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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
Preop CTV margins (large)
If high grade, >8 cm, 3 cm sup/inf, 1.5 cm radially (+T2 edema)
26
Preop CTV margins (small)
If low grade or <8 cm, 2 cm sup/inf, 1 cm radially (+T2 edema)
27
Postop CTV margins
CTV1 (50 Gy): 4 cm sup/inf, 1.5 cm radially | CTV2 (10-16 Gy): 2 cm sup/inf, 1.5 cm radially
28
Retroperitoneal sarcoma margins
GTV --> ITV --> 1.5 cm circumferentially (CTV)
29
Bowel contour approach
contour as bowel bag
30
Stomach constraint for RP sarcoma
V45 <100%, V50 <50%, Dmax <56 Gy
31
Kidney constraint for RP sarcoma
If 1 kidney: V18 < 15% | If 2 kidneys: Mean < 15 Gy, V18 < 50%
32
Dose for Dupuytren's Disease
3 Gy x 5 --> 6-8 weeks --> 3 Gy x 5
33
Association with desmoid tumor
FAP (get c-scope!)
34
Stain for desmoid tumors
beta catenin
35
Treatment for desmoids
1. consider obs 2. NSAIDs 3. Gleevec or other TKI, tamoxifen 4. Surgery (25-40% recurrence rate) 5. Radiation
36
RT dose for desmoid
56-58 Gy, 50 Gy if R1 resection
37
RT control rate for desmoid
75-80%
38
Virus associated with Kaposi sarcoma
HHV-8
39
Radiology pattern for osteosarcoma
"sunburst" or "Codman's triangle"
40
Typical dose for osteosarcoma
70 Gy
41
Radiology pattern for Ewing sarcoma
onion peel
42
Treatment strategy for Ewing
VAC/IE --> surgery --> VAC/IE
43
RT dose for Ewing
Gross disease 55.8 Gy | Microscopic disease 50.4
44
RT dose for chondrosarcoma
68-70 Gy
45
Path finding for chordoma
chromosome 6 is duplicated
46
Dose for chordoma
74 Gy RBE
47
RP Sarcoma - greater risk of local failure liposarcoma vs. leiomyosarcoma
liposarcoma is higher (dediff > well diff) | leiomyosarcoma is low (10%
48
Dose for RP sarcomas
Typically preop 50 Gy
49
STRASS study design
Abdominal recurrence free survival - preop RT --> surgery vs. surgery alone
50
Findings of STRASS study
No difference in abdominal failure for preop RT vs. surgery alone. Higher rates of tox for neoadjuvant RT (lymphopenia)
51
Rate of severe GI tox from preop RT for RP sarcoma
5-12% grade 3+ | 60-70% grade 2
52
Rough local recurrence rates for RP sarcoma after preopRT
~40-50%
53
Stewart Treves syndrome
Higher risk of angiosarcoma in women with chronic lymphedema after breast cancer
54
Factors associated with distant recurrence
1. Large tumor size 2. high grade 3. deep location 4. recurrent disease at presentation 5. histologic subtype (leiomyosarcoma)
55
Factors associated with local recurrence
1. Age >50 2. Positive margins 3. Previous recurrence 4. histologic subtype (fibrosarcoma and MPNST)
56
Path hallmark of well diff and dediff liposarcoma
12q amplification
57
Path hallmark of Ewing sarcoma
t(11:22)
58
NCIC study: requirement of boost for + margins
16-20 Gy | Required in 11%
59
MSK BT trial
Increased local control for high grade tumors but not lower grade tumors and did not affect DSS or DMFS
60
Subgroups that did not benefit from BT
1. Positive margins | 2. Low grade
61
Chemotherapy
Shown in meta analysis to have OS benefit for doxorubicin plus ifosfamide
62
RP sarcoma greater risk of distant failure
Leiomyosarcoma (50%) > liposarcoma
63
ncic margin
5 cm sup/inf (phase I) | 2 cm sup/inf (phase II, 16-20 Gy)
64
IMRT vs 3DCRT
Improved local control (~50%) with IMRT, also better tox
65
When to obtain biopsy of RP sarcoma
if preop chemo or RT planned, or if evidence of dedifferentiation
66
Cowden syndrome
multiple harmatoma syndrome, PTEN mutation
67
FAP
Gardner syndrome, APC mutation, desmoids
68
Li-Fraumeni
p53, bone and soft tissue sarcomas, breast, leukema, adrenal tumors
69
Factors associated with late local recurrence
1. Location (internal trunk)/RP | 2. Tumor size >10
70
Factors associated with late distant recurrence
Grade >1
71
Margin status in early postop trials
``` NCI = positive margins excluded MSK = Positive and negative margins included ```
72
Differences in RT benefits by margin status
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
Differences in RT doses (MSK/NCI)
MSK: 45 Gy over 4-6 d NCI: 45 Gy wide and then 18 Gy boost to tumor bed
74
Dermatofibrosarcoma protuberans role of RT
Only for positive margins - locally aggressive tumor
75
Stage of N1 disease
IIIB for RP sarcoma, IV for extremity
76
Ewing translocation
t(11;22)
77
Rhabdo translocation
t(2;13) or t(1;13) resulting in PAX-FOXO translocation

RO Clinical Boards (13 decks)

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