271 - Sarcomas Flashcards
Radiation has a role in the treatment of which sarcoma?
Ewing sarcoma
- arises in bones of children and soft tissues of adults
- always considered high grade
- affects shaft of the bone in “onion skinning” pattern
Gold standard = multimodal therapy with surgery, chemotherapy, and radiation
What is the primary treatment for GI stromal tumors (GIST)?
Surgery
Imatinib = adjuvant (if it has KIT mutation)
Imatinib is first line for metastatic treatment
Where in the bone do osteosarcomas arise?
- Metaphysis
- characterized by lytic and blastic features
What is the treatment for osteosarcoma?
Neoadjuvant and/or adjuvant chemotherapy + surgery is standard of care
adjuvant chemo = doxorubicin + cisplatin
What counts as a “response to chemotherapy” in treating metastatic carcinoma?
>30% shrinkage of the tumor
~1/5 of patients achieve response on doxorubicin or ifosfamide
Combination therapy may increase response rate
Where in the bone do Ewing sarcomas arise?
Shaft (diaphysis) of the bone
Always high grade; treat aggressivley with surgery, chemo, radiation
What is the primary treatment for a localized sarcoma?
Surgery
+ Adjuvant radiation for all soft tisue >5cm
What is the most appropriate treatment for widely metatastic, high-grade sarcoma?
Chemotherapy
Adriamycin singlet or doublet
Which sarcoma is most sensitive to chemotherapy?
Which agent?
Synovial sarcoma;
is sensitive to Ifosfamide
What is the criteria for diagnosing Li Fraumeni syndrome?
Need all of the following:
- Pt dx with sarcoma at a young age (<45)
- 1st degree relatie dx with any cancer at a young age (<45)
- Another 1st degree or second decree relative dx with any cancer at a young age (<45) or a sarcoma at any age
Li Fraumeni: autosomal dominant mutation of the p53 tumor suppressor gene
List 4 genetic syndromes (and their mutation) associated with sarcoma
- Li Fraumeni - p53
- Retinoblastoma - RB
- NFM-1 - NF-1
- Gardner - APC
Chemotherapy has no role in which bone sarcoma?
Chondrosarcoma
What is the most common mutation in GI stromal tumors?
KIT mutation
Gain of function –> results in abnormal, constituitively active tyrosine kinase activity
What kind of chemotherapy should be used for metatstatic soft tissue sarcomas?
Anthracycline-based
(doxorubicin)
What subtype of liposarcoma is most sensitive to chemotherapy?
High-grade myxoma
Doxorubicin, ifosfamide
