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Oncology boards > Sarcoma > Flashcards

Sarcoma Flashcards

1
Q

Clinical behavior of desmoid tumors

A

Don’t metastasize but are locally aggressive

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2
Q

Precipitants of desmoid tumors

A

1) High estrogen states - during or following pregnancy (extra-abdominal or abdominal)
2) Trauma
3) FAP

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3
Q

Clinical course of desmoid tumors

A

highly variable clinical course (stable to spontaneously regressing to slow growing to rapidly progressive)

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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
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5
Q

Management of gross residual disease after resection of a desmoid tumor

A
  • Observation or RT rather than more surgery
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6
Q

Management of large intra-abdominal desmoid tumors in the setting of Gardner syndrome

A
  • Surgery still preferred
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7
Q

Klinefelter presentation

A
  • tall
  • micropenis
  • hypogonadism (small firm testes) and signs of androgen deficiency (gynecomastia, sexual dysfunction, osteoporosis)
  • learning and developmental disabilities
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8
Q

New drug approved for RET translocated sarcoma

A

selpercatinib

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9
Q

New drug approved for desmoid tumors

A

nirogacestat

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10
Q

Drug to know for giant cell tumor of bone

A

denosumab

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11
Q

Drug recently approved for alveolar soft part sarcoma

A

atezolizumab

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12
Q

Drug to know for tenosynovial giant cell tumor + major SE to be aware of

A
  • pexidartinib
  • highly hepatotoxic, requiring REMS
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13
Q

Drug approved for advanced epithelioid sarcoma

A

tazometastat

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14
Q

Osteosarcoma age distribution

A

bimodal (smaller peak in 60-80)

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15
Q

Radiographic features of osteosarcoma

A
  • sunbursting (see google image)
  • codman triangle (see google image)
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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)
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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)
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18
Q

Major prognostic indicator in osteosarcoma

A
  • response to neoadjuvant chemotherapy
    *greater than 90% osteonecrosis after 3 cycles has highest relapse free survival
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19
Q

Management of high grade localized osteosarcoma (most are high grade)

A

neoadjuvant chemo
Surgery
+/- adjuvant

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20
Q

Management of low grade localized osteosarcoma

A

surgery alone

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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

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22
Q

First line chemotherapy for metastatic osteosarcoma

A

Doxorubicin based
Cisplatin/doxorubicin without high dose methotrexate

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23
Q

Second line for metastatic osteosarcoma

A

Ifosfamide +/- etoposide

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24
Q

Oligometastatic paradigm exist in osteosarcoma

A
  • Yes, for limited number of lung mets
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25
Q

Systemic therapy for localized ewing sarcoma

A
  • cyclophosphamide + doxorubicin + vincristine alternating with ifosfamide + etoposide (VACIE)
    12-14 cycles neoadjuvant and adjuvant
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26
Q

Radiographic finding of ewing sarcoma

A
  • infiltrative pattern looking like onion skinning (see image online)
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27
Q

What to do when cumulative maximum of doxorubicin has been reached in osteosarcoma patient

A

Replace doxorubicin with actinomycin D

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28
Q

Management of localized bone sarcoma if surgery infeasible

A

Radiation

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29
Q

Management of metastatic ewing sarcoma

A

Same as osteosarcoma

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30
Q

Second line for ewing

A

topotecan combinations
temodar + irinotecan
topotecan + cytoxan

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31
Q

chondrosarcoma radiology buzzword

A
  • scalloped edging
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32
Q

localized chondrosarcoma management

A
  • surgery alone (chemo and radioresistant tumors)
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33
Q

metastatic chondrosarcoma systemic therapy options

A
  • pazopanib
  • dasatinib
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34
Q

targeted therapy options for chondrosarcoma

A
  • IDH inhibitors
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35
Q

Management of dedifferentiated chondrosarcoma

A
  • osteosarcoma chemo regimen
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36
Q

Management of mesenchymal chondrosarcoma

A
  • Ewing’s regimen
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37
Q

Origin of sarcoma

A

mesenchymal cells (as opposed to carcinoma which arise from epithelial cells)

38
Q

Sarcoma risk factors

A
  • most are sporadic
  • radiation (average 15 years)
  • vinochloride, agent orange
  • HIV
  • HHV8
  • ## chronic lymphedema (angiosarcoma)
39
Q

Genetic syndromes associated with sarcoma

A

FAP (desmoid tumors)
Li fraumeni
NF1 (peripheral nerve sheath tumors)

40
Q

Viral etiology of Kaposi sarcoma

41
Q

Mutations to know for dedifferentiated liposarcoma

A
  • MDM2
  • CDK4 amplification
42
Q

Sarcoma subtypes that metastasize through lymphatics

A

SCARE
Synovial sarcoma
Clear cell sarcoma
Angiosarcoma
Rhabdomyosarcoma
Epitheliod sarcoma

