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Sarcoma Flashcards

1
Q

What is the AJCC 8th edition T classification of STS?

A
  • T1: ≤ 5cm
  • T2: > 5cm ≤ 10 cm
  • T3: > 10 cm ≤ 15 cm
  • T4: > 15 cm
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2
Q

What are the AJCC 8th edition N and M classifications of STS?

A
  1. N
    - N0: no lymph nodes
    - N1: regional LN metastases
  2. M
    - M0: none
    - M1: distant metastasis
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3
Q

What are the AJCC 8th ed. prognostic stage groups STS?

A
  • IA: T1 NO MO G1 or GX
  • IB: T2,T3,4 NO MO G1 or GX
  • II: T1 NO MO G2-3
  • IIIA: T2 NO MO G2, G3
  • IIIB: T3, T4 NO MO G2, G3
  • IV: N1 or M1

Note that N1 or M1 automatically makes a pt stage IV

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4
Q

What is the AJCC 8th edition TNM classification of bone sarcomas?

A
  • T
    – T1: ≤8cm
    – T2: > 8cm
    – T3: Discontinuous tumors in the primary bone
  • LNs
    – N0: LN-
    – N1: LN+
  • Distant Metastases:
    – M0: none
    – M1a: Lung
    – M1b: Bone or other distant sites
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5
Q

What are the AJCC 8th ed. prognostic stage groups RP sarcomas?

A
  • IA- T1 NO MO G1 or GX
  • IB- T2,T3,T4 NO MO G1 or GX
  • II- T1 NO MO G2-3
  • IIIA- T2 NO MO G2, G3
  • IIIB- T3, T4 NO MO G2, G3
    – IIIB- N1
  • IV: M1
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6
Q

What are some common sites of metastases for retroperitoneal sarcomas?

A
  • Liver
  • Lungs
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7
Q

What are some common histologies of RP sarcomas?

A
  • Leiomyosarcoma
  • Liposarcoma
  • Undifferentiated STS
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8
Q

Of all sarcomas, what % are RP sarcomas?

A

10-15%

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9
Q

What was the pt population, randomization, and primary endpoint of NCIC pre-op vs. post-op RT for extremity STS?

A
  • Pts: Extremity STS
  • Randomization:
    – Pre-op RT: 50 Gy
    — Post-op boost of 16-20 Gy for +margins
    – Post-op RT: 66-70 Gy
  • Endpoint: Rates of wound complications
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10
Q

What were the results of NCIC pre-op vs. post-op RT for extremity STS?

A
  • Pre-op vs. Post-op:
    – Acute wound complications: 35% vs. 17%
    – Late effects:
    — Fibrosis, ≥ Gr 2: 32% vs. 48% (p=0.007)
    — Edema: 15% vs. 23%
    — Joint stiffness: 18% vs. 23%
    – OS: 72% vs. 85% (p=0.04)
    — Trial was not powered to detect this difference
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11
Q

What are the adverse prognostic factors for distant recurrence in extremity STS?

A

Distant recurrence:
- Intermediate/large tumor size
- High grade
- Deep location
- Recurrent disease at presentation
- Leiomyosarcoma

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12
Q

What are the adverse prognostic factors for local recurrence in extremity STS?

A
  • Age (>/< 50)
  • Size (>/< 5 cm)
  • margins (+ vs. close vs. -)
  • Grade (high vs. low)
  • Previous recurrence
  • Histologic subtypes
    – fibrosarcoma
    – malignant peripheral-nerve tumor

MSKCC Nomogram

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13
Q

What is the most common sarcoma in adults?

A
  • Leiomyosarcoma
  • 20-25% of all STS
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14
Q

Which genetic alteration is a/w leiomyosarcoma?

A
  • 12q amplification including MDM2 and CDK4 genes
  • Shared by well-differentiated and dedifferentiated leiomyosarcoma
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15
Q

What are the different subtypes of liposarcoma?

A
  • Well-differentiated/dedifferentiated liposarcoma
  • Myxoid/round cell liposarcoma
  • Pleomorphic liposarcoma.
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16
Q

Which genetic alteration is a/w Ewing sarcoma?

