24 Neoplastic - Other Flashcards

1
Q

What % of H&N paragangliomas are familial

A

7-10%

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2
Q

What is m/c paraganglioma of H&N

A

carotid body tumor

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3
Q

What percent of carotid body tumors are multicentric

A

10% (30-40% hereditary form)

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4
Q

What is inheritance pattern of familial carotid body tumors

A

Auto Dom but only the genes passed from the paternal side are expressed (maternal genetic imprinting)

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5
Q

What is a “nonchromaffin” paraganglioma

A

not secreting significant amounts of catecholamines

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6
Q

What percent of cervical paragangliomas secrete

A

5%

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7
Q

What is the gene a/w cervical paragangliomas and what percent have the underlying germline mutation

A

Succinate dehydrogenase SDH; 40%

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8
Q

What is Shamblin’s classification for carotid body tumors

A

Group I: Small and easily excised.
Group II: Adherent to the vessels; resectable with careful subadventitial dissection.
Group III: Encase the carotid; require partial or complete vessel resection

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9
Q

Dissection of carotid body tumors is in what plane

A

subadventitial

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10
Q

What are the 2 types of temporal bone paragangliomas

A

Glomus jugulare involving the adventitia of the jugular bulb and glomus tympanicum involving Jacobson’s nerve (jugulotympanic glomus if unable to discern site of origin).

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11
Q

How do glomus tumors differ clinically from carotid body tumors

A

More common in females, less likely to secrete or met, and are more radiosensitive

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12
Q

What is the primary advantage of stereotactic radiosurgery for tx of recurrent glomus jugulare tumors compared with surgery and conventional RT

A

Lower incidence of cranial nerve injury

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13
Q

When is stereotactic radiosurgery contraindicated in the treatment of recurrent glomus jugulare tumors?

A

For larger tumors (>3-4 cm)

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14
Q

How do most glomus jugulare tumors respond to external beam radiation

A

Less than 50% show tumor regression radiographically; lack of tumor growth is more common

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15
Q

Risk of esoph CA in pts who smoke and drink vs those who don’t

A

100x higher

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16
Q

Which areas of world is incidence of esoph CA highest

A

Middle east, Southern and eastern africa, Northern China

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17
Q

RF for esoph CA

A

Tobacco, etoh, achalasia, Plummer-vinson, prior HNC, tylosis, Barrett’s dz

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18
Q

What are the clinical features of Plummer-Vinson syndrome

A
  • IDA
  • Upper esoph web
  • HypoT
  • Glossitis/cheilitis
  • Gastritis
  • Dysphagia
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19
Q

In pts with Plummer-Vinson synd where is SCC of esoph most likely to occur

A

Postcricoid area

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20
Q

Metaplasia of the distal esoph is otherwise known as

A

Barrett’s esophagus

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21
Q

What percent of people with gastroesophageal reflux disease have Barrett’s esophagus and what percent of these people will develop adenocarcinoma?

A

5%; 5-10%

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22
Q

CA of cervical esoph is usu which type

A

SCC

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23
Q

What is usual cause of death from esoph CA

A

aspiration PNA

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24
Q

When do pts w/ synovial sarcoma usu present

A

25-36 yo

25
Q

Where are most synovial sarcomas of the H&N

A

HP and PPS

26
Q

What is the incidence of regional mets in synovial sarcomas of the H&N

A

12.5%

27
Q

What is the incidence of local recurrence

A

60-90% usu within 2 yrs

28
Q

What is the m/c cause of death from synovial sarcoma of H&N

A

lung mets

29
Q

what is primary mode of tx for synovial sarcoma of H&N

A

Wide excision and postop RT

30
Q

What is 5 yr survival rate

A

40-50%

31
Q

What prognostic significance does presence of microcalcifications have

A

better prog

32
Q

What is the 2nd leading cause of death among kids age 1-14?

A

cancer

33
Q

What is m/c solid malig tumor in infants < 1 yo

A

Neuroblastoma

34
Q

What are the precursor cells of neuroblastoma

A

Neural crest cells

35
Q

What is the survival rate after complete excision of lesions in kids < 1 yo?

A

90%

36
Q

What type of nystagmus is often seen in kids with neuroblastoma

A

Opsoclonus

37
Q

What is m/c H&N tumor of children

A

Lymphoma

38
Q

In what age groups is Hodgkin’s lymphoma m/c

A

Bimodal peak incidence w/ one peak in 15-34 yo and another in later adulthood

39
Q

What percent of Hodgkin’s lymphoma cases are a/w EBV

A

40%

40
Q

T/F: Axillary, inguinal, and Waldeyer’s ring involvement is uncommon in pts w/ Hodgkin’s lymphoma

A

True

41
Q

Involvement of 2 or more LN sites on the same side of the diaphragm is designated as which stage according to the Ann Arbor system?

A

Stage II

42
Q

Which lymphoma accounts for 50% of childhood malignancies in equatorial Africa

A

Burkitt’s lymphoma

43
Q

What is the m/c soft tissue sarcoma of the H&N in kids

A

Rhabdomyosarcoma

44
Q

In what age groups is RMS m/c

A

2-5 and 15-19

45
Q

What is the tx for RMS

A

Multimodality; primary CRT followed by surgery for recurrent or residual dz

46
Q

Involvement in which area of the H&N by RMS has best prog

A

orbit

47
Q

What is the m/c type of well diff thyroid carcinoma in kids

A

papillary

48
Q

M/c benign neoplasm of larynx in kids

A

RRP

49
Q

What percent of pts with RRP develop distal tracheal and pulmonary spread of papillomas

A

5%

50
Q

High expression of which nuclear Ag is significantly a/w distal tracheobronchial spread and inc freq of recurrences

A

Ki-67

51
Q

What % of pts with RRP require trach

A

15%

52
Q

What % of pts with distal spread of RRP have had a previous trach

A

95%

53
Q

What is the incidence of stomal papilloma recurrence rate after trach for RRP

A

> 50%

54
Q

What is m/c subtypes of HPV isolated from RRP

A

6 and 11 (found in >95%)

55
Q

Which of these subtypes is a/w a more aggressive dz course

A

11

56
Q

What are the m/c resp complications of distal RRP

A

Pneumatocele, abscess, and tracheal stenosis

57
Q

Severe cerebellar sx with a nl MRI suggests what condition

A

Paraneoplastic cerebellar degeneration

58
Q

What are the 2 primary subtypes of paraneoplastic cerebellar degeneration

A

Vestibulocerebellar syndrome and opsoclonus-myoclonus syndrome

59
Q

Which malig most commonly cause paraneoplastic cerebellar degeneration

A

In adults, ovarian, uterine, breast, and small cell lung CA

In kids, neuroblastoma