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42 Cards in this Set

  • Front
  • Back
what 4S NB should be treated with chemo
MYCN amplified
which renal tumors should have brain imaging
clear cell sarcoma
rhabdoid

MRI
inital eval for wilms
CT chest/ abd

CXR
work-up for clear cell sarcoma
scans
bone marrow aspirate/biopsy
skeltal survey
bone scan
MRI brain
distinctions on imaging between diffuse intrinsic pontine glioma and pilocytic astrocytoma
DIPG: enlarged pons, not enhancing, can engulf the basilar artery

PA: contrast enhancing, pushes pons out of the way, not likely to engulf the basilar artery
a diagnosis of diabetes insipitus often precedes the diagnosis of which brain tumor
germinoma
treatment for ependymoma
gross total resection
local RT
2 most common locations for germinoma
suprasellar
pineal
2 most common radiation associated brain tumors
meningiomas
GBM
what is the prognosis of a RT induced GBM compared to a de novo GBM
worse
imaging description of a GBM
intraparanchymal
necrotic center
contrast enhanciong rim
staging for HD
Ann Arbor
1 - single node region
2 - 2 or more node regions, same side diaphragm
3 - nodes both sides of the diaphragm
4 - diffuse or disseminated
If the spleen is involved in HD, what is the stage?
stage 3
what is stage 1 NHL
single nodal or extranodal tumor, EXCLUDING the mediastinum and abdomen
what is stage 2 NHL
One of the following:
- single extranodal with regional node involvement
- 2 or more nodes or extranodal tumors on the same side of the diaphragm
- ** localized (completely or grossly resected) GI disease
what is stage 3 NHL
one of the following:
- two or more extranodal tumors on oppposite sides of the diaphragm
- only intrathoracic disease (lung, pleura, mediastinum, thymic
-extensive unresectable abdominal disease
-paraspinal or epidural disease
stage 4 NHL
BM or CNS +
Characteristics that confer unfavorable prognosis in HD
- B symptoms
- stage 4
- bulky disease (> 33% chest diameter measured at diaphragm)
which type of lymphoma most commmonly involves the skin
anaplastic large cell lymphoma
diagnostic translocation for ALCL
t2;5 (npm/alk fusion)
CD markers that differentiate HD from ALCL
both are CD30 + but HD is CD 45 + and ALCL is CD 45 -
Li Fraumeni
increased risk for which CA
adenocortical CA
OS
RMS
breast
brain
leukemia
risk of CA in NF1
NF
optic glioma
pheo
AML
risk of CA in NF2
vestibular schwannoma
central NF
meningioma
risk of which CA in Gorlin-Goltz
medulloblastoma
basal cell carcinoma
genetic risk in family with POSITIVE family history of
RB: (unilateral OR bilateral disease)

- risk for parents to have another child with RB
- risk for an AFFECTED patient to have offspring with RB
- risk for a NORMAL sibling of the affected patient to have offspring with RB
- risk for parents to have another child with RB - 40%
- risk for an AFFECTED patient to have offspring with RB - 40%
- risk for a NORMAL sibling of the affected patient to have offspring with RB - 7%
genetic risk in family with negative family history of
RB and UNILATERAL disease

- risk for parents to have another child with RB
- risk for an AFFECTED patient to have offspring with RB
- risk for a NORMAL sibling of the affected patient to have offspring with RB
- risk for parents to have another child with RB - 1%
- risk for an AFFECTED patient to have offspring with RB - 8%
- risk for a NORMAL sibling of the affected patient to have offspring with RB - 1%
genetic risk in family with negative family history of
RB and BILATERAL DISEASE

- risk for parents to have another child with RB
- risk for an AFFECTED patient to have offspring with RB
- risk for a NORMAL sibling of the affected patient to have offspring with RB
- risk for parents to have another child with RB - 6%
- risk for an AFFECTED patient to have offspring with RB - 40%
- risk for a NORMAL sibling of the affected patient to have offspring with RB - 1%
where is the RB1 gene
13q14
most common second malignancies in RB
OS
soft tissue sarcomas
melanoma
where do SMNs most commonly occur in RB
60% occur in the RT field
what is trilateral RB
bilateral RB with intracranial neuroblastic tumor of the pineal gland
in what forms of RMS should a lymph node biopsy be performed
paratesticular
extremity
in what forms of RMS should CSF cytology be obtained?
parameningeal
group system for RMS

group 1:
group 2:
group 3:
group 4:
group 1: localized disease, completely resected
group 2: gross total resection with evidence of regional spread
group 3: INCOMPLETE resection with gross residual disease
group 4: distant metastatic disease
what sites are considered favorable in RMS:
orbit
non parameningeal head and neck
non bladder/prostate GU
biliary tract
Staging for RMS (1 to 4)
Stage 1: favorable site - REGARDLESS of other features
Stage 2: unfavorable site, only small tumors, negative nodes
Stage 3: unfavorable site, large tumor AND/OR postive nodes
Stage 4: metastatic
which RMS patients do not get RT
group 1 embryonal
which type of non RMS tumor has the best response to chemotherapy
synovial sarcoma
what are patients with Rothmund Thomson syndrome at risk of developing?
32% develop OS
what is Rothmund Thomson syndrome
small stature
skin rash
skeletal malformation
where is the mutation in Rothmund Thomson syndrome
mutation in RECQL4 helicase gene