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Ph RadOnc Residency PBRROE2-3 Review 2021 > Soft Tissue Sarcoma (Excluding Retroperitoneum) > Flashcards

Soft Tissue Sarcoma (Excluding Retroperitoneum) Flashcards

1
Q

STS

What are the “environmental” factors associated with the development of sarcoma?

A 
radiation exposure
and
chemical exposure
(vinyl chloride
dioxin
arsenical pesticides
phenoxy herbicides)
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2
Q

STS

What are the “viruses” factors associated with the development of sarcoma?

A

HIV

HHV-8

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3
Q

STS

What lymphedama is associated with the development of sarcoma?

A

Stewart-Treves Syndrome

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4
Q

STS

What “genetic predispositions” factors associated with the development of sarcoma?

A

Li Fraumeni syndrome
Werner syndrome
NF1 (MPNST)
FAP/Gardner syndrome (abdominal desmoid)

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5
Q

STS

What are the four categories of STS accdording to the WHO?

A
  • benign
  • intermediate, locally agressive
  • intermediate, rarely metastasizing
  • malignant
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6
Q

STS

Identify the chromosomal translocation associated with this STS.

synovial sarcoma

A

t(X,18)

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7
Q

STS

Identify the chromosomal translocation associated with this STS.

Ewing sarcoma

A

t(11,22)

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8
Q

STS

Identify the chromosomal translocation associated with this STS.

myxoid liposarcoma

A

t(12,16)

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9
Q

STS

Identify the chromosomal translocation associated with this STS.

clear cell sarcoma

A

t(12,22)

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10
Q

STS

What are the basis of grade classification of the FNCLCC into low, intermediate, and high?

A

mitotic activity
differentiation
necrosis

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11
Q

STS

What is the most common and other common sites of presentation of STS?

A

thigh.

extremity (lower 45%, upper 15%)
trunk 15 to 20%
retroperitoneum 10 to 15%
H&N 9%

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12
Q

STS

Lymph node involvement in sarcomas is rare.
However, there are certain histologic types with predilections to involve them.
What are these?

A

Epithelioid sarcoma (20 to 35%)
Rhabdomyosarcoma (20 to 25%)
Clear cell sarcoma (10 to 18%)
cutaneous Angiosarcoma of the scalp (10 to 15%)

(ERCA)

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13
Q

STS

What is the single most
frequent site of distant metastasis?

A

Lung (34%)

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14
Q

STS

What histologic subtype has a predilection to spread more to the retroperitoneum?

A

Myxoid liposarcoma (50% of recurrurences)

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15
Q

STS

What histologic subtype has a predilection for skip metastases?

A

Epithelioid sarcoma

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16
Q

STS

What histologic subtype has a higher rate of positive resection margins and LR compared to other STS, conversely lower rate of lung metastases?

A

Myxofibrosarcoma

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17
Q

STS

What is the preferred initial imaging modality for the evaluation of patients suspected to have STS?

A

MRI

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18
Q

STS

What is the preferred biopsy approach for the evaluation of patients suspected to have STS?

A

CT-guided core biopsy

Incisional biopsy is accurate and acceptable, but it is
more invasive than core biopsy. If utilized, incisional biopsies should be
performed carefully with the subsequent definitive resection in mind.Tumor
cells can potentially seed an incision, thereby necessitating removal of skin
incisions at the time of surgical resection. It is important that the biopsy
approach does not transgress an uninvolved compartment or joint as this would
create a situation where a much more radical resection would need to be
performed. Consequences of inappropriately placed incisional biopsies can be
significant and include the need to perform more complex operations, with the
potential for subsequent loss of function, LR, and death.
Fine needle aspirate
(FNA) can confirm malignancy for recurrent disease, but typically does not yield
enough tissue to establish an initial diagnosis.

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19
Q

STS

AJCC staging
Identify the stage

M1

A

Stage IV

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20
Q

STS

AJCC staging
Identify the stage

N1

A

Stage IV

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21
Q

STS

AJCC staging
Identify the stage

T2
G1
N0
M0

A

Stage IB

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22
Q

STS

AJCC staging
Identify the stage

T3
G1
N0
M0

A

Stage IB

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23
Q

STS

AJCC staging
Identify the stage

T4
G1
N0
M0

A

Stage IB

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24
Q

STS

AJCC staging
Identify the stage

T1
G1
N0
M0

A

Stage IA

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25
Q

STS

AJCC staging
Identify the stage

T2
G2
N0
M0

A

Stage IIIA

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26
Q

STS

AJCC staging
Identify the stage

T2
G3
N0
M0

A

Stage IIIA

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27
Q

STS

AJCC staging
Identify the stage

T1
G3
N0
M0

A

Stage II

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28
Q

STS

AJCC staging
Identify the stage

T1
G2
N0
M0

A

Stage II

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29
Q

STS

AJCC staging
Identify the stage

T3
G2
N0
M0

A

Stage IIIB

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30
Q

STS

AJCC staging
Identify the stage

T4
G2
N0
M0

A

Stage IIIB

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31
Q

STS

AJCC staging
Identify the stage

T4
G3
N0
M0

A

Stage IIIB

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32
Q

STS

AJCC staging
Identify the stage

T3
G3
N0
M0

A

Stage IIIB

33
Q

STS

AJCC
TNMG

Define T1

A

0 to 5 cm

34
Q

STS

AJCC
TNMG

Define T2

A

≥5 to 10 cm

35
Q

STS

AJCC
TNMG

Define T3

A

≥10 to 15 cm

36
Q

STS

AJCC
TNMG

Define T4

A

≥15 cm

37
Q

STS

What is the most powerful predictor for DFS and OS?

