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Solid Tumor Oncology > Sarcoma > Flashcards

Sarcoma Flashcards

1
Q

Pagets disease can transform to what sarcoma?

A

osteosarcoma

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2
Q

neurofibromatosis 1 puts you at higher risk for what sarcoma?

A

Malignant peripheral nerve sheath tumor

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3
Q

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

A

Osteosarcoma

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4
Q

FAP has higher risk of what sarcoma?

A

desmoid tumor

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5
Q

lymphedema puts a patient at increased risk of what sarcoma?

A

Angiosarcoma

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6
Q

What is the single most important prognostic factor in osteosarcoma?

A

Grade

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7
Q

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

A

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

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8
Q

What is the standard of care treatment for osteosarcoma?

A

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

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9
Q

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

A

Continue MAP

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10
Q

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

A

Ifosfamide + MTX

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11
Q

Three treatment options for recurrent osteosarcoma

A

Ifosfamide
Gem + Docetaxel
Regorafenib

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12
Q

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

A

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

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13
Q

What is the classic genetic change in Ewing Sarcoma?

A

t(11;22) EWS;FLI1 gene

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14
Q

What is standard of care systemic treatment for Ewing sarcoma?

A

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

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15
Q

What is the general treatment paradigm for chondrosarcoma?

A

Not sensitive to systemic therapy
Surgical resection as much as possible

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16
Q

What chromosomal translocation is seen in alveolar rhabdomyosarcoma?

A

t(2;13)

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17
Q

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

A

t(17;22)
Imatinib

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18
Q

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

A

t(2;19)
Use ALK inhibitor

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19
Q

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

A

Depth of invasion

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20
Q

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

A

Close margins
Tumors >5 cm

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21
Q

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

A

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

22
Q

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

A

Doxorubicin + Ifosfamide

23
Q

Second line chemotherapy for soft tissue sarcoma

A

Gemcitabine + Docetaxel

23
Q

Eribulin is approved for what sarcoma

A

liposarcoma

24
Q

Pazopanib is approved for what sarcoma population?

A

Non-liposarcoma

25
Q

Tazemetostat is approved for what sarcoma?

A

Epithelioid

26
Q

Nab-Sirolimus is approved for what sarcoma?

A

PEComas

27
Q

Atezolizumab is approved for what sarcoma?

A

Alveolar soft tissue sarcoma

28
Q

What TKI is approved for non-liposarcomas?

A

Pazopanib

29
Q

1st line therapy for metastatic GIST

A

Imatinib

30
Q

What mutations in GIST respond better to imatinib?

A

Exon 11 KIT mutation

31
Q

What is standard second line therapy for metastatic GIST?

A

Sunitib

32
Q

What is the standard third line therapy for metastatic GIST?

A

Regorafenib

33
Q

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

A

Ripretinib

34
Q

How do PGDFRA mutations affect treatment in metastatic GIST?

A

Relatively resistant to standard therapy. Use Avapritinib

35
Q

Pexidartinib is approved to treat what sarcoma?

A

Tenosynovial giant cell tumor

36
Q

What is the two most common mutations seen in GIST?

A

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

37
Q

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?

A

Observation

38
Q

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

A

Imatinib 800 mg has better PFS compared to 400 mg

39
Q

Standard treatment for intraabdominal desmoid tumor?

A

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

40
Q

Standard of treatment for desmoid tumors found outside the abdomen?

A

Complete excision with R0 resection.

41
Q

What is the preferred chemotherapy for desmoid tumor?

A

Doxil or Doxorubicin

42
Q

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

A

Nirogacestat
Sorafenib

43
Q

What is a notable toxicity of Nirogacestat?

A

Ovarian dysfunction

44
Q

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

A

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

45
Q

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

A

3 years

46
Q

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

A

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

47
Q

Systemic therapy for unresectable giant cell tumor of the bone?

A

Denosumab

48
Q

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

A

Resistant to imatinib and sunitinib. Go to regorafenib

49
Q

Preferred treatment for SDH deficient GIST?

A

Sunitinib or regorafenib or pazopanib

Solid Tumor Oncology (21 decks)

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