Sarcoma Flashcards
(92 cards)
1
Q
Clinical behavior of desmoid tumors
A
Don’t metastasize but are locally aggressive
2
Q
Precipitants of desmoid tumors
A
1) High estrogen states - during or following pregnancy (extra-abdominal or abdominal)
2) Trauma
3) FAP
3
Q
Clinical course of desmoid tumors
A
highly variable clinical course (stable to spontaneously regressing to slow growing to rapidly progressive)
4
Q
Management of desmoid tumor
A
- IF asymptomatic or minimally symptomatic, initial period of observation (Let disease biology declare itself. Significant rate of spontaneous regression and periods of growth arrest. Placebo arm had a 20% response rate in sorafenib trial).
- IF causing significant symptoms, multimodality evaluation
5
Q
Management of gross residual disease after resection of a desmoid tumor
A
- Observation or RT rather than more surgery
6
Q
Management of large intra-abdominal desmoid tumors in the setting of Gardner syndrome
A
- Surgery still preferred
7
Q
Klinefelter presentation
A
- tall
- micropenis
- hypogonadism (small firm testes) and signs of androgen deficiency (gynecomastia, sexual dysfunction, osteoporosis)
- learning and developmental disabilities
8
Q
New drug approved for RET translocated sarcoma
A
selpercatinib
9
Q
New drug approved for desmoid tumors
A
nirogacestat
10
Q
Drug to know for giant cell tumor of bone
A
denosumab
11
Q
Drug recently approved for alveolar soft part sarcoma
A
atezolizumab
12
Q
Drug to know for tenosynovial giant cell tumor + major SE to be aware of
A
- pexidartinib
- highly hepatotoxic, requiring REMS
13
Q
Drug approved for advanced epithelioid sarcoma
A
tazometastat
14
Q
Osteosarcoma age distribution
A
bimodal (smaller peak in 60-80)
15
Q
Radiographic features of osteosarcoma
A
- sunbursting (see google image)
- codman triangle (see google image)
16
Q
Biopsy required for osteosarcoma
A
- incisional or core always required (never FNA, need architecture in sarcoma, similar to lymphoma)
- always done by sarcoma expert (biopsy track has to be removed surgically)
17
Q
Osteosarcoma preferred staging modalities
A
- MRI of primary location
- CT chest (sarcoma can have hematogenous seeding to lung)
- bone scan (can have skip mets along bones)
18
Q
Major prognostic indicator in osteosarcoma
A
- response to neoadjuvant chemotherapy
*greater than 90% osteonecrosis after 3 cycles has highest relapse free survival
19
Q
Management of high grade localized osteosarcoma (most are high grade)
A
neoadjuvant chemo
Surgery
+/- adjuvant
20
Q
Management of low grade localized osteosarcoma
A
surgery alone
21
Q
Neoadjuvant systemic therapy for osteosarcoma
A
3 cycles of doxorubicin + cisplatin
*high dose methotrexate can be added if young with good renal function
22
Q
First line chemotherapy for metastatic osteosarcoma
A
Doxorubicin based
Cisplatin/doxorubicin without high dose methotrexate
23
Q
Second line for metastatic osteosarcoma
A
Ifosfamide +/- etoposide
24
Q
Oligometastatic paradigm exist in osteosarcoma
A
- Yes, for limited number of lung mets
25
Systemic therapy for localized ewing sarcoma
- cyclophosphamide + doxorubicin + vincristine alternating with ifosfamide + etoposide (VACIE)
12-14 cycles neoadjuvant and adjuvant
26
Radiographic finding of ewing sarcoma
- infiltrative pattern looking like onion skinning (see image online)
27
What to do when cumulative maximum of doxorubicin has been reached in osteosarcoma patient
Replace doxorubicin with actinomycin D
28
Management of localized bone sarcoma if surgery infeasible
Radiation
29
Management of metastatic ewing sarcoma
Same as osteosarcoma
30
Second line for ewing
topotecan combinations
temodar + irinotecan
topotecan + cytoxan
31
chondrosarcoma radiology buzzword
- scalloped edging
32
localized chondrosarcoma management
- surgery alone (chemo and radioresistant tumors)
33
metastatic chondrosarcoma systemic therapy options
- pazopanib
- dasatinib
34
targeted therapy options for chondrosarcoma
- IDH inhibitors
35
Management of dedifferentiated chondrosarcoma
- osteosarcoma chemo regimen
36
Management of mesenchymal chondrosarcoma
- Ewing's regimen
37
Origin of sarcoma
mesenchymal cells (as opposed to carcinoma which arise from epithelial cells)
38
Sarcoma risk factors
- most are sporadic
- radiation (average 15 years)
- vinochloride, agent orange
- HIV
- HHV8
- chronic lymphedema (angiosarcoma)
-
39
Genetic syndromes associated with sarcoma
FAP (desmoid tumors)
Li fraumeni
NF1 (peripheral nerve sheath tumors)
40
Viral etiology of Kaposi sarcoma
HHV-8
41
Mutations to know for dedifferentiated liposarcoma
- MDM2
- CDK4 amplification
42
Sarcoma subtypes that metastasize through lymphatics
SCARE
Synovial sarcoma
Clear cell sarcoma
Angiosarcoma
Rhabdomyosarcoma
Epitheliod sarcoma
43
Primary prognostic factors for sarcoma
Depth of disease (superficial have better prognosis)
Size
Grade
44
Preferred surgery type for sarcoma
"limb sparing", equivalent outcomes to amputation
45
Location in body where radiation is controversial in sarcoma
Retroperitoneal
46
Management of local recurrence in sarcoma
Reresection w/ XRT if feasible
47
Systemic therapy regimen for sarcoma generally speaking
doxorubicin + ifosfamide (ifosfamide addition improves PFS but no OS benefit) + mesna (AIM regimen)
48
First line for leiomyosarcoma
doxorubicin + dacarbazine
49
second line for sarcoma
gemcitabine +/- docetaxel
50
drug with good activity for liposarcoma (especially mixoid) and leiomyosarcoma
trabectedin
51
Drug that has good activity for angiosarcoma and kaposi
taxol
52
eribulin has activity for which sarcoma subtype?