A
  • t(11;22) translocation
  • Present in ~85%
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17
Q

Which genetic alteration is a/w myxoid round cell liposarcoma?

A

t(12;16) → FUS-DDIT3 fusion gene

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18
Q

Which genetic alteration is a/w RMS?

A
  • t(1;13) → PAX7/FOX01 fusion
  • t(2;13) → PAX3/FOX01 fusion
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19
Q

Which genetic alteration is a/w alveolar soft part sarcomas?

A

t(x;17) → ASPL-TFE3 fusion gene

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20
Q

Which genetic alteration is a/w synovial sarcomas?

A

t(x;18) → SS18-SSX1 fusion gene

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21
Q

Which genetic alteration is a/w clear cell sarcomas?

A
  • t(12;22) → ATF1-ESWR1 fusion gene
  • Present in ~90% cases
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22
Q

What was the pt population, randomization, and primary endpoint of the STRASS 1 trial for sarcomas?

A
  • Pts:
    – Sarcomas of the retroperitoneal or intraperitoneal spaces
  • Randomization:
    – pre-op RT (50.4 Gy in 28 fx) → surgery (multi-visceral en bloc resection)
    – surgery alone
  • Endpoint:
    – Abdominal RFS
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23
Q

What were the results and the official main conclusion of the STRASS 1 trial for sarcomas?

A
  • Median FU 43.1 mos
  • RT + surgery vs. surgery only
    – 5-yr crude abdominal recurrence: 40-50% both arms
    – Median abdominal RFS: 4.5 yrs vs 5 yrs (p=0.95)
    – Liposarcoma 3-yr RFS: 71.6% vs. 60.4%
  • Conclusion: preoperative RT should not be considered a SOC treatment for RP sarcoma
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24
Q

What are the main RO criticisms of the STRASS 1 trial for sarcomas?

A
  • RT showed a large absolute reduction in local recurrences despite not affecting the primary endpoint. Surgery alone vs. RT + Surgery
    – LR following RO/R1: 29% vs. 12%
    – LR for all comers: 29% vs.15%
  • R1/2 margins are common in SP sarcomas, yet the trial only required 50.4 Gy RT, which is inadequate
  • Tox rates in the radiotherapy plus surgery arm were unexpectedly high (77% grade 3-4) likely 2/2 poor RT protocol compliance (26% major deviation rate)
  • ROs were allowed to deviate from international guidelines for RP sarcoma
  • Did not require several OAR constraints
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25
Q

What is the LR rate for RP sarcomas s/p any tx?

A
  • 5-yrs: 25%
  • 8-yrs: 31%
  • 10-yrs: 35%
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26
Q

When can brachytherapy monotherapy alone be considered for int/high-grade sarcoma s/p resection?

A
  • size < 10 cm
  • High-grade tumors
  • -margins

Pisters trial (JCO 1996) showed that brachytherapy improved LC in high-grade and -margins, but NOT in +margins.

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27
Q

What doses are used for brachytherapy monotherapy alone for int/high-grade sarcoma s/p resection w/ -margins?

A
  • LDR/PDR: 45-50 Gy
    – NCCN pref: 45 Gy
  • HDR: 30-54 Gy (2-4.5 Gy fx given BID)
    – 36 Gy in 3.6 Gy BID over 10 fx
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28
Q

What doses are used for brachytherapy boost post-EBRT for int/high-grade STS s/p resection?

A
  • LDR/PDR: 16-20 Gy
  • HDR: 14-16 Gy in (2-4.5 Gy fx given BID)
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29
Q

Which sarcomas metastasize to LNs and at what rates?

A
  • CARE (lowest to highest rates)
    – Clear cell sarcoma: 11.1%
    – Angiosarcoma: 11.1%
    – RMS: 19%
    – Epitheliod sarcoma: 20%
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30
Q

What is the expected rate of ≥ Gr 3 GI tox in RP sarcomas tx w/ pre-op RT f/b surgery?

A

~ 5%

Toronto Cross-Sectional Study

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31
Q

What is the per million incidence of RP sarcomas?

