Sarcoma Flashcards

1
Q

T1

A

<5 cm

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2
Q

T2

A

5-10 cm

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3
Q

Group IA

A

grade 1, T1

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4
Q

Group 2

A

T1, grade 2-3

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5
Q

Group 3A

A

T2, grade 2-3

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6
Q

Group 3B

A

T3-4, grade 2-3

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7
Q

Group 4

A

N1 or M1

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8
Q

Mutation with RT-associated sarcoma

A

3p rearrangement

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9
Q

Common RT-associated histologies

A

undifferentiated (pleomorphic) sarcoma
angiosarcoma
malignant fibrous histiocytoma, fibrosarcoma, MPNST

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10
Q

Histologies with nodes

A

CARE: clear cell, angiosarcoma, rhabdo, epithelioid

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11
Q

Histology with spine mets

A

Myxoid liposarcoma

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12
Q

Hallmark of NF1

A

benign neurofibromas, MPNST

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13
Q

Hallmark of familial polyposis

A

desmoid tumors

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14
Q

Hallmark of retinoblastoma and Li-Fraumeni syndrome

A

Bone and STS

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15
Q

Type of biopsy required

A

core

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16
Q

Imaging required

A

MRI, CT chest (PET only if LN risk)

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17
Q

Typical pre-op dose

A

50 Gy

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18
Q

Typical post-op dose

A

60-66 Gy

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19
Q

Original NCI study

A

43 patients, amputation vs. limb sparing surgery + RT (45-50 Gy plus boost to 60-70 Gy); RT improved LC but no effect on OS

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20
Q

2nd NCI study

A

141 patients with extremity sarcoma, limb sparing surgery + RT (with concurrent chemo) vs. surgery with chemo - improved LC with RT, no difference in OS

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21
Q

MSKCC study

A

164 patients with limb sparing surgery with adjuvant BT (Ir-192) vs. observation - LC advantage for BT ONLY in high grade, 50% wound complication rates

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22
Q

O’Sullivan study

A

190 patients RCT - preop (50 Gy) vs. postop (66 Gy) showing greater rates of wound complications within 4 months in PREOP group (35% vs. 17%) no differences in outcomes at tradeoff of greater risk of fibrosis/edema in POSTOP group

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23
Q

Site of worst wound complications for preop RT

A

Lower extremity/thigh

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24
Q

RTOG 0630

A

Volume reduction study showed improvement in 2 year toxicity no diff in LC

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25
Q

Preop CTV margins (large)

A

If high grade, >8 cm, 3 cm sup/inf, 1.5 cm radially (+T2 edema)

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26
Q

Preop CTV margins (small)

A

If low grade or <8 cm, 2 cm sup/inf, 1 cm radially (+T2 edema)

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27
Q

Postop CTV margins

A

CTV1 (50 Gy): 4 cm sup/inf, 1.5 cm radially

CTV2 (10-16 Gy): 2 cm sup/inf, 1.5 cm radially

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28
Q

Retroperitoneal sarcoma margins

A

GTV –> ITV –> 1.5 cm circumferentially (CTV)

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29
Q

Bowel contour approach

A

contour as bowel bag

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30
Q

Stomach constraint for RP sarcoma

A

V45 <100%, V50 <50%, Dmax <56 Gy

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31
Q

Kidney constraint for RP sarcoma

A

If 1 kidney: V18 < 15%

If 2 kidneys: Mean < 15 Gy, V18 < 50%

32
Q

Dose for Dupuytren’s Disease

A

3 Gy x 5 –> 6-8 weeks –> 3 Gy x 5

33
Q

Association with desmoid tumor

A

FAP (get c-scope!)

