[size=20px]A 12-year-old boy undergoes radical inguinal orchiectomy for a 1.5 cm right testicular mass and the pathology is consistent with rhabdomyosarcoma (RMS). Such patients with paratesticular RMS should undergo radiation therapy (RT) to involved retroperitoneal nodal beds. The next best step to determine whether RT is indicated in this patient is:

 

a PET-CT scan of the abdomen and pelvis

 

b Standard CT scan of the abdomen and pelvis

 

c Assessment of PAX3,7/FOXO fusion status

 

d Retroperitoneal lymph node sampling[/size]

D

D

D

D

Correct answer

D Retroperitoneal lymph node sampling

 

Regional nodal involvement is considered an unfavorable prognostic factor in rhabdomyosarcoma (RMS). Typically, lymph nodes that are >1 cm or FDG-PET avid are considered suspicious, but PET-CT (and standard CT) poorly predicts histologically positive nodes in RMS. For this reason, consensus guidelines suggest “regional nodes should undergo surgical evaluation in all patients with extremity and trunk tumors and paratesticular RMS patients older than age 10 years. In addition, surgical lymph node evaluation is strongly recommended to evaluate lymph nodes in all PAX/FOXO fusion positive tumors.” Based upon his age, this patient should undergo RPLND. Negative PAX 3,7/FOXO fusion status is a favorable prognostic factor but would not accurately predict a lack of retroperitoneal nodal involvement in this patient.