A

2.7 per 1 million

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32
Q

What is the 10-year survival of chondrosarcoma grade 1 s/p R0 GTR?

A
  • ~80%
    – Grade 1 chondrosarcomas are classified by WHO as “atypical cartilaginous tumors”
    – Considered locally aggressive neoplasms and not malignant tumors
    – Rarely metastasize
33
Q

What is the LC and median OS benefit of IORT w/ low-dose RT vs. high-dose RT in post-op per Sinderlar et al. 1993?

A
  • IORT + LD-EBRT vs. HD-EBRT
    – LRR: 40% vs. 80% (Sig.)
    – Median OS: 45 vs. 52 mo (NS)
34
Q

What is the rate of tumor regrowth during RT for sarcomas?

A
  • 45%!
  • Mostly happens in the first 2 weeks
35
Q

What is the rate of tumor shrinkage during RT for sarcomas?

A

33%

36
Q

Why are large margins and CBCT at least twice weekly recommended when radiating sarcomas?

A

Because around 45% of tumors can grow during RT, leading to underdosing if inadequate margins are used or tumor growth is not monitored w/ CBCT

37
Q

What factors predict a higher rate of major wound complications for extremity or trunk STS s/p pre-op RT and surgery?

A
  • Diabetes
  • Tumor > 10 cm
  • Tumors < 3 mm from the skin
  • Use of vascular flap or STSG

Baldini et al, 2013

38
Q

What are the 8-yr LRR for RP liposarcomas and leiomyosarcoma?

A
  • 8-yr LRR:
    – Leiomyosarcoma: 10%
    – Well-differentiated liposarcoma: 35%
    – Dedifferentiated liposarcoma grade 2-3: 48%
39
Q

What is the risk of fracture after surgery and RT for extremity sarcoma?

A

4-8%

40
Q

Which factors predict a higher risk of fracture after surgery and RT for extremity sarcoma?

A
  • Sex (Fem > Male)
  • Age (> 55 yrs)
  • Radiation dose (50 Gy vs. 60-66 Gy)
  • Compartment
  • Tumor size
  • Periosteal stripping
41
Q

How is the risk of fracture after surgery and RT for extremity sarcoma related to RT dose?

A

5-yr risk of fracture
- 50 Gy: 0.6%
- 60-66 Gy: 7%

42
Q

What dosimetric constraints for the bone may be used to reduce the risk of fracture?

A
  • V40 <64%
  • Dmean <37 Gy
  • Dmax <59 Gy
  • Avoid circumferential bone coverage with the 50 Gy IDL
43
Q

Which syndrome is characterized by chronic lymphedema that leads to the formation of soft tissue sarcomas (STS)?

A
  • Stewart Treves Syndrome (STS)
    – Rare
    – Soft tissue sarcoma (STS)
    – Occurs in women w/ lymphedema after breast cancer therapy
    – No effective therapy

STS is a/w STS!

44
Q

What kind of sarcoma is formed during Stewart Treves Syndrome (STS)?

A

Angiosarcoma

45
Q

Which sarcoma has no AJCC staging system?

A
  • Kaposi
  • Use AIDS Clinical Trial Group (ACTG) TIS (tumor, immune system, systemic illness) system
46
Q

Which adj. (or neoadj.) CHT regimen has demonstrated benefit in

A
  • Doxorubicin ± Ifosfamide (best together)
    – LRR, Distant recurrence, and OS benefit
    – Used for pts at high risk of developing mets
    — Tumor > 5 cm
    — Int-high grade
47
Q

What was the pt population, randomization, and primary EP of the NCI amputation vs. limb-sparing trial (Rosenberg et al. 1982)?

A
  • Pt: High-grade extremity STS
  • Randomization:
    – WLE + post-op 50 Gy + 10-20 Gy boost + adj CHT
    – Amputation + adj CHT
  • Adj CHT: Doxorubicin, Cylophosphamide, MTX
  • Primary EP:
48
Q

What were the results of the NCI limb-sparing trial?

