consist of reduplicated ________ and ________ processes.

A

basement membrane and cytoplasmic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

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

A

B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

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

A

S-100 protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

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

A

ANCIENT schwannoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What 5 features can be present in an ANCIENT schwannoma?

A
  1. hemorrhage 2.hemosiderin 3.inflammation 4.fibrosis 5. nuclear atypia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

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

A

plexiform schwannoma (lol)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

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

A

Plexiform schwannoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Plexiform schwannoma is occasionally associated with what two entities?

A

NF2 or schwannomatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Schwannoma: recurrence? malignant transformation?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Vestibular schwannomas in patients with ___ are difficult to manage.

A

NF2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is the most common type of peropheral nerve neoplasm?

A

neurofibroma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What are the two main cells that comprise a neurofibroma?

A

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

32
Q

Solitary neurofibromas are most common in what age group?

A

young adults

33
Q

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

A

the skin…tongue and buccal mucosa

34
Q

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

A

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

35
Q

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

A

wavy…MAST

36
Q

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

A

silver

37
Q

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

A

S-100 protein

38
Q

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

A

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

39
Q

NF1 accounts for __ - __ % of neurofibromatosis cases

A

85-97%

40
Q

What is the tumor suppressor gene product of the NF1 gene?

What chromosome does the NF1 gene occur on?

A

neurofibromin…….17q11.2

41
Q

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

A

2 or more

42
Q

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

A

6…café au lait…5..15

43
Q

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

A

2…PLEXIFORM

44
Q

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

A

axillary or inguinal

45
Q

Diagnostic criteria for NF1: Optic _____

A

glioma

46
Q

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

A

Lisch

47
Q

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

A

osseous

48
Q

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

A

first

49
Q

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

A

elephantiasis neuromatosa

50
Q

The ________ variant of neurofibroma, which feels

like a “________,” is considered PATHOGNOMONIC for NF1.

A

plexiform….bag of worms

51
Q

When do NF1 tumors typically begin to manifest?

A

puberty

52
Q

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

A

2/3 mild disease…pregnancy

53
Q

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 _____

A

smooth edge: California….Maine

54
Q

Coast of California

A

NF1

55
Q

Coast of Maine

A

Polyostotic fibrous dysplasia

56
Q

What is the clinical term for freckling of the axilla?

A

Crowe sign

57
Q

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

A

Lisch nodules: NEARLY ALL NF1 pts

58
Q

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

A

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

59
Q

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?

A

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

60
Q

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

A

25-37%

61
Q

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.

A

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

62
Q

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.

A

hemifacial hyperplasia…Noonan….central giant cell granulomas

63
Q

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?

A

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

64
Q

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

A

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

65
Q

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

A

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

66
Q

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

A

elephant man

67
Q

What are the 4 places affected by MEN1?

A

parathyroid, pancreatic islets, anterior pituitary, and adrenal cortex

68
Q

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

A

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

69
Q

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

A

RET…10

70
Q

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

A

other neuroendocrine tumors

71
Q

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

A

MTC, adrenal pheochromocytomas, primary hyperparathyroidism

72
Q

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

A

mucosal neuromas

73
Q

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

A

marfanoid

74
Q

Are neurofibromas encapsulated?

A

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

75
Q

What is the alternate name for NF1?

A

von Recklinghausen disease of the skin

76
Q

How many types of neurofibromatosis are there?

A

at least 8 forms

77
Q

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

A

50%

Neville and Damm (72 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions