a 69-year - old male complained of a 4 month history of a progressively enlarging , painless right groin swelling during routine urological review . he had been diagnosed with locally advanced prostate cancer 2 years previously , for which he had received radical radiotherapy ( 74gy/37 fractions ) and was on maintenance androgen deprivation therapy . contrast computed tomography of abdomen and pelvis showed a non - obstructed right inguinal hernia containing a contrast filled loop of small bowel ( figure 1 ) . intra - operatively a solid mass involving the spermatic cord and extending into the proximal scrotum was seen . pathology revealed a right paratesticular sarcoma , of the leiomyosarcoma type involving the spermatic cord ( figure 2 ) . figure 1transverse section of a contrast enhanced computed tomographic scan of the abdomen and pelvis showing a right inguinoscrotal mass . 
  transverse section of a contrast enhanced computed tomographic scan of the abdomen and pelvis showing a right inguinoscrotal mass . figure 2histologically the tumor appears as malignant smooth muscle spindle cells with typical cigar - shaped nuclei , which stain to alpha actin , desmin , and vimentin antibodies . histologically the tumor appears as malignant smooth muscle spindle cells with typical cigar - shaped nuclei , which stain to alpha actin , desmin , and vimentin antibodies . soft - tissue sarcomas of the genitourinary tract are uncommon , accounting for only 12% of urological malignancies , and 2.1% of soft - tissue sarcomas in general . more specifically para - testicular sarcoma ; sarcomas of the spermatic cord are extremely rare . leiomyo - sarcoma is the commonest type of paratesticular sarcoma . only around 110 cases of leiomyosarcoma of the spermatic cord histologically , the most common spermatic cord type arises from undifferentiated mesenchymal cells of the cremasteric muscle and vas deferens . the less frequent epididymal form arises from the smooth muscle surrounding the basement membrane of the epididymal canal . leiomyosarcoma may be divided anatomically into deep soft - tissue , cutaneous - subcutaneous , and vascular . the american joint committee on cancer ( ajcc ) classifies spermatic cord leiomyosarcoma as deep tissue . nodal spread can involve external iliac , hypogastric , common iliac and para - aortic nodes . the vas deferens can act as a conduit for local spread , which may involve the scrotum , inguinal canal or pelvis . clinical presentation is typically with a painless , firm , progressively enlarging , intra - scrotal mass which is extra - testicular . the differential diagnosis includes inguinoscrotal hernia , hydrocoele , testicular malignancy and other rarer tumors including benign leiomyoma , fibrous mesothelioma , various benign fibrous tumors and pseudotumors , and fibromatosis . the exact degree to which surrounding soft tissues should be resected and the optimal margin measurements remain controversial . in 27% of cases , which were completely excised , nodal involvement appears to be more commonly associated with paratesticular sarcoma than other soft tissue sarcomas . in two separate studies catton and colleagues have shown nodal failure rates varying between 14% ( 2 of 14 patients ) and 28% ( 6 of 21 patients ) . however , the literature has reported rates of up to 29% . due to this high incidence of nodal recurrence , both prophylactic retroperitoneal lymph node dissection and adjunctive nodal radiotherapy have been used . to date there are no reports of an improvement in survival with adjunctive nodal dissection . local recurrence rates of 27% have been reported with wide excision alone , although the study population involved was small . the median follow - up for each was 4.2 years , with the exception of the study by fagundes et al . it is of note that , in this study , median follow - up for non - irradiated and irradiated groups differed , being 123 and 63 months respectively . this may have lead to an artificially increased rate of recurrence in the non - irradiated group . local recurrence has been described as the achilles heel of sarcoma management , and spermatic cord sarcomas are no exception . sarcomas of all grades have a tendency to infiltrate local tissues , leading to morbidity from local disease progression , and a higher rate of metastasis and disease - specific mortality . in one study , which looked at 102 patients with primary disease of the genitourinary tract alone , 33 developed local recurrence ( median time to local recurrence being 10.6 years ) with 20 sarcoma - specific deaths . the risk associated with sarcomas can be reduced with adjunctive radiotherapy , or adjunctive surgery as required . with the exception of the dotan et al . 's study , previous attempts at examining urological sarcomas with regards to optimal management have been hindered by small cohort numbers , and the rarity of the condition . however , prolonged disease - specific survival has consistently been shown to be associated with tumor histology , size ( < 5 cm ) , grade , achieving complete resection , paratesticular or bladder tumor site and lack of metastases at presentation . the largest study of genitourinary sarcomas to date looked at 131 patients ; 57 having a para - testicular sarcoma . 51% of the paratesticular patients were disease - free at the median follow - up time of 4.2 years , with 3% still showing evidence of disease , and 39% having died of the disease in the interim . adjunctive radio- and chemotherapy were not consistently used in this retrospective study population , and therefore their effects were not considered . this is unfortunate , as it remains to be conclusively proven that adjuvant radiation or chemotherapy is effective in para - testicular sarcomas specifically . the standard management of wide excision with adjunctive radio - therapy is based on the success of adjuvant radiotherapy in the management of sarcomas in general . evidence for the routine use of adjuvant chemotherapy for adult high grade sarcoma is controversial . in 1997 the sarcoma meta - analysis collaboration showed significant relapse free survival rates ( median follow - up 9.4 years ) with adjuvant doxorubicin in soft - tissue sarcomas ( n=1568 ) . randomized control trial to date failed to show an improvement with chemotherapy ( n=351 ) . phase i and ii trials showed promising preliminary results with objective response rates between 3.7% and 17.1% . as the majority of studies into paratesticular sarcomas are retrospective by necessity , the inconsistency of management of the patients involved makes a comparison of therapies difficult .