A
  • RT vs. Amputation:
    – LR 15% vs. 0% amputation (p=0.06)
    – 5-yr DFS: 71% vs. 78% (NS)
    – 5-yr OS: 83% vs. 88% (NS)
49
Q

What were the pt population, randomization, and primary EP of RTOG 0630 for extremity STS?

A
  • Randomization:
    – Cohort A: Pre-op IGRT w/ CHT (arm closed early and not reported)
    – Cohort B: Pre-op IGRT w/o CHT
    – IMRT or 3DCRT allowed. 50 Gy. IMRT used in 75% of patients
  • Primary EP: Late toxicities
50
Q

What were the results and conclusions of RTOG 0630 for extremity STS?

A
  • Results were compared to the preoperative arm of the CAN-NCIC-SR2 trial (Davis et al Radiother Oncol 2005).
    – 2-yr LC was 94%
    — All local failures were within the CTV
    – Grade 2 late toxicity (subcutaneous tissue fibrosis, joint stiffness, or edema) was 10.5% in this RTOG 0630 trial vs. 37% (P<0.001)
    – Grade 2 or greater tox:
    – FIbrosis: 5.3% vs. 31.5%
    – Stiffness: 3.5% vs. 17.8%
    – Edema: 5.3% vs 15.1%
51
Q

What was the RT dose and how were RT volumes drawn in RTOG 0630 for extremity STS?

A
  • Dose
    – 50 Gy in 25 fx
  • Contouring
    – Grade 2/3, Size > 8 cm
    — CTV = GTV + 3 cm sup/inf + 1.5 cm radial
    – Grade 2/3, Size ≤ 8 cm
    — CTV = GTV + 2 cm sup/inf + 1 cm radial
52
Q

When should the drain site be included in the CTV for post-op RT for extremity STS?

A
  • Large CTV >5 cm
  • Intermediate and high-grade tumors
  • R1 resections
53
Q

What are the different types of RT-induced STS?

A
  • HALF:
    1. High-grade undifferentiated pleomorphic sarcoma (26%)
    2. Angiosarcoma (21%)
    3. Leiomyosarcoma (12%)
    4. Fibrosarcoma (12%)
54
Q

Do synovial sarcomas metastasize to LNs?

A

No!

55
Q

What is the general tx paradigm for Dermatofibrosarcoma protuberans (DFSP)?

A
  • Surgical resection w/ -margins
  • Surgical resection w/ close (<1cm) or +margins → PORT
56
Q

What is Dermatofibrosarcoma protuberans (DFSP)?

A
  • Locally aggressive cutaneous STS
  • Very rare
  • ↑ local recurrence
  • ↓ metastatic spread
57
Q

Which genetic abnormality is a/w Dermatofibrosarcoma protuberans (DFSP)?

A
  • (t(17;22) (q22;13)
  • PDGTB/COL1A1 gene product
  • Leads to continuous activation of the PDGF receptor b (PDGFRB), which is a tyrosine kinase.
58
Q

What are the most common locations for Ewings sarcoma?

A
  • Pelvis: 26%;
  • Femur: 20%;
  • Chest wall: 16%
    – Ribs: 10%
    – Scapula: 4%
    – Clavicle: 1.5%
    – Sternum: 0.5%
59
Q

What are the most common locations for osteosarcoma?

A
  • Femur (42%, with 75% of these tumors in the distal femur)
  • Tibia (19%, with 80% of these tumors in the proximal tibia)
  • Humerus (10% w/ 90% of these in the proximal humerus)
  • Skull and jaw
  • Pelvis (8%);
  • Ribs (1.25%)
60
Q

What are the absolute contraindications for the resection of RP sarcomas?

A
  1. Spinal cord involvement
  2. Distant metastases
  3. Peritoneal implants
  4. Involvement of the superior mesenteric vessels
  5. Extensive vascular involvement (e.g. aorta)
61
Q

Is involvement of inferior vena cava or iliac veins, a contraindication to RP sarcoma resection?

A

Yes, it is a relative contraindication

62
Q

What are the OAR constraints for Pre-op RT for RP sarcomas?

