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87 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Which sarcomas have a higher risk for LN spread? |
1) Synovial 2) Clear Cell |
SCARE |
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Median Age of Sarcoma |
45-55 yo |
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Describe the distribution of sarcoma primaries |
50% extremities |
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Sarcoma: 5 yr OS for all comers |
50-60% |
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Where do extremity sarcomas usually fail? |
Lung (within 2-3 yrs)
Exception: Myxoid liposarcoma tends to have nonpulmonary mets |
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Where do retroperitoneal sarcomas usually fail? |
Locally |
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Sarcoma Prognostic Factors |
Histology |
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Sarcoma Risk Factors |
RT |
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Which genetic conditions are associated with sarcoma? |
Neurofibromatosis |
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What is the preferred surgery and margin for sarcoma? |
Wide local excision with at least 1 cm margin |
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In sarcoma, what is the recurrence rate with: - Simple excisional biopsy? - Wide local excision? - Amputation? |
Excisional biopsy: 80% Wide local excision alone: 30-60% Amputation: 10-20% |
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When should you consider amputation? |
1) Involvement of major artery or nerve 2) Extensive skin involvement 3) Extensive bony involvement requiring whole bone resection 4) Failure of neoadjuvant chemo/RT 5) Recurrence after prior adjuvant RT |
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With surgery alone, what is the pattern of failure? |
20% local 35% distant |
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Describe the NCI #1 trial design (Rosenberg, Tepper) |
High grade sarcoma:
1) Amputation --> Post-op chemo 2) WLE --> RT (60-70 Gy) --> Adjuvant chemo
NB: Chemo was adriamycin, cyclophosphamide and methotrexate |
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What was the significance of NCI trial #1? |
Demonstrated equivalent DFS and OS with amputation vs limb-sparing surgery and adjuvant therapy
NB: Pts with positive margins had increased local recurrence |
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Describe the NCI #2 trial design (Yang et al 1998) |
High AND low grade sarcoma:
1) WLE alone 2) WLE --> RT to 63 Gy
NB: High grade pts also got adjuvant chemo |
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What was the significance of NCI trial #2 (Yang et al 1998)? |
Demonstrated that limb-sparing surgery withOUT adjuvant RT results in worse local control
NB: True in both low and high grade sarcoma No change in OS (bc of distant mets) |
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Advantages of pre-op RT in sarcoma |
hjk |
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Of extremity sarcomas, what percentage involves the leg?
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75%
NB: 75% of those are above the knee. |
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Name the five most common sarcoma histologies
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Undifferentiated pleomorphic sarcoma (formerly MFH)
Liposarcoma Leiomyosarcoma Synovial MPNST |
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Synovial sarcoma translocation
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t(X;18)
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Clear cell translocation
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t(12;22)
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Ewing sarcoma/PNET translocation |
t(11;22)
NB: Philadelphia chromosome is t(9;22) |
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Alveolar rhabdomyosarcoma translocation |
t(2;13) and t(1;13) |
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Which type of sarcoma is associated with Gardner syndrome? |
Desmoids |
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What percentage of sarcomas present with LN involvement? |
5% |
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Which sarcoma histology is less likely to spread to lung? |
Myxoid liposarcoma |
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Risk factors that predict local recurrence in sarcoma |
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Risk factors that predict distant failure in sarcoma |
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Sarcoma: T1a |
≤5 cm and superficial |
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Sarcoma: T1b |
≤5 cm and deep |
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Sarcoma: T2a |
>5 cm and superficial |
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Sarcoma: T2b |
>5 cm and deep |
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Sarcoma: N1 |
Any regional nodes |
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Is a retroperitoneal sarcoma considered superficial or deep? |
ALWAYS deep |
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Sarcoma: Stage IA |
T1a or T1b, grade 1 |
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Sarcoma: Stage IB |
T2a or T2b, grade 1 |
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Sarcoma: Stage IIA |
T1a or T1b, grade 2-3 |
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Sarcoma: Stage IIB |
T2a or T2b, grade 2 |
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Sarcoma: Stage III |
T2a or T2b, grade 3 N1 |
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Sarcoma: Stage IV |
M1 |
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Preferred biopsy method in sarcoma |
Core needle Excision biopsy oriented so that it may be excised during surgery |
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In which patients with extremity sarcoma can you forego adjuvant RT? |
Low grade (stage 1) sarcoma s/p surgery with at least 1 cm clear margins |
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Which sarcoma study?
Amputation vs limb-sparing + EBRT |
NCI #1 (Rosenberg) |
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Which sarcoma study?
WLE alone vs WLE + EBRT |
NCI #2 (Yang et al) |
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Which sarcoma study?
WLE alone vs WLE + postop brachy |
Pisters 1996 |
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Describe the Pisters 1996 trial design |
High and low grade sarcoma:
1) WLE alone 2) WLE + intraoperative brachy (42-45 Gy over 4-6 days) |
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Significance of Pisters 1996 trial |
Demonstrated improved local control with brachytherapy in high grade sarcoma with negative surgical margins
NB: No change in DFS or OS |
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Which sarcoma study?
