24 Neoplastic - Other Flashcards
What % of H&N paragangliomas are familial
7-10%
What is m/c paraganglioma of H&N
carotid body tumor
What percent of carotid body tumors are multicentric
10% (30-40% hereditary form)
What is inheritance pattern of familial carotid body tumors
Auto Dom but only the genes passed from the paternal side are expressed (maternal genetic imprinting)
What is a “nonchromaffin” paraganglioma
not secreting significant amounts of catecholamines
What percent of cervical paragangliomas secrete
5%
What is the gene a/w cervical paragangliomas and what percent have the underlying germline mutation
Succinate dehydrogenase SDH; 40%
What is Shamblin’s classification for carotid body tumors
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
Dissection of carotid body tumors is in what plane
subadventitial
What are the 2 types of temporal bone paragangliomas
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).
How do glomus tumors differ clinically from carotid body tumors
More common in females, less likely to secrete or met, and are more radiosensitive
What is the primary advantage of stereotactic radiosurgery for tx of recurrent glomus jugulare tumors compared with surgery and conventional RT
Lower incidence of cranial nerve injury
When is stereotactic radiosurgery contraindicated in the treatment of recurrent glomus jugulare tumors?
For larger tumors (>3-4 cm)
How do most glomus jugulare tumors respond to external beam radiation
Less than 50% show tumor regression radiographically; lack of tumor growth is more common
Risk of esoph CA in pts who smoke and drink vs those who don’t
100x higher
Which areas of world is incidence of esoph CA highest
Middle east, Southern and eastern africa, Northern China
RF for esoph CA
Tobacco, etoh, achalasia, Plummer-vinson, prior HNC, tylosis, Barrett’s dz
What are the clinical features of Plummer-Vinson syndrome
- IDA
- Upper esoph web
- HypoT
- Glossitis/cheilitis
- Gastritis
- Dysphagia
In pts with Plummer-Vinson synd where is SCC of esoph most likely to occur
Postcricoid area
Metaplasia of the distal esoph is otherwise known as
Barrett’s esophagus
What percent of people with gastroesophageal reflux disease have Barrett’s esophagus and what percent of these people will develop adenocarcinoma?
5%; 5-10%
CA of cervical esoph is usu which type
SCC
What is usual cause of death from esoph CA
aspiration PNA
When do pts w/ synovial sarcoma usu present
25-36 yo
Where are most synovial sarcomas of the H&N
HP and PPS
What is the incidence of regional mets in synovial sarcomas of the H&N
12.5%
What is the incidence of local recurrence
60-90% usu within 2 yrs
What is the m/c cause of death from synovial sarcoma of H&N
lung mets
what is primary mode of tx for synovial sarcoma of H&N
Wide excision and postop RT
What is 5 yr survival rate
40-50%
What prognostic significance does presence of microcalcifications have
better prog
What is the 2nd leading cause of death among kids age 1-14?
cancer
What is m/c solid malig tumor in infants < 1 yo
Neuroblastoma
What are the precursor cells of neuroblastoma
Neural crest cells
What is the survival rate after complete excision of lesions in kids < 1 yo?
90%
What type of nystagmus is often seen in kids with neuroblastoma
Opsoclonus
What is m/c H&N tumor of children
Lymphoma
In what age groups is Hodgkin’s lymphoma m/c
Bimodal peak incidence w/ one peak in 15-34 yo and another in later adulthood
What percent of Hodgkin’s lymphoma cases are a/w EBV
40%
T/F: Axillary, inguinal, and Waldeyer’s ring involvement is uncommon in pts w/ Hodgkin’s lymphoma
True
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?
Stage II
Which lymphoma accounts for 50% of childhood malignancies in equatorial Africa
Burkitt’s lymphoma
What is the m/c soft tissue sarcoma of the H&N in kids
Rhabdomyosarcoma
In what age groups is RMS m/c
2-5 and 15-19
What is the tx for RMS
Multimodality; primary CRT followed by surgery for recurrent or residual dz
Involvement in which area of the H&N by RMS has best prog
orbit
What is the m/c type of well diff thyroid carcinoma in kids
papillary
M/c benign neoplasm of larynx in kids
RRP
What percent of pts with RRP develop distal tracheal and pulmonary spread of papillomas
5%
High expression of which nuclear Ag is significantly a/w distal tracheobronchial spread and inc freq of recurrences
Ki-67
What % of pts with RRP require trach
15%
What % of pts with distal spread of RRP have had a previous trach
95%
What is the incidence of stomal papilloma recurrence rate after trach for RRP
> 50%
What is m/c subtypes of HPV isolated from RRP
6 and 11 (found in >95%)
Which of these subtypes is a/w a more aggressive dz course
11
What are the m/c resp complications of distal RRP
Pneumatocele, abscess, and tracheal stenosis
Severe cerebellar sx with a nl MRI suggests what condition
Paraneoplastic cerebellar degeneration
What are the 2 primary subtypes of paraneoplastic cerebellar degeneration
Vestibulocerebellar syndrome and opsoclonus-myoclonus syndrome
Which malig most commonly cause paraneoplastic cerebellar degeneration
In adults, ovarian, uterine, breast, and small cell lung CA
In kids, neuroblastoma
