Bone Tumor

Bone Tumors

Bone Tumors Part 1

Has a phase 3 trial demonstrated an OS benefit with any particular neoadjuvant treatment for osteosarcoma ?

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Yes, cis/dox shown to potentially have superior OS vs MAP

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Bone Tumors

What was MAP shown to be superior to in first-line osteosarcoma ?

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Doxo/Cisplatin

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What is the MAP regimen used in osteosarcoma ?

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MTX/Doxo/Cisplatin

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Is there randomized data showing an OS improvement in first-line metastatic osteosarcoma ?

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Yes, MAP regimen

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Is there randomized data showing an OS improvement in second-line metastatic osteosarcoma ?

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No

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Which regimen for Ewing Sarcoma has shown an OS improvement from a randomized phase 3 trial in the neo-adjuvant setting ?

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VACA – IE

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Has Auto-HSCT in the adjuvant setting for high risk localized Ewing Sarcoma proven to improve OS ?

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Yes

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What did the R2Loc trial assess in Ewing Sarcoma ?

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Adjuvant Auto-HSCT vs standard chemo (VAI) in high-risk localized Ewing Sarcoma

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Which phase III trial has demonstrated OS or PFS benefit in relapse/refractory Ewing Sarcoma ?

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None

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What does the exon 9 mutation for GIST imply?

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Imatinib 800mg has more response than 400mg

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Which translocation and which treatment are used in dermatofibrosarcoma?

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t(17;22) – imatininb

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Which chemotherapy regimen is used for osteosarcoma undergoing curative intent treatment?

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MAP – methotrexate, doxorubicin, cisplatin

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What is another name for cKIT?

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CD117

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Which 2 mutations drive most GIST?

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cKIT and PDGFR

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What other IHC stain corroborates a diagnosis of GIST besides cKIT?

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DOG1

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What TKI is active in liposarcoma?

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Erlotinib

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Which cytogenetic changes are seen with Ewing sarcoma?

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T(11;22)

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Which osseous landmarks are common sites for Ewing Sarcom and osteosarcoma?

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Osteosarcoma–metaphysis
Ewing sarcoma–diaphysis

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What is the general treatment pathway for osteosarcoma?

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chemotherapy –> surgery –> chemotherapy +/- XRT

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Bone Tumors

Where is the most common osseous site for osteosarcoma?

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Metaphysis

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What is the most common primary bone sarcoma?

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Osteosarcoma

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Bone Tumors

Which genetic mutation is responsible for desmoid tumors?

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APC mutation

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Bone Tumors

Which soft tissue sarcoma subtype has a propensity for brain metastases?

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Soft alveolar soft tissue sarcoma

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Where is the most common metastatic site for soft tissue sarcoma?

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Lungs

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What is the proper clinical staging for N1 STS?

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stage III

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Which are the 2 curable soft tissue sarcomas that need to be distinguished?

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Rhabdomyosarcoma and Ewing sarcoma

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Which subtype of soft tissue sarcoma is trabectedin highly active in?

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Liposarcoma

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Which STS histology is pazopanib not approved for?

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Liposarcoma

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What is the size cut-off between a T1 and T2 soft tissue sarcoma?

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5 cm

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Which 2 osseous sarcomas require chemotherapy?

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Osteosarcoma and Ewing sarcoma

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What are the 5 most common primary osseous tumors?

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Osteosarcoma, Ewing sarcoma, chordoma, chondrosarcoma, giant cell tumor

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How do you treat a solitary fibrous soft tissue sarcoma?

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Bevacizumab

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Bone Tumors

Which chemotherapy agent is most active in angiosarcoma?

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Taxanes

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How do you treat a PEComa?

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Sunitinib

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Which chemotherapy regimen is preferred for synovial sarcoma?

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ITP

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Bone Tumors Part 2

Which sarcoma treatment agent is a vesicant ?

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Trabectidine, vinka alkaloids, anthracyclines, cisplatin (at high enough concentration)

Bone Tumors Part 2
Bone Tumors Part 2

What did the PALETTE trial assess ?