A

AJCC TNMG stage of the tumor

38
Q

STS

What is the single most important individual prognostic factor for lower survival rates?

A

high grade

39
Q

STS

What are the predictors of higher LR?

A

resection margins,
tumor location (RP and H&N),
presentation with locally recurrent disease,
advancing age

40
Q

STS

In general, what is the “standard of care” for high-grade STS?

A

wide resection(conservative surgery) + pre or postoperative radiation therapy (RT)

41
Q

STS

RCTs

This trial randomized high-grade extremity STS to amputation vs CS + EBRT (60-70 Gy)

Both arms received chemo with doxorubicin, cyclophosphamide, methotrexate.

LR were similar, 0% (0 of 16) and 15% (4 of 27) P=.06

No OS difference.

A

Rosenberg
NCI
(1981)

42
Q

STS

RCTs

This trial randomized high-grade and low grade extremity STS to CS alone vs. CS + EBRT (63Gy)

High grade arms received chemo with doxorubicin, cyclophosphamide

LR were lower for high-grade and low-grade tumors treated with EBRT.

No OS difference.

A

Yang
NCI
(1998)

43
Q

STS

RCTs

This trial randomized high-grade and low grade extremity and trunk STS to CS alone vs. CS + BRT (42 to 45 Gy)

LR were lower for high-grade (with negative margins only) (+BRT) but not for low-grade tumors even with BRT.

No survival difference.

A

Pisters
MSKCC
(1996)

44
Q

STS

What subsets of patients will benefit from post-CS brachytherapy in general (based on MSKCC 1996 study)?

A

High-grade extremity/trunk STS with negative margins.

45
Q

STS

Low-grade STSs are usually treated with surgery alone.
What are the indications for RT in the setting of low-grade disease?

A

positive resection margins,

locally recurrent disease following initial wide excision,

tumor location that would not be amenable to subsequent salvage surgery

46
Q

STS

RT volumes
Pre-operative RT GTV

A

enhancing mass on T1 postgadolinum MRI

47
Q

STS

RT volumes
Pre-operative RT CTV

A

GTV + 4-cm in the longitudinal directions + 1.5-cm radially

48
Q

STS

RT volumes
Pre-operative RT PTV

A

CTV + 0.5 to 1 cm

49
Q

STS

RT volumes
Pre-operative CTV

Upon consulting with the surgeon for planned skin resection and you decide to spare the skin for superficial dose avoidance, how much of the volume will you edit out beneath the skin?

A

5 mm

50
Q

STS

RT volumes
Pre-operative CTV

Where do you take into consideration the peritumoral edema on T2 MRI? CTV or GTV?

A

CTV.

Most likely, the peritumoral edema will be included in the 4 cm margin.

Extending beyond 4cm is upon the discretion of the radiation oncologist.

51
Q

STS

RT volumes
Post-operative CTV

A

operative bed (postoperative changes on MRI including all tissues handled during surgery, incision and drainage sites) + 4 cm longitudinally and 1.5 cm radially.

52
Q

STS

RT volumes
Post-operative PTV

A

0.5 to 1 cm

53
Q

STS

RT volumes
Post-operative cone-down CTV

A

preoperative tumor location + 2 cm margins

54
Q

STS

This study investigated to decrease longitudinal CTV margins to 3 cm for high-grade and 2 cm for low-grade preoperative extremity STS, which showed recurrences within fields rather than marginal.

A

RTOG 0630

55
Q

STS

This study investigated to decrease CTV margins in all directions to 2 cm for postoperative extremity STS, however, has been only presented in abstract form and needs longer follow-up.

A

VORTEX trial

56
Q

STS

RT volumes
Post-operative brachytherapy margins

A

2 cm longitudinal
1-2 cm lateral
(American Brachytherapy Society)

57
Q

STS

RT dose
Standard preoperative RT dose (negative margins)

A

50 Gy/2/25

58
Q

STS

RT dose
Standard preoperative RT dose (positive margins)

A

50 Gy/2/25

EBRT boost 16 to 20 Gy (1.8-2)

BRT boost:
LDR 15-25 Gy
HDR 12-20 Gy (2-4 bid)

total of approximately 65 Gy

59
Q

STS

RT dose
Standard postoperative RT dose (negative margins)

A

60-66 Gy (1.8-2)

60
Q

STS

RT dose
Standard postoperative RT dose (positive margins)

A

66-68 Gy (1.8-2)

45-50 standard field
balance given in reduced field

61
Q

STS

RT dose
Standard postoperative BRT dose (LDR monotherapy)

A

45 Gy

62
Q

STS

RT dose
Standard postoperative BRT dose (HDR monotherapy)

A

30-50 Gy (2-4 bid)

63
Q

STS

Why do you have to “spare a strip” of limb circumference in the RT of extremity STS?

