[size=20px]A 12-year old boy is referred to you with a suspected inguinal hernia. Instead, on palpation of the scrotum you suspect a paratesticular mass. There are no enlarged lymph nodes palpable in the inguinal canal or on computerized tomography imaging of the abdomen and pelvis. For this patient with a paratesticular mass you recommend

 

a transscrotal needle biopsy only.

 

b transscrotal needle biopsy and sentinel lymph node sampling of inguinal nodes.

 

c transscrotal open biopsy and sentinel lymph node sampling of inguinal nodes.

 

d inguinal open biopsy and sentinel lymph node sampling of inguinal nodes.

 

e inguinal open biopsy and ipsilateral retroperitoneal lymph node dissection.[/size]

E

D

E

D

D

B

D

D

D

E

E

a

D

E

D

Testicular would disrupt lymphatic channels and increase recurrence rate

There is no role for retro peritoneal lymph node dissection prior to chemo

Correct answer

E inguinal open biopsy and ipsilateral retroperitoneal lymph node dissection.

 

Paratesticular rhabdomyosarcomas are tumors arising in the spermatic cord, paratesticular appendages, paratesticular tunica or epididymis. Transscrotal biopsy is never recommended because of the risk of tumor contamination of the scrotal soft tissue. Biopsy should be done through an inguinal approach with control of the testicular cord. In patients with rhabdomyosarcoma, determination of lymph node status is an important part of pretreatment staging. If the frozen section of the biopsy demonstrates rhabdomyosarcoma, an orchiectomy and resection of the cord are performed.

 

In boys with a paratesticular tumor

 

- clinically enlarged inguinal nodes should be removed for pathologic confirmation at any age

 

- radiographically enlarged retroperitoneal nodes should undergo ipsilateral retroperitoneal lymph node dissection

 

- if the patient is older than 10 years, staging ipsilateral retroperitoneal lymph node dissection (SIRPLND) is required regardless of radiologic findings. The risks of radiation in younger patients and the sensitivity to chemotherapy have led to limitation of SIRPLND for occult retroperitoneal nodes to older children.

 

The precise technique for SIRPLND is detailed in the Cancer Handbook on the APSA website under Rhabdomyosarcoma.

 

Ipsilateral Staging Retroperitoneal Node Dissection - Recommended technique

 

A. Open ipsilateral node dissection

 

A nerve-sparing ipsilateral template modification to the standard bilateral RPLND is recommended. The approach can be transperitoneal, lateral extraperitoneal (i.e. used in renal transplant or spine fusion exposure) or laparoscopic. Figures 1 and 2 adapted from Ritchie are the templates for the dissection. In either right or left sided lesions the dissection boundaries above the inferior mesenteric artery are similar, but contralateral dissection below the IMA is avoided to assure preservation of the sympathetic fibers at L2 - 4 which are the most important fibers for ejaculatory function.

• Right Sided Lesions: The dissection for right sided lesions begins at the level of both renal veins encompassing the aorta and cava and intra-aortocaval tissue across to the left gonadal vein / ureteral junction down to the inferior mesenteric artery and then down the right side to the level of the right common iliac artery where it is crossed by the ureter. The ipsilateral spermatic vessels are removed to the deep inguinal ring where the previously ligated stump of the cord is removed.
  
• Left Sided Lesions: A similar dissection for left sided lesions is carried out with the exception of the right lateral margin, which is the vena cava rather than the right ureter. (This is due to a different pattern of nodal spread) The infra-IMA dissection is the mirror image of that described for right sided lesions. The ipsilateral spermatic vessels are removed to the deep inguinal ring where the previously ligated stump of the cord is removed.
  

The specimen should be marked so that the highest node(s) is identified

 

B. Laparoscopic node dissection

 

Ipsilateral retroperitoneal node dissection can be carried out effectively using laparoscopic techniques by experienced surgeons. If, in the opinion of the surgeon, surgical goals will be compromised by the laparoscopic approach for any reason after the start of a procedure, then conversion to an open node dissection is mandatory