Soft tissue sarcomas

Question 1

True about soft tissue sarcoma

Answer 1

• There are more than 11 000 new diagnoses of soft tissue sarcoma annually in the US
• Most soft tissue sarcomas-specific deaths are due to uncontrolled sarcoma-specific deaths are due to uncontrolled pulmoanry metastasis
• The overall 5 year survival rate of akk stages of sift tissue sarcoma approximates 50 to 60%

Question 2

Asscoiated with the development of sarcomas

Answer 2

• Radiation exposure
  
  • p53
• herbicide exposure
  
  • phenoxyacetic acids and to wood preservatives containing chlorophenols
• Chronic lymphedema
  
  • after axilalry dissections

TRAUMA HAVE NO CAUSAL RELATIONSHIP ESTABLISHED

Question 3

Internationale Contre le Cancer (AJCC/UICC) sarcoma staging system?

Answer 3

• the 7th edition of AJCC is based on
  
  • histologic grade of aggresiveness
    
    • cellularity
    • differentiation
    • pleomorphism
    • necrosis
    • # of mitosis per high power field
  • tumor size
  • dept
  • presence of nodal or distant metastasis
• Histologic grade is the most importnat prognostic factor

Question 4

Known molecular pathogenic events in sarcoma

Answer 4

• Chromosomal translocation
• Oncogene amplification
• Complex hgenomic reaarrangements

Question 5

Recommendations for the management of soft tissue masses

Answer 5

• Soft tissue tumors that are enlarging or gretaer than 3cm should evaluated with radiologic imaging (UTZ or CT) and a tissue diagnosis should be made using core needle biopsy
• Once a sarcoma diagnosis is established, obtain (MRI) for extremity lesions nad CT for other anatomic locations and evaluate for metastatic disease with chest CT for intermediate or high grade (2 or 3) ir large (T2) tumors
• A wide local excision with 1 to 2 cm margin is adequate therapy for low grade lesions and T1 tumors
• Radiation therapy plays a critical role in the management of large (T2), intermediate or high grade tumors
• PAtients with locally advanced high-grade sarcomas or distant metastases should be evaluated for chemotherapy
• An aggressive surgical approach should be taken in the treatmetn of patients with an isoalted local recurrence or resectable distant metastasis

Question 6

True about desmoid tumors

Answer 6

• Local recurrence is observed up to 1/3 of patients regardless of microscopic margin of resection status
• Primary therapy - surgical resection with wide lcoal excision
• Radiation therapy may be effective in patinets with unresectable tumors or as adjuvant following surgery for recurrent disease
  
  • 50 to 54 Gy
• systemic treatment - tumor associated symptoms who have not responded to other interventions
  
  • methotrexate and vinblastine
  • single agent pegylated liposomal doxorubicin and sorafenib