43
Q

Primary prognostic factors for sarcoma

A

Depth of disease (superficial have better prognosis)
Size
Grade

44
Q

Preferred surgery type for sarcoma

A

“limb sparing”, equivalent outcomes to amputation

45
Q

Location in body where radiation is controversial in sarcoma

A

Retroperitoneal

46
Q

Management of local recurrence in sarcoma

A

Reresection w/ XRT if feasible

47
Q

Systemic therapy regimen for sarcoma generally speaking

A

doxorubicin + ifosfamide (ifosfamide addition improves PFS but no OS benefit) + mesna (AIM regimen)

48
Q

First line for leiomyosarcoma

A

doxorubicin + dacarbazine

49
Q

second line for sarcoma

A

gemcitabine +/- docetaxel

50
Q

drug with good activity for liposarcoma (especially mixoid) and leiomyosarcoma

A

trabectedin

51
Q

Drug that has good activity for angiosarcoma and kaposi

52
Q

eribulin has activity for which sarcoma subtype?

A

liposarcoma

53
Q

pazopanib is active in all sarcoma except for what subtype?

A

liposarcoma

54
Q

What sarcoma subtype is atezolizumab approved for?

A

alveolar soft part sarcoma

55
Q

Pexidartenib 1) mechanism 2) approved sarcoma subtype 3) SE to know

A

1) CSF-1R
2) tenosynovial giant cell tumor
3) hepatotoxic

56
Q

tazometastat 1) mechanism 2) sarcoma subtype approved for

A

1) EZH2 inhibitor
2) epithelioid

57
Q

palbociclib and ribociclib indication in sarcoma

A

well and dedifferentiated liposarcoma

58
Q

nabsirolimus indication

59
Q

Imatinib indication in sarcoma

A

Dermatofibrosarcoma protuberans

60
Q

How are desmoid tumors treated?

A

Observation, assuming no causing significant local symptoms (often spontaneously regress or stable)

61
Q

Do desmoid tumors have metastatic potential?

62
Q

Systemic therapies with activity in desmoid tumors

A

sorafenib
doxil
nirogestat (only drug actually approved)

63
Q

Desmoid tumors are associated with what genetic syndrome?

A

Gardner syndrome

64
Q

Cornerstone of treatment for HIV-associated Kaposi

65
Q

Systemic therapy regimens for Kaposi

A

1) Taxol
2) liposomal doxorubicin

66
Q

Major risk factor for angiosarcoma

A
  • chronic lymphedema
67
Q

Management of oligometastatic osteosarcoma

A

metastasis directed therapy - surgery preferred if resectable, if not SBRT

68
Q

later line systemic therapy options for KS

A
  • paclitaxel
  • bleomycin, vinblastine, vincristine, etoposide
    IF no symptomatic pulmonary or visceral disease AND AIDS-related, pomalidomide (ORR 67%)
69
Q

first line systemic therapy for KS

A

liposomal doxorubicin

70
Q

Localized osteosarcoma management

A
  • neoadjuvant MAP, followed by surgery, and adjuvant MAP
71
Q

Most common site of origin of GIST

72
Q

Mutation most commonly found in gastric GIST

A

c-KIT exon 11

73
Q

Mutation most commonly found in small bowel GIST

A

c-KIT exon 9

74
Q

Mutation in succinate-dehydrogenase-deficient GIST, which typically occurs in adolescents and young adults and arises in the stomach

75
Q

First line for synovial sarcoma

A
  • doxorubicin monotherapy
    IF visceral crises, doxorubicin + ifosfamide
76
Q

Second line for KS

77
Q

Third line for KS per asco sep

A

pomalidomide

78
Q

Active drug for giant cell tumor of bone

79
Q

preferred second line for epithelioid sarcoma

A
  • tazemetostat
80
Q

What is high dose imatinib?

A

400 mg BID

81
Q

Giant cell tumor of bone house

A

Scene = Giant standing over tub/giant cell tumor. Dan kicking a soccer ball/denosumab.

82
Q

angiosarcoma room

A

Scene = Dartmouth football players tackling each other/Taxol has good activity.

83
Q

Chondrosarcoma room

A

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
Q

dermatofibroma protuberans house

A

Scene = Matt from Dartmouth sitting in hot tubs with long green tubs surround him/imatinib has activity.

85
Q

desmoid tumors house

A

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
Q

GIST room

A

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
Q

Leiomyosarcoma room

A

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
Q

KS room

A

Scene = Football players tackling each other + Adrian from AK in tub of fat/taxol + doxil.

88
Q

Liposarcoma room

A

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
Q

osteosarcoma room

A

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
Q

Tenosynovial giant cell tumor of bone room

A

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
Q

Eribulin clinical benefit compared to dacarbazine

A

Comparable PFS and OS

Oncology boards (41 decks)

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