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Pazopanib in a randomized fashion versus placebo in metastatic sarcoma, showed PFS improvement

Bone Tumors Part 2
Bone Tumors Part 2

What is the treatment for dermatofibrosarcoma protuberans ?

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Imatinib

Bone Tumors Part 2
Bone Tumors Part 2

Has sorafenib been proven to improve PFS or OS in a randomized trial for desmoid tumors ?

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Yes, Gounder et al. Alliance trial

Bone Tumors Part 2
Bone Tumors Part 2

Which cytotoxic agents are active in metastatic rhabdomyosarcoma ?

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Vincristine, dactinomycin, etoposide, doxorubicin, ifosfamide, cyclophosphamide

Bone Tumors Part 2
Bone Tumors

Is there are targeted therapy for synovial sarcoma?

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No

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which soft tissue sarcoma has an ALK translocation?

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Inflammatory myofibroblastic tumor (IMT)

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Which 2 genes are mutated in sporadic and familial GIST?

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cKIT and PDGFRa

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Bone Tumors

How do you treat CDK4 amplified well-differentiated liposarcoma?

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Palbociclib

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How do you treat DFP with a t(17;22) translocation?

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Imatinib

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Which chromosomal translocation is seen with dermatofibroma protuberans (DFP)?

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t(17;22)
cKIT

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Which cytogenetic changes are seen with a well-differentiated liposarcoma?

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Supernumerary ring and giant marker chromosome

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Which chromosomal aberrations are seen in a high percentage of Ewing sarcoma?

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22q12 EWSR1 gene
t(11;22), t(21;22), t(2;22), t(7;22), t(17;22)

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Bone Tumors

At what age do you start to see less primary localized osseous tumors and more metastatic osseous tumors?

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40 years old

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What extra treatment option is available for low-grade versus intermediate–high grade
chondrosarcoma?

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Low-grade=intralesional excision with or without surgery or wide local excision or radiotherapy

Intermediate–high grade = wide local excision or radiotherapy

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Where do the 2 types of chordoma arise?

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Sacrum and skull base are 85%

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What are the 3 variants of chordoma?

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Chondroid, conventional, dedifferentiated

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How do you treat dedifferentiated chordoma?

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use an Ewing regimen

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What are the treatment options for skull base and sacrococcygeal tumors?

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Skull base intralesional versus wide local excision

Sacral coccyx–wide excision versus radiotherapy

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What surgical option is there for low-grade bone tumors with positive margins that are difficult to
obtain?

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Intralesional excision followed by bone graft

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What are treatment options for systemic chordoma?

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Imatinib, erlotinib, sunitinib, lapatinib, sorafenib

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What is the minimum number of weeks to give neoadjuvant VAC/IE for Ewing sarcoma?

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9 weeks

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What is the general modality of treating Ewing sarcoma that is metastatic?

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VAC/IE followed by excision or chemotherapy or amputation followed by further VAC/IE

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How do you treat positive margins for Ewing sarcoma?

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Radiotherapy

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Which drugs are active in Ewing sarcoma?

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Vincristine, Adriamycin, cyclophosphamide, ifosfamide, etoposide
Second line–gemcitabine, docetaxel, temozolomide, topotecan

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How do you treat giant cell tumor?

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Denousumab and interferon alfa-2b

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What treatment options are available for giant cell bone tumors which were not fully resectable
secondary to morbidity?

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Serial embolization

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Bone Tumors

Is radiation active in giant cell tumors?

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Yes

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Which types of osteosarcoma can be treated up front with wide excision?

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Periosteal osteosarcoma
Low-grade osteosarcoma

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How do you treat high-grade local osteosarcoma?

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Preoperative chemotherapy followed by wide excision
Positive margins treated with further surgery, radiation and chemotherapy
Negative margins given adjuvant chemotherapy
Good response to chemo with

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Bone Tumors

Which first-line chemotherapy agents are used for metastatic osteosarcoma?

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Doxorubicin plus cisplatin
Methotrexate + cisplatin + doxorubicin +/- ifosfamide

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What second line options are available for metastatic osteosarcoma?

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Paclitaxel, gemcitabine, cyclophosphamide, etoposide, samarium, radium, Sorafenib

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