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Oral Path I > Neural Tissue Tumors > Flashcards

Neural Tissue Tumors Flashcards

1
Q

what neural tissue tumors are categorized as a hyperplasias?

A

traumatic neuromas

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2
Q

what neural tissue tumors are considered hamartomas?

A

multiple endocrine neoplasia syndrome (MEN syndrome)

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3
Q

what is a harmartoma?

A

a developmental anomaly

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4
Q

what is MEN syndrome?

A

an AUTOSOMAL DOMINANT condition involving the endocrine system, with one variant (MEN-IIB) having oral manifestations consisting of multiple neuromas on the mucosal surfaces, lips and anterior dorsal tongue

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5
Q

what neural tissue tumors are considered benign neoplasms?

A
  • neurileomas (Schwannoma)
  • neurofibromas
  • granular cell tumor
  • congenital gingival granular cell tumor
  • neuroectodermal tumor of infancy
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6
Q

what neural tissue tumor is considered a malignant neoplasm

A

malignant neoplasms of nerve origin (neurogenic sarcomas)

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7
Q

key features of traumatic neuromas

A
  • these are NOT TRUE NEOPLASMS
  • they are HYPERPLASIA
  • most common site MENTAL FORAMEN
  • PAINFUL NODULAR GROWTH
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8
Q

what are traumatic neuromas?

A

PAINFUL nodular proliferation of the nerve sheath and fibrous tissue that result from as severed nerve attempting to reunite

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9
Q

what glands does MEN syndrome affect?

A

endocrine glands (PARATHYROID, PANCREAS, THYROID, ADRENAL GLAND)

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10
Q

what chromosome is affected in the MEN IIB syndrome?

A

chromosome 10

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11
Q

multiple oral neuromas are often the first indicator of what syndrome?

A

MEN syndrome

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12
Q

what germline mutation causes MEN IIB syndrome?

A

RET proto-onco gene

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13
Q

why is it so important to be able to recognize MEN syndrome

A

because it has the potential to malignantly transform into fatal conditions such as THYROID CARCINOMA or PLEOCHROMOCYTOMA

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14
Q

what are neurilemomas?

A

benign, PAINLESS, fibroblastic proliferation of SCHWANN CELLS (nuerilemma cells) that produce distinctive patterns referred to as ANTONI A and ANTONI B tissue

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15
Q

key features of neurilemomas

A
  • affects SCHWANN CELLS
  • PAINLESS
  • form around IA NERVE and DORSAL SURFACE of TONGUE
  • can affect the ACOUSTIC nerve (CN VIII) - called acoustic neuroma
  • produced distinct tissue patterns called ANTONI A and ANTONI B
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16
Q

what is the difference between ANTONI A and ANTONI B tissue patterns of neurilemomas?

A
  • antoni A - are well organized and leave behind acellular pink areas known as VEROCAY BODIES
  • antoni B - have NO ORGANIZATION
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17
Q

in order to diagnose do you need to see both ANTONI A and ANTONI B histologic tissue patterns

A

NO, you usually see both antoni A and antoni B together, but the presence of ANTONI A alone is DIAGNOSTIC

18
Q

what are neurofibromas?

A

benign neoplasm arising from PERINEURAL FIBROBLASTS leading to increased COLLAGEN

19
Q

key features of neurofibromas

A
  • arise from PERINURAL FIBROBLASTS
  • usually single lesions, but can have MULTIPLE lesions
  • histology: lots of COLLAGEN and SPINDLE CELLS
20
Q

MULTIPLE neurofibromas are associated with what condition?

A

multiple neruofribromatosis, VON RECKLINGHAUSEN DISEASE

21
Q

key features of von Reckilnghausen disease

A
  • aka neurofibromatosis type I
  • multiple skin and oral neurofibromas
  • CAFE AU LAIT SPOTS are common
  • AUTOSOMAL DOMINANT
  • mutation in NF GENE (tumor suppressor factor), located on CHROMOSOME 17
  • there is a risk of malignant transformation
  • TX: excision (malignant recurrence with a vengeance)
22
Q

how would you diagnose von Reckilnghausen disease?

A

there are 7 diagnostic criteria and you must have 2 for diagnosis:

1) 6 or more cafe au lait spots
2) freckling of axial or inguinal regions
3) optic gioma
4) 2 or more Lisch nodules (iris hemotomas)
5) distinct osseous lesion
6) first degree realative with NF1 dx
7) 2 or more neurofibromas

23
Q

key features of granular cell tumors

A
  • classified as a NERVE TUMOR
  • thought to arise from SCHWANN CELLS
  • predilection for middle aged males
  • 70% of head and neck lesions occur in mouth, most commonly on LATERAL** and DORSAL SURFACES of the TONGUE
  • PAINLESS
  • SUBMUCOSAL MASS
24
Q

are granular cell tumor malignant?

A

NO!

25
Q

granular cell tumors may be confused with what other disease?

A
  • SQUAMOUS CELL CARCINOMA

- granulomas

26
Q

what is a congenital gingival granular cell tumor?

A

a granular cell tumor on the gingival of newborns

27
Q

do congenital gingival gingival granular cell tumors have PSEUDOEPITHELIALIOMATOUS hyperplasia?

A

NO!!! That is a histological distinction of granular cell tumors in adults. PSEUDOEPITHELIALIOMATOUS hyperplasia is NOT see in congenital gingival granular cell tumors

28
Q

who are more at risk of developing congenital granular cell tumors, girls or boys?

A

newborn GIRLS

29
Q

what is the most common location of congenital granular cell tumors?

A

in the MX and MN of newborn GIRLS

30
Q

are congenital granular cell tumors sessile or pedunculated?

A

PEDUNCULATED and can become quite LARGE if left untreated

31
Q

what cells are melanotic neuroectodermal tumors of infancy derived from?

A

NEURAL CREST CELLS

32
Q

how can you histologically unique about melanotic neuroectodermal tumors of infancy?

A

they have two distinct cell types LARGE PIGMENTED cells, producing melanin and SMALL DARK cells

33
Q

what age group are neuroectodermal tumors more common in?

A

INFANTS (less then 6 months old)

34
Q

how do melanotic neuroectodermal tumors of infancy present clinically?

A

most often present in the MAXILLA (may present in other places) as PIGMENTED (BLACK or BROWN) nodules

35
Q

what neural tissue tumor is know to be malignant?

A

malignant neoplasms of nerve origin (neurogenic sarcomas)

36
Q

malignant neoplasms of nerve origin (neurogenic sarcomas) arise from what cells?

A

FIBROBLAST or SCHWANN CELLS

37
Q

are malignant neoplasms of nerve origin (neurogenic sarcomas) common or rare?

A

they RARELY occur in the oral cavity

38
Q

what is the rate of hematogenous metastasis for malignant neoplasms of nerve origin (neurogenic sarcomas)?

A

50%

39
Q

do neurogenic sarcomas have potential for metastasis?

A

YES!! the are also known as malignant neoplasms of nerve origin and have a 50% chance of hematogenous metastases

40
Q

what is the prognosis of malignant neoplasms of nerve origin (neurogenic sarcomas)?

A

BAD, very POOR prognosis!! most patients die of their disease

41
Q

malignant neoplasms of nerve origin (neurogenic sarcomas) are often associated with…

A

nerve trunk lesions → PAIN

Oral Path I (5 decks)

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