liposarcoma
53
pazopanib is active in all sarcoma except for what subtype?
liposarcoma
54
What sarcoma subtype is atezolizumab approved for?
alveolar soft part sarcoma
55
Pexidartenib 1) mechanism 2) approved sarcoma subtype 3) SE to know
1) CSF-1R
2) tenosynovial giant cell tumor
3) hepatotoxic
56
tazometastat 1) mechanism 2) sarcoma subtype approved for
1) EZH2 inhibitor
2) epithelioid
57
palbociclib and ribociclib indication in sarcoma
well and dedifferentiated liposarcoma
58
nabsirolimus indication
PEComa
59
Imatinib indication in sarcoma
Dermatofibrosarcoma protuberans
60
How are desmoid tumors treated?
Observation, assuming no causing significant local symptoms (often spontaneously regress or stable)
61
Do desmoid tumors have metastatic potential?
No
62
Systemic therapies with activity in desmoid tumors
sorafenib
doxil
nirogestat (only drug actually approved)
63
Desmoid tumors are associated with what genetic syndrome?
Gardner syndrome
64
Cornerstone of treatment for HIV-associated Kaposi
HAART
65
Systemic therapy regimens for Kaposi
1) Taxol
2) liposomal doxorubicin
66
Major risk factor for angiosarcoma
- chronic lymphedema
67
Management of oligometastatic osteosarcoma
metastasis directed therapy - surgery preferred if resectable, if not SBRT
68
later line systemic therapy options for KS
- paclitaxel
- bleomycin, vinblastine, vincristine, etoposide
IF no symptomatic pulmonary or visceral disease AND AIDS-related, pomalidomide (ORR 67%)
69
first line systemic therapy for KS
liposomal doxorubicin
70
Localized osteosarcoma management
- neoadjuvant MAP, followed by surgery, and adjuvant MAP
71
Most common site of origin of GIST
stomach
72
Mutation most commonly found in gastric GIST
c-KIT exon 11
73
Mutation most commonly found in small bowel GIST
c-KIT exon 9
74
Mutation in succinate-dehydrogenase-deficient GIST, which typically occurs in adolescents and young adults and arises in the stomach
- SDHB
75
First line for synovial sarcoma
- doxorubicin monotherapy
IF visceral crises, doxorubicin + ifosfamide
76
Second line for KS
taxol
77
Third line for KS per asco sep
pomalidomide
78
Active drug for giant cell tumor of bone
denosumab
79
preferred second line for epithelioid sarcoma
- tazemetostat
80
What is high dose imatinib?
400 mg BID
81
Giant cell tumor of bone house
Scene = Giant standing over tub/giant cell tumor. Dan kicking a soccer ball/denosumab.
82
angiosarcoma room
Scene = Dartmouth football players tackling each other/Taxol has good activity.
83
Chondrosarcoma room
Scene = Floor covered in scallops/radiology buzzword = scalloped edging. Dasani water bottle with frying pan on top of it/First line for metastatic disease = dasatinib + pazopanib. Ivan sitting on far end + Ina Garten/IDH inhibitors.
84
dermatofibroma protuberans house
Scene = Matt from Dartmouth sitting in hot tubs with long green tubs surround him/imatinib has activity.
85
desmoid tumors house
Scene = Dezzi standing in middle and niagara falls washing over him/nirogacestat. Evil wizzard from lord of the rings standing in closet + Adrian from AK sitting in tub of fat/sorafenib has activity + doxil.
86
GIST room
Scene: Huge dog sitting in middle with a top hat on + tacks covering ceiling + /CD34 + CD117 positive + DOG1 positive. futurist bald robot girl on kitchen counter getting eaten out by Tove/Exon 18 is imatinib resistant and requires avapritinib
87
Leiomyosarcoma room
Scene = Adrian from AK sitting in Liam’s crib + big car on top of magazines/first line = doxorubicin + dacarbazine. Alex Trabek sitting by window/trabectedin has good activity.
88
KS room
Scene = Football players tackling each other + Adrian from AK in tub of fat/taxol + doxil.
88
Liposarcoma room
Scene = Huge pile of CDs + taking MDMA/MDM2 + CDK4. Erdem sitting on table getting BJ from Holly/eribulin has good activity. Stake on left side full of pans/pazopanib does not have good activity for liposarcoma. Canoe hanging from ceiling on left/palbociclib + ribociclib approved.
89
osteosarcoma room
Scene = Intense sun coming through window + big golden triangle/Radiographic features = sunbursting + codman triangle. Walls are titanium metal + Adrian from Alaska standing by window + Jessie from breaking bad smoking a cigarette/neoadjuvant systemic = doxorubicin + cisplatin +/- methotrexate depending on renal function. Second story w/ Ethan playing guitar + phosphate sparkles going off next to him/second line = ifosfamide + etoposide.
90
Tenosynovial giant cell tumor of bone room
Scene = Ridding hippo/Highly hepatotoxic, requiring REMS. Harvey is playing darts/pexidartinib is new drug to know. Arrow directly into Harvey’s spine/pexidartinib mechanism = CSF-1R. /denosumab very active.
91
Eribulin clinical benefit compared to dacarbazine
Comparable PFS and OS
