[size=20px]A 8 year old boy presents with a solid 15 cm retroperitoneal extrarenal mass that appears to encase the right ureter and involve the inferior vena cava. Which of the following regarding retroperitoneal sarcomas is true?

 

A there is no role for resection and reconstruction of the ureter or major vascular structures

 

B liposarcoma is the most common histologic type

 

C involvement of adjacent organs at the time of presentation is rare

 

D initial complete resection carries a 50% recurrence rate

 

E exploration with an attempt at primary resection should be performed[/size]

E

B

B

A

B

D

E

A

Correct answer

D initial complete resection carries a 50% recurrence rate

 

Surgical management of retroperitoneal sarcoma continues to be a challenge. Because in this location tumors can grow to alarming size (mean 14 cm) before developing symptoms, 62% present with locoregional involvement. Rhabdomyosarcoma and fibrosarcoma are the two most common histologic types.

 

Performance of a complete resection remains an important prognostic factor. Despite a recurrence rate of 50% after initial complete resection, those who undergo complete resection do have a significantly better disease free survival rate. Children who are deemed unresectable due to involvement of adjacent vital structures on pretreatment imaging should undergo neoadjuvant chemotherapy and radiation therapy to improve resectability. Following neoadjavant therapy, ureteral and vascular reconstructions should be anticipated and undertaken rather than performing an incomplete resection.

D