273 - Sarcomas

Question 1

Which soft-tissue sarcoma is most prevalent?

Answer 1

Leiomyosarcoma

Question 2

Radiation has a role in the treatment of which sarcoma?

Answer 2

Ewing sarcoma

Question 3

What is the primary treatment for GI stromal tumors?

Answer 3

Surgery

Imatinib = adjuvant (if c-KIT mutation)

Imatinib is first line for metastatic treatment

Question 4

Where in the bone do osteosarcomas arise?

Answer 4

Metaphysis

Question 5

What is the treatment for osteosarcoma?

Answer 5

Surger

+ neoadjuvant +/- adjuvant chemo (doxorubicin + cisplatin)

Question 6

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

Answer 6

>30% shrinkage of the tumor

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

Combination therapy may increase response rate

Question 7

Where in the bone do Ewing sarcomas arise?

Answer 7

Shaft of the bone

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

Question 8

What is the primary treatment for a localized sarcoma?

Answer 8

Surgery

+ Adjuvant radiation for all soft tisue >5cm

Question 9

What is the most appropriate treament for widely metatstic, high-grade sarcoma?

Answer 9

Chemotherapy

Adriamycin singlet or doublet

Question 10

Which sarcoma is most sensitive to chemotherapy?

Which agent?

Answer 10

Synovial sarcoma

Ifosfamide

Question 11

What is the criteria for diagnosing Li Fraumeni syndroem?

Answer 11

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

Question 12

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

Answer 12

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

Question 13

Chemotherapy has no role in which bone sarcoma?

Answer 13

Chondrosarcoma

Question 14

What is the most common mutation in GI stromal tumors?

Answer 14

KIT mutation

GOF; results in abnormal, constituitively active tyrosine kinase activity

Question 15

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

Answer 15

Antrhacycline-based

(doxorubicin)

Question 16

What subtype of liposarcoma is most sensitive to chemotherapy?

Answer 16

High-grade myxoma

Doxorubicin, ifosfamide

Question 17

What complications are common in patients treated for sarcoma?

Answer 17

• Cardiopyopathy
  
  • 2/2 doxorubicin + ifosfamide
• Renal tubular and/or glomerular dysfunction
  
  • 2/2 doxorubicin + ifosfamide
• Infertility
  
  • 2/2 alkylating agents
  • Affects post-pubescent males more than females
• Secondary malignancy

Question 18

What is the most common primary malignant tumor of bone?

Answer 18

Osteosarcoma

Question 19

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

Answer 19

Treat all with surgery

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

Question 20

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

Answer 20

• Osteosarcoma
  
  • Metaphysis
  • Lytic and blastic change
• Ewing sarcoma
  
  • Diaphysis
  • Infiltrative pattern

Question 21

What translocation is associated wtih synovial sarcoma?

Answer 21

t(x;18)

Very sensitive to chemotherapy!! (ifosfamide)

Question 22

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

Answer 22

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

Question 23

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

Answer 23

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

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