Name the 3 translocations associated with Burkitt's leukemia/lymphoma.
What are
1) t (8;14)
2) t (2,8)
3) t (8;22)
all target c-myc
p 491 of ash-sap
100
The 2 primary sites for GIST involvement are:
What are
1) stomach
2) jejunum/ileum(SB)
100
so really, about how many subtypes of sarcomas are there really?
What is
over 50!
100
How long should patients receive maintenance chemotherapy for a diagnosis of ALL?
What is
2-3 years
100
what is the most common form of sarcoma?
What is
GIST- 5-10 cases/million persons in the US
200
Name the most common translocation seen in adult ALL.
What is
t(9;22)- seen in 20-30% of adult cases
200
Name the specific genetic mutation most commonly seen in GIST tumors (gene and exon).
What is
CKIT- exon 11
others would include exon 9 and PDGFRA
200
Name the 4 components for staging of sarcomas.
What are
1) size
2)grade
3)depth/location
4)metastasis
200
What age group has the best prognosis when diagnosed with ALL?
What is
1-9
200
Name the virus associated with the development of Kaposi's sarcoma.
What is
HHV-8
300
Name the 2 chromosomal mutations commonly associated with Ewings sarcoma.
What are
t(11;22) (85%)
t(21;22) (5-10%)
300
Name at least 2 factors that make a GIST high risk.
What is
1) size (>10cm)
2) number of mitosis (>5 per 50 HPF)
3) site of origin
4) rupture
5)metastatic disease
300
Describe the typical xray finding for osseous ewings sarcoma.
What is
"onion skinning" affecting the shaft of the bone
300
For philadelphia positive ALL, which breakpoint on chromosome 22 is most common?
What is
the 2 breakpoints (Mbcr-p210 and the mbcr-p190) are about equal in frequency
300
In bony sarcomas lymph node involvement is stage ____?
What is
IVB
400
Name at least 2 genetic syndromes and their mutation that are associated with the development of sarcomas.
What is
1) Li- Fraumeni (p53)
2)neurofibromatosis (NF1)
3) retinoblastoma syndrome (RB)
4) gardners syndrome (APC)
5)werners syndrome (helicase)
asco sep P 327/flash review 269
400
Treatment options for GIST tumors progressive on Imatinib would include: name at least 2
What is
1) increased dose of Imatinib (600 or 800)
2) sunitinib
3) surgical resection
4) local radiation therapy
asco-sep p334/
400
In soft tissue sarcomas, when should you consider adjuvant chemotherapy and with what agent(s)?
What is
larger, high grade STS of the extremity- adria based chemotherapy
flash review-280/asco sep p.331
study from 1997
400
Which genetic abnormality is associated with a better prognosis in ALL?
What is
hyperdiploidy (>50)
although may not be as good in adults as it is in children. pediatrics also uses triple trisomy (4,10,17)
400
Standard of care treatment for Ph1+ ALL in adults should include:
What is
induction therapy +TKI followed by allo BMT
500
Name the chromosomal translocation associated with desmoplastic small round cell tumors.
What is
t(11;22) (p13;q12)
the ewings translocation is (p24;q12)
500
Name the cell of origin for GIST tumors.
What is
the cells of Cajal (pacemaker cells of the GIT)
500
In patients with osteosarcomas who achieve a poor response to neoadjuvant chemotherapy as assessed by pathologic review, further treatment should include:
What is
a full course of chemotherapy despite the failure to show histologic response.
500
Assessment of MRD in ALL using PCR is very sensitive. What level of involvement constitutes molecular remission?
<5%
<1%
<.1%
<.01%
What is
<.01%
explanation p 493 in ash-sap. >1% as bad a >5% by morphology (persistent disease). <.01 translates to excellent long term prognosis and a way to monitor for early relapse.
500
Name the most common AIDs associated cancer in the US and its front line therapy