A

avoid subsequent lymphedema and pain

64
Q

STS

How much strip of limb circumference and to receive what dose should you spare the RT of extremity STS?

A

minimum of 1-cm to receive 20 Gy.

65
Q

STS

Dosimetric parameters to avoid complication such as bone fracture.

A

V40: <64%

meandose: <37 Gy
maxdose: <59 Gy

66
Q

STS

What is the most significant acute radiation therapy toxicity of pre-operative RT?

A

wound healing problems and wound complications

67
Q

STS

What are the predictors of poor wound healing and pre-operative RT?

A 
tumor size > 10 cm,
lower extremity location,
tumor proximity to skin surface <3 mm,
vascularized flap closure, and diabetes mellitus
(Baldini et al.)
68
Q

STS

Subcutaneous tumors whether high-grade or low-grade can be treated with surgery alone using wide-excision.

TRUE or FALSE?

A

Excellent local control appears to be associated with wide resection performed for tumors in a subcutaneous location.

Rydholm et al. described only four LRs (5%) among 73 subcutaneous tumors treated with wide excision.

In addition, Gibbs et al. reported no LRs among 35 patients with subcutaneous STS treated with wide excision alone.
Among these tumors, 47% were high grade and 32% were >5 cm.

Pisters et al. described a prospective series of patients with tumors <5 cm who had negative resection margins and were treated with surgery alone.
The overall crude LR rate was 8%, and among the subcutaneous tumors, the rate was only 5%.

69
Q

STS

Give some parameters that must be met in the selection criteria if considering treatment with surgery alone

A
  • subcutaneous location
  • > 1 cm margin
  • low-grade
  • tumor locations amenable to limb-sparing salvage surgery upon recurrence
  • non-recurrent presentation
  • patient willingness to close follow-up
70
Q

STS

What are the 2 most commonly used drugs in the management of STS?

A

doxorubicin and ifosfamide

71
Q

STS

What is the most commonly used drug in the management of leoimyosarcoma?

A

gemcitabine

trabectidine

72
Q

STS

This is a complicated technique that has been used in Europe for the treatment of locally advanced STS that would otherwise require amputation.
This procedure involves isolating the arterial and venous circulation of the limb by connecting it to an extracorporeal circulation, where it is oxygenated and instilled with systemic agents, most commonly, melphalan and tumor necrosis factor.
A tourniquet is applied to the limb to prevent leakage into the systemic circulation and the limb is often treated with hyperthermia as well.
The treatment can have significant morbidity, but reported limb salvage success rates are quite high.

A

Isolated limb perfusion (ILP)

73
Q

STS
(from inservice bank)

  1. The following are Malignant tumors of Soft tissue except
    a. Adult Fibrosarcoma
    b. Angiomyofibroblastoma
    c. Myxofibrosarcoma
    d. Sclerosing epithelia fibrosarcoma
    e. none of the above
A

B

74
Q

STS
(from inservice bank)

  1. Histologic Grading of the tumor will provide the ff information except
    a. degree of malignancy
    b. predicts outcome
    c. probability of distance relapse
    d. treatment
    e. none of the above
A

E

75
Q

STS
(from inservice bank)

  1. A 2 cm sized high grade sarcoma without nodal and distant site involvement is staged:
    a. Stage 0
    b. I
    c. II
    d. III
    e. IV
A

D

76
Q

STS
(from inservice bank)

  1. For Primary Extremity Sarcoma, the factors that increased distant recurrence rates are the ff except.
    a. Large tumor size
    b. High grade tumor
    c. Histologic subtype of liposarcoma
    d. Deep location
    e. recurrent disease at presentation
A

C

77
Q

STS
(from inservice bank)

  1. The risk factors for local recurrence are the ff except
    a. Margin of resection
    b. histologic grade
    c. Age
    d. tumor size
    e. none of above
A

C

78
Q

STS
(from inservice bank)

  1. Using EBRT in the management of Sarcoma, one of the ff is the advantages of Preop EBRT
    a. Treatment volume is well define
    b. proven better compared to surgery alone treatment
    c. less risk for wound complication on surgery
    d. all of above
A

A

79
Q

STS
(from inservice bank)

  1. One of the following is true in the use of chemotherapy for Soft tissue sarcoma (STS)
    a. combination chemotherapy is superior to single agent regimen in Metastatic disease in terms of overall survival
    b. taxanes are the recommended single agent chemotherapy for STS
    c. In terms of tumor response, combination chemotherapy is superior to single agent regimen
    d. all of above
A

C

Ph RadOnc Residency PBRROE2-3 Review 2021 (57 decks)

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