Sarcoma Flashcards by Michael Pierro

Pagets disease can transform to what sarcoma?

osteosarcoma

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neurofibromatosis 1 puts you at higher risk for what sarcoma?

Malignant peripheral nerve sheath tumor

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Rb deletion puts a patient at higher risk for what sarcoma?

Osteosarcoma

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FAP has higher risk of what sarcoma?

desmoid tumor

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lymphedema puts a patient at increased risk of what sarcoma?

Angiosarcoma

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What is the single most important prognostic factor in osteosarcoma?

Grade

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What is different about osteosarcoma in jaw/mandible compared to other places?

Jaw has a very favorable biology, behaving more like a low grade

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What is the standard of care treatment for osteosarcoma?

Neoadjuvant MAP (MTX, adriamycin, cisplatin)
Surgery
If good response: post-op MAP
If not good reponse: Ifosfamide+MTX

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Patient with osteosarcoma receives pre-operative MAP and has a good response seen at surgery. What to do in adjuvant setting?

Continue MAP

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Patient with osteosarcoma treated with preoperative MAP has surgery and doesn’t have a good response. What to do in adjuvant setting?

Ifosfamide + MTX

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Three treatment options for recurrent osteosarcoma

Ifosfamide
Gem + Docetaxel
Regorafenib

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What are x-ray and pathology clues that a tumor is Ewing Sarcoma?

Found in diaphyses, in flat bones. Moth eaten appearance
On path: small, round, blue cell tumors

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What is the classic genetic change in Ewing Sarcoma?

t(11;22) EWS;FLI1 gene

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What is standard of care systemic treatment for Ewing sarcoma?

VAC/IE:
Vincristine, Doxo, Cyclophosphamide alternating with Ifos + Etop every 2 weeks

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What is the general treatment paradigm for chondrosarcoma?

Not sensitive to systemic therapy
Surgical resection as much as possible

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What chromosomal translocation is seen in alveolar rhabdomyosarcoma?

t(2;13)

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What chromosomal translocation has been seen in dermatofibrosaroma protuberans? How does that affect therapy?

t(17;22)
Imatinib

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What chromosomal translocation is seen in inflammatory myofibroblastic tumor? How does that affect treatment?

t(2;19)
Use ALK inhibitor

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What is the most important prognostic factor in soft tissue sarcoma?

Depth of invasion

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Who should get adjuvant RT in soft tissue sarcoma? (2)

Close margins
Tumors >5 cm

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For soft tissue sarcoma, who should NOT get limb-sparing surgery? (2)

Margin-free resection is impossible
Involvement of major vessels/nerves

Standard of care for first line chemotherapy for soft tissue sarcoma?

Doxorubicin + Ifosfamide

Second line chemotherapy for soft tissue sarcoma

Gemcitabine + Docetaxel

Eribulin is approved for what sarcoma

liposarcoma

Pazopanib is approved for what sarcoma population?

Non-liposarcoma

Tazemetostat is approved for what sarcoma?

Epithelioid

Nab-Sirolimus is approved for what sarcoma?

PEComas

Atezolizumab is approved for what sarcoma?

Alveolar soft tissue sarcoma

What TKI is approved for non-liposarcomas?

Pazopanib

1st line therapy for metastatic GIST

Imatinib

What mutations in GIST respond better to imatinib?

Exon 11 KIT mutation

What is standard second line therapy for metastatic GIST?

Sunitib

What is the standard third line therapy for metastatic GIST?

Regorafenib

What is the standard 4th line therapy for metastatic GIST?

Ripretinib

How do PGDFRA mutations affect treatment in metastatic GIST?

Relatively resistant to standard therapy. Use Avapritinib

Pexidartinib is approved to treat what sarcoma?

Tenosynovial giant cell tumor

What is the two most common mutations seen in GIST?

KIT (80-95%) PGDFR (5-10%)

You see a patient with FAP who develops desmoid tumor at the site of previous hemicolectomy. They are asymptomatic and it is not near any important structures. management?

Observation

For patients with GIST and KIT exon 9 mutation. How could their treatment differ from other patients?

Imatinib 800 mg has better PFS compared to 400 mg

Standard treatment for intraabdominal desmoid tumor?

Surgical resection. If asymptomatic and no imminent local invasion risk, can observe

Standard of treatment for desmoid tumors found outside the abdomen?

Complete excision with R0 resection.

What is the preferred chemotherapy for desmoid tumor?

Doxil or Doxorubicin

What are two non-chemotherapy systemic therapies for desmoid tumor?

Nirogacestat Sorafenib

What is a notable toxicity of Nirogacestat?

Ovarian dysfunction

What are indications for adjuvant imatinib in GIST? (4)

Tumor diameter >10 cm Tumor >5 cm and mitotic rate >5 Mitotic rate >10 Tumor rupture

In GIST, when indicated, how long do you give adjuvant imatinib for?

3 years

How do the surgical findings in a patient with Ewing sarcoma affect adjuvant treatment?

Doesn't affect systemic therapy (still needs adjuvant chemo (VAC/IE)) Positive margins needs adjuvant RT

Systemic therapy for unresectable giant cell tumor of the bone?

Denosumab

In GIST, what do Exon 17 KIT mutations mean for treatment?

Resistant to imatinib and sunitinib. Go to regorafenib

Preferred treatment for SDH deficient GIST?

Sunitinib or regorafenib or pazopanib