34
Q

Stain for desmoid tumors

A

beta catenin

35
Q

Treatment for desmoids

A
  1. consider obs
  2. NSAIDs
  3. Gleevec or other TKI, tamoxifen
  4. Surgery (25-40% recurrence rate)
  5. Radiation
36
Q

RT dose for desmoid

A

56-58 Gy, 50 Gy if R1 resection

37
Q

RT control rate for desmoid

A

75-80%

38
Q

Virus associated with Kaposi sarcoma

A

HHV-8

39
Q

Radiology pattern for osteosarcoma

A

“sunburst” or “Codman’s triangle”

40
Q

Typical dose for osteosarcoma

A

70 Gy

41
Q

Radiology pattern for Ewing sarcoma

A

onion peel

42
Q

Treatment strategy for Ewing

A

VAC/IE –> surgery –> VAC/IE

43
Q

RT dose for Ewing

A

Gross disease 55.8 Gy

Microscopic disease 50.4

44
Q

RT dose for chondrosarcoma

A

68-70 Gy

45
Q

Path finding for chordoma

A

chromosome 6 is duplicated

46
Q

Dose for chordoma

A

74 Gy RBE

47
Q

RP Sarcoma - greater risk of local failure liposarcoma vs. leiomyosarcoma

A

liposarcoma is higher (dediff > well diff)

leiomyosarcoma is low (10%

48
Q

Dose for RP sarcomas

A

Typically preop 50 Gy

49
Q

STRASS study design

A

Abdominal recurrence free survival - preop RT –> surgery vs. surgery alone

50
Q

Findings of STRASS study

A

No difference in abdominal failure for preop RT vs. surgery alone. Higher rates of tox for neoadjuvant RT (lymphopenia)

51
Q

Rate of severe GI tox from preop RT for RP sarcoma

A

5-12% grade 3+

60-70% grade 2

52
Q

Rough local recurrence rates for RP sarcoma after preopRT

A

~40-50%

53
Q

Stewart Treves syndrome

A

Higher risk of angiosarcoma in women with chronic lymphedema after breast cancer

54
Q

Factors associated with distant recurrence

A
  1. Large tumor size
  2. high grade
  3. deep location
  4. recurrent disease at presentation
  5. histologic subtype (leiomyosarcoma)
55
Q

Factors associated with local recurrence

A
  1. Age >50
  2. Positive margins
  3. Previous recurrence
  4. histologic subtype (fibrosarcoma and MPNST)
56
Q

Path hallmark of well diff and dediff liposarcoma

A

12q amplification

57
Q

Path hallmark of Ewing sarcoma

A

t(11:22)

58
Q

NCIC study: requirement of boost for + margins

A

16-20 Gy

Required in 11%

59
Q

MSK BT trial

A

Increased local control for high grade tumors but not lower grade tumors and did not affect DSS or DMFS

60
Q

Subgroups that did not benefit from BT

A
  1. Positive margins

2. Low grade

61
Q

Chemotherapy

A

Shown in meta analysis to have OS benefit for doxorubicin plus ifosfamide

62
Q

RP sarcoma greater risk of distant failure

A

Leiomyosarcoma (50%) > liposarcoma

63
Q

ncic margin

A

5 cm sup/inf (phase I)

2 cm sup/inf (phase II, 16-20 Gy)

64
Q

IMRT vs 3DCRT

A

Improved local control (~50%) with IMRT, also better tox

65
Q

When to obtain biopsy of RP sarcoma

A

if preop chemo or RT planned, or if evidence of dedifferentiation

66
Q

Cowden syndrome

A

multiple harmatoma syndrome, PTEN mutation

67
Q

FAP

A

Gardner syndrome, APC mutation, desmoids

68
Q

Li-Fraumeni

A

p53, bone and soft tissue sarcomas, breast, leukema, adrenal tumors

69
Q

Factors associated with late local recurrence

A
  1. Location (internal trunk)/RP

2. Tumor size >10

70
Q

Factors associated with late distant recurrence

A

Grade >1

71
Q

Margin status in early postop trials

A
NCI = positive margins excluded
MSK = Positive and negative margins included
72
Q

Differences in RT benefits by margin status

A

NCI: only negative margins, adjuvant RT improved local control for low grade and high grade
MSK: neg/pos margins, using BT (Ir-192) benefit on for high grade tumors

73
Q

Differences in RT doses (MSK/NCI)

A

MSK: 45 Gy over 4-6 d
NCI: 45 Gy wide and then 18 Gy boost to tumor bed

74
Q

Dermatofibrosarcoma protuberans role of RT

A

Only for positive margins - locally aggressive tumor

75
Q

Stage of N1 disease

A

IIIB for RP sarcoma, IV for extremity

76
Q

Ewing translocation

A

t(11;22)

77
Q

Rhabdo translocation

A

t(2;13) or t(1;13) resulting in PAX-FOXO translocation