Sarcoma Flashcards
Pagets disease can transform to what sarcoma?
osteosarcoma
neurofibromatosis 1 puts you at higher risk for what sarcoma?
Malignant peripheral nerve sheath tumor
Rb deletion puts a patient at higher risk for what sarcoma?
Osteosarcoma
FAP has higher risk of what sarcoma?
desmoid tumor
lymphedema puts a patient at increased risk of what sarcoma?
Angiosarcoma
What is the single most important prognostic factor in osteosarcoma?
Grade
What is different about osteosarcoma in jaw/mandible compared to other places?
Jaw has a very favorable biology, behaving more like a low grade
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
Patient with osteosarcoma receives pre-operative MAP and has a good response seen at surgery. What to do in adjuvant setting?
Continue MAP
Patient with osteosarcoma treated with preoperative MAP has surgery and doesn’t have a good response. What to do in adjuvant setting?
Ifosfamide + MTX
Three treatment options for recurrent osteosarcoma
Ifosfamide
Gem + Docetaxel
Regorafenib
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
What is the classic genetic change in Ewing Sarcoma?
t(11;22) EWS;FLI1 gene
What is standard of care systemic treatment for Ewing sarcoma?
VAC/IE:
Vincristine, Doxo, Cyclophosphamide alternating with Ifos + Etop every 2 weeks
What is the general treatment paradigm for chondrosarcoma?
Not sensitive to systemic therapy
Surgical resection as much as possible
What chromosomal translocation is seen in alveolar rhabdomyosarcoma?
t(2;13)
What chromosomal translocation has been seen in dermatofibrosaroma protuberans? How does that affect therapy?
t(17;22)
Imatinib
What chromosomal translocation is seen in inflammatory myofibroblastic tumor? How does that affect treatment?
t(2;19)
Use ALK inhibitor
What is the most important prognostic factor in soft tissue sarcoma?
Depth of invasion
Who should get adjuvant RT in soft tissue sarcoma? (2)
Close margins
Tumors >5 cm
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
