24 Neoplastic - Other

Question 1

What % of H&N paragangliomas are familial

Answer 1

7-10%

Question 2

What is m/c paraganglioma of H&N

Answer 2

carotid body tumor

Question 3

What percent of carotid body tumors are multicentric

Answer 3

10% (30-40% hereditary form)

Question 4

What is inheritance pattern of familial carotid body tumors

Answer 4

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

Question 5

What is a “nonchromaffin” paraganglioma

Answer 5

not secreting significant amounts of catecholamines

Question 6

What percent of cervical paragangliomas secrete

Answer 6

5%

Question 7

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

Answer 7

Succinate dehydrogenase SDH; 40%

Question 8

What is Shamblin’s classification for carotid body tumors

Answer 8

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

Question 9

Dissection of carotid body tumors is in what plane

Answer 9

subadventitial

Question 10

What are the 2 types of temporal bone paragangliomas

Answer 10

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).

Question 11

How do glomus tumors differ clinically from carotid body tumors

Answer 11

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

Question 12

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

Answer 12

Lower incidence of cranial nerve injury

Question 13

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

Answer 13

For larger tumors (>3-4 cm)

Question 14

How do most glomus jugulare tumors respond to external beam radiation

Answer 14

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

Question 15

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

Answer 15

100x higher

Question 16

Which areas of world is incidence of esoph CA highest

Answer 16

Middle east, Southern and eastern africa, Northern China

Question 17

RF for esoph CA

Answer 17

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

Question 18

What are the clinical features of Plummer-Vinson syndrome

Answer 18

• IDA
• Upper esoph web
• HypoT
• Glossitis/cheilitis
• Gastritis
• Dysphagia

Question 19

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

Answer 19

Postcricoid area

Question 20

Metaplasia of the distal esoph is otherwise known as

Answer 20

Barrett’s esophagus

Question 21

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

Answer 21

5%; 5-10%

Question 22

CA of cervical esoph is usu which type

Answer 22

SCC

Question 23

What is usual cause of death from esoph CA

Answer 23

aspiration PNA

Question 24

When do pts w/ synovial sarcoma usu present

Answer 24

25-36 yo

Question 25

Where are most synovial sarcomas of the H&N

Answer 25

HP and PPS

Question 26

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

Answer 26

12.5%

Question 27

What is the incidence of local recurrence

Answer 27

60-90% usu within 2 yrs

Question 28

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

Answer 28

lung mets

Question 29

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

Answer 29

Wide excision and postop RT

Question 30

What is 5 yr survival rate

Answer 30

40-50%

Question 31

What prognostic significance does presence of microcalcifications have

Answer 31

better prog

Question 32

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

Answer 32

cancer

Question 33

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

Answer 33

Neuroblastoma

Question 34

What are the precursor cells of neuroblastoma

Answer 34

Neural crest cells

Question 35

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

Answer 35

90%

Question 36

What type of nystagmus is often seen in kids with neuroblastoma

Answer 36

Opsoclonus

Question 37

What is m/c H&N tumor of children

Answer 37

Lymphoma

Question 38

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

Answer 38

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

Question 39

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

Answer 39

40%

Question 40

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

Answer 40

True

Question 41

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?

Answer 41

Stage II

Question 42

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

Answer 42

Burkitt's lymphoma

Question 43

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

Answer 43

Rhabdomyosarcoma

Question 44

In what age groups is RMS m/c

Answer 44

2-5 and 15-19

Question 45

What is the tx for RMS

Answer 45

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

Question 46

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

Answer 46

orbit

Question 47

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

Answer 47

papillary

Question 48

M/c benign neoplasm of larynx in kids

Answer 48

RRP

Question 49

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

Answer 49

5%

Question 50

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

Answer 50

Ki-67

Question 51

What % of pts with RRP require trach

Answer 51

15%

Question 52

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

Answer 52

95%

Question 53

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

Answer 53

> 50%

Question 54

What is m/c subtypes of HPV isolated from RRP

Answer 54

6 and 11 (found in >95%)

Question 55

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

Answer 55

11

Question 56

What are the m/c resp complications of distal RRP

Answer 56

Pneumatocele, abscess, and tracheal stenosis

Question 57

Severe cerebellar sx with a nl MRI suggests what condition

Answer 57

Paraneoplastic cerebellar degeneration

Question 58

What are the 2 primary subtypes of paraneoplastic cerebellar degeneration

Answer 58

Vestibulocerebellar syndrome and opsoclonus-myoclonus syndrome

Question 59

Which malig most commonly cause paraneoplastic cerebellar degeneration

Answer 59

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