271 - Sarcomas

Question 1

Radiation has a role in the treatment of which sarcoma?

Answer 1

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

Question 2

What is the primary treatment for GI stromal tumors (GIST)?

Answer 2

Surgery

Imatinib = adjuvant (if it has KIT mutation)

Imatinib is first line for metastatic treatment

Question 3

Where in the bone do osteosarcomas arise?

Answer 3

• Metaphysis
• characterized by lytic and blastic features

Question 4

What is the treatment for osteosarcoma?

Answer 4

Neoadjuvant and/or adjuvant chemotherapy + surgery is standard of care

adjuvant chemo = doxorubicin + cisplatin

Question 5

What counts as a “response to chemotherapy” in treating metastatic carcinoma?

Answer 5

>30% shrinkage of the tumor

~1/5 of patients achieve response on doxorubicin or ifosfamide

Combination therapy may increase response rate

Question 6

Where in the bone do Ewing sarcomas arise?

Answer 6

Shaft (diaphysis) of the bone

Always high grade; treat aggressivley with surgery, chemo, radiation

Question 7

What is the primary treatment for a localized sarcoma?

Answer 7

Surgery

+ Adjuvant radiation for all soft tisue >5cm

Question 8

What is the most appropriate treatment for widely metatastic, high-grade sarcoma?

Answer 8

Chemotherapy

Adriamycin singlet or doublet

Question 9

Which sarcoma is most sensitive to chemotherapy?

Which agent?

Answer 9

Synovial sarcoma;

is sensitive to Ifosfamide

Question 10

What is the criteria for diagnosing Li Fraumeni syndrome?

Answer 10

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

Question 11

List 4 genetic syndromes (and their mutation) associated with sarcoma

Answer 11

• Li Fraumeni - p53
• Retinoblastoma - RB
• NFM-1 - NF-1
• Gardner - APC

Question 12

Chemotherapy has no role in which bone sarcoma?

Answer 12

Chondrosarcoma

Question 13

What is the most common mutation in GI stromal tumors?

Answer 13

KIT mutation

Gain of function –> results in abnormal, constituitively active tyrosine kinase activity

Question 14

What kind of chemotherapy should be used for metatstatic soft tissue sarcomas?

Answer 14

Anthracycline-based

(doxorubicin)

Question 15

What subtype of liposarcoma is most sensitive to chemotherapy?

Answer 15

High-grade myxoma

Doxorubicin, ifosfamide

Question 16

What complications are common in patients treated for sarcoma due to survivorship?

Answer 16

• Cardiomyopathy
• Renal tubular and/or glomerular dysfunction
• Infertility (affects post pubescent males more than females)
• Secondary malignancy

Question 17

What is the most common primary malignant tumor of bone?

Answer 17

Osteosarcoma

Question 18

Summarize the differences in treatment for osteosarcoma, chondrosarcoma and Ewing sarcoma

Answer 18

Treat all with surgery

• Ewing
  
  • Add chemotherapy and radiation
• Osteosarcoma
  
  • Add chemotherapy (adjuvant or neoadjuvant)
• Chondrosarcoma
  
  • Surgery alone

Question 19

What are the differences in growth pattern between osteosarcoma and Ewing sarcoma?

Answer 19

• Osteosarcoma
  
  • occurs at the metaphysis (neck of long bone)
  • Lytic and blastic change
• Ewing sarcoma
  
  • occurs at the diaphysis (shaft of long bone)
  • Infiltrative pattern

Question 20

What translocation is associated wtih synovial sarcoma?

Answer 20

t(x;18)

Very sensitive to chemotherapy!! (ifosfamide)

Question 21

What is the role of chemotherapy in the treatment of sarcoma?

Answer 21

• Not indicated for localized sarcoma
• May shrink (but not eliminate) microscopic, metastatic disease
  
  • Use anthracyclines and/or alkylating agents

Question 22

List 4 characteristics of a mass that would raise your suspicion for sarcoma

Answer 22

• Increasing in size
• >5 cm
• Deep to the deep fascia
• Painful

Refer to multi-disciplinary center if any are present, even if not painful