A
  1. Stomach and duodenum:
    - V45 ≤ 100%, V50 < 50%, Max Dose 56 Gy
  2. Small and large bowel contoured as peritoneal cavity
    - V15 < 830 cm3 , V45 ≤ 195 cm3
  3. Small bowel contoured as individual loops
    - V15 < 120 cm3 , V55 < 20 cm3
  4. Large bowel contoured as individual loops
    - V60 < 20 cm3
63
Q

What is the def. RT dose for desmoid tumors?

A

56 Gy in 28 fx

64
Q

What is the characteristic protein expressed by chordomas?

A
  • Brachyury
  • It is ranscription factor protein that controls the development of notochord and mesoderm in humans
65
Q

What is the tissue of origin of chordomas?

A
  • Notochord remnants
  • Can be found from the base-of-skull to sacral spine
66
Q

What is the median age at dx of chrodomas?

A
  • 60 yrs
  • Base of skull chordomas present earlier
67
Q

What proportion of chordomas arise at the base of the skull?

A

1/3 (clival region)

68
Q

What proportion of chordomas arise from the sacrococcygeal region?

A

1/2

69
Q

What is the general tx approach to chordomas?

A

Surgical resection ± RT

70
Q

What cancers/tumors are a/w Li-Fraumeni syndrome?

A
  • LABS
    1. Leukemia
    2. Adrenal Gland Cancer
    3. Breast
    4. Sarcoma
71
Q

What is Gardner syndrome, and what cancers/tumors are a/w it?

A
  1. Subtype of Familial Adenomatous Polyposis (FAP).
  2. Autosomal dominant
  3. Results from mutations in the APC gene on chromosome 5.
  4. Multiple colon polyps
  5. Cancer/tumor associations:
    - Osteomas of the skull
    - Fibromas
    - Thyroid cancer
    - Epidermoid cysts
    - Desmoid tumors
72
Q

What is Cowden Syndrome, and what cancers/tumors are a/w it?

A
  1. aka Multiple hamartoma syndrome
  2. Autosomal dominant
  3. a/w PTEN mutations
  4. Cancer/tumor associations:
    - Benign Gl hamartomas
    - Increased risk of several malignancies
    – Breast
    – Thyroid
    – Uterine
73
Q

What is field cancerization and which sarcoma is usually a/w it?

A
  • Def: Large areas of cells at a tissue surface or within an organ are affected by carcinogenic alterations
  • a/w angiosarcoma
  • poor prognosis
74
Q

Which MRI sequence is used to delineate pre-op GTV for sarcomas?

A
  • T1 post-contrast
75
Q

How do you delineate CTV for RPS?

A
  • When using 4D CT Sim
    – Geometric 1.5 cm expansion on iGTV
  • When not using 4D CT Sim
    – Cephalo-caudad: 2-2.5 cm
    – Radial: 1.5-2 cm
76
Q

What are the approaches to managing STS s/p non-oncologic resection w/ grossly + margin (R2)?

A
  1. Preferred: Pre-op RT followed by re-resection
  2. Adjuvant RT alone to a dose of 66-70 Gy, if the patient is not a further surgical candidate
    3) Observation to allow the disease to ‘declare’ itself at which point a pre-op and surgery approach.
77
Q

What were the findings of the NCI limb-sparing trial (Yang et al, 1998)?

A
  • STS s/p limb-sparing surgery and -margins (1-2 cm) → observation vs. EBRT (45 Gy + 18 Gy boost to tumor bed)
    – Gross residual disease or +margins were excluded
    – Low-grade tumors LC: 60 vs. 95% (SS)
    – High-grade tumors LC: 75 vs. 100% (SS)
    – 10-yr OS ~75% (NS)
78
Q

What is the age of dx of osteosarcoma?

A
  • Bimodal distribution:
    – 10-24 yrs old
    – >70 yrs old
79
Q

What are the most common sites of STS in adults?

A
  • Lower extremity 46.4%
  • Trunk 18%
  • Upper extremities 13%
  • Retroperitoneum 12.5%
  • Head and neck 9%
  • Mediastinum 1.3%

American college of surgeons

Sarc-Skin (2 decks)

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