Preop vs postop RT |
NCI Canada (O'Sullivan/Davis) |
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Describe the NCI Canada trial design |
Extremity sarcoma
1) Preop RT to 50 Gy/25 fx --> surgery --> 16-20 Gy boost for +margin 2) Surgery --> 50 Gy/25 fx + 16-20 Gy boost
Primary endpoint: Wound complications |
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What was the significance of NCI Canada (O'Sullivan/Davis)? |
1) Demonstrated significantly more wound complications with preop vs postop RT (particularly in the upper thigh) 2) Worse long-term fibrosis with post-op 3) No difference btwn the two in local control, DFR or OS
NB: Closed early |
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What were the wound complication rates in NCI Canada (O'Sullivan/Davis)? |
Pre-op: 35% Post-op: 17%
NB: Trend twds worse fibrosis and stiffness long-term in the post-op group (NS) |
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Advantages of preop RT in sarcoma |
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Advantages of postop RT in sarcoma |
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Is there evidence to support the use of chemo in sarcoma? |
British meta-analysis shows some benefit in LC, DMFS and DFS
NB: Largest benefit in high grade sarcoma of the extremity |
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General management of sarcoma with preop RT (NCCN) |
50 Gy/25 fx --> Surgery with clips --> Boost for positive margins*
EBRT: - 16-18 Gy for micro - 20-26 Gy for gross HDR Brachy: - 14-16 Gy at 3-4 Gy/fx for micro - 18-24 Gy for gross IORT: - 10-12.5 Gy for micro - 15 Gy for gross
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General management of sarcoma with post-op RT (NCCN) |
Surgery with clips --> 50 Gy plus boost
EBRT Boost: - 10-16 Gy negative margins - 16-18 Gy micro - 20-26 Gy gross |
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Dose for post-op LDR brachy for sarcoma (NCCN) |
45 Gy over 4-6 days |
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Dose for post-op HDR brachy for sarcoma (NCCN) |
36 Gy/10 fx BID (3.6 Gy/fx) |
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When is bone at the greatest risk of fracture following sarcoma RT? |
≤18 months |
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Follow-up for sarcoma |
H&P and chest imaging:
Imaging of primary as indicated OT/PT for functional rehabilitation |
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Which STS patients may be observed after surgery? |
Any low grade pt with negative margins OR
Stage IA (T1a-b and low grade) with any margins
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Which sarcoma patients should receive RT at some point? |
Stage IB with close/positive margins Stage IIA and above |
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What is a hemangiopericytoma? |
Malignant tumor originating from fibroblasts and associated with vessels - Most commonly occurs in the leg. Can also appear in the retroperitoneum, H&N and meninges of the CNS. |
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How often does hemangiopericytoma spread distantly? |
25%, mostly to lung |
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General tx strategy for hemangiopericytoma |
Maximal safe resection --> RT to 60-65 Gy (site dpdt) |
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5 yr OS for hemangiopericytoma |
70% with complete resection |
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What is the natural history of desmoid tumors? |
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Desmoid tumors are associated with which gene mutation? |
APC (2%) |
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What percentage of desmoids are intraabdominal? |
10-30%
NB: Often associated with Gardner syndrome |
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Risk factors for development of desmoid tumors |
Gardner syndrome ?Trauma ?Pregnancy |
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Workup for desmoid tumors |
Complete H&P CT or MRI of the involved region Biopsy
NB: Desmoids have no metastatic potential so systemic imaging is NOT required. |
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What is the local recurrence rate for desmoid tumors? With and without RT? Positive or negative margins? |
Surgery alone: LR 13% vs 52% with a positive margin Surgery + RT: LR 7% vs 26% with a positive margin |
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Which group of patient with desmoid tumor should not routinely be offered adjuvant RT following surgery? |
Negative margins* Retroperitoneal/intraabdominalh
*RT can be considered for very large lesions |
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Dose for adjuvant RT in desmoid tumor |
Adjuvant: 50 Gy Definitive: 54-58 Gy |
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Target volume in desmoids |
Cover gross tumor/tumor bed and tracking structures in the compartment (nerves, vessels) + 3-5 cm margin longitudinally |
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Nonsurgical, nonradiation options for desmoid tumor |
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What is MAID? |
Mesna Adriamycin Ifosfamide Dacarbazine
Chemo combination used in sarcoma |
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Which chemotherapy agent is most often used in GIST? |
Imatinib (Gleevec)
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Do you need a biopsy in retroperitoneal sarcoma? |
If there is very high suspicion and the plan is to do surgery first, then NO.
If there is doubt or a plan to do any neoadjuvant therapy, then yes.
NB: Core needle is preferred. |
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Which genetic mutation is associated with GIST? |
85-95% are KIT+
NB: Also called CD117 |
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How common are LNs in GIST? |
Extremely rare |
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Where does GIST spread to? |
Locally Liver or peritoneum |
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Biopsy in GIST |
EUS-guided is preferred over percutaneous |
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When should preop imatinib be considered in GIST? |
When use of the drug could potentially reduce surgical morbidity |
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Rate of recurrence after complete resection in GIST |
50% |
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What role does RT play in GIST? |
Minimal Maybe palliative for mets? |
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