it occurs predominantly in three regions : the head and neck , genitourinary tract and extremities . the majority of cases of rhabdomyosarcoma in the genitourinary tract occurs in the bladder and paratesticular organs . the etiology is uncertain , however , the origin is presumed to be an overgrowth of the sarcomatous component of teratoma . here , we report a case of a two - year - old child with primary pure intratesticular rhabdomyosarcoma . a two - year old child presented with a slow - growing painless right testicular mass since two months . complete blood count , serum alpha - fetoprotein ( 2.0 ng / ml ) , beta - human chorionic gonadotropin ( 5.0 iu / l ) and lactate dehydrogenase ( 120.0 scrotal and abdominal ultrasound examination showed a large hypoechoic intratesticular mass measuring 6.8  4.5  3.5 cm in the right scrotum . it was reported as high - grade spindle cell tumor ; possibility of sarcomatous elements of malignant germ cell tumor should be ruled out . grossly , the testis was enlarged and measured 6.4  4.0  3.0 cm . the tumor was completely contained within the tunica albuginea . the testis was almost completely replaced by tumor with a small portion of residual normal testis [ figure 1 ] . histopathological examination revealed a highly cellular tumor composed of pleomorphic cells including round to spindle , strap - like and racquet - shaped cells arranged in sheets . few large cells having abundant granular eosinophilic cytoplasm with cross - striations , eccentrically situated large vesicular nucleus and prominent nuclei was evident . numerous sections were studied in an attempt to find other elements of germ cell tumor , but none could be found . immunohistochemistry showed positive results for vimentin , desmin and myogenin [ figure 2d , e ] while negative for alpha - smooth muscle actin , placental alkaline phosphatase ( plap ) , cd-117 , s-100 , cytokeratin , and epithelial membrane antigen . small portion of residual normal testis was also seen primary intratesticular rhabdomyosarcoma : h and e stain : ( a ) and ( b ) highly cellular tumor composed of pleomorphic cells including round to spindle , strap - like cells . mitotic figures were seen ( 100 ) , ( c ) racquet - shaped rhabdomyoblast showed abundant granular eosinophilic cytoplasm , eccentrically situated large vesicular nucleus with prominent nucleoli ( 400 ) . immunohistochemistry showed desmin ( d ) and myogenin ( e ) positivity in tumor cells ( 100 ) orchidectomy was followed by chemotherapy . retroperitoneal lymph node dissection ( rplnd ) was not performed in our case as radiologically there was no lymphadenopathy . in spite of chemotherapy , patient developed multiple metastases to lung , pelvic and periaortic lymph nodes after 16 months and died after 20 months of diagnosis . paratesticular sarcomas are mostly rhabdomyosarcoma , seen in children . however , intratesticular sarcomas are very rare and mostly associated with a germ cell tumor . primary intratesticular rhabdomyosarcoma is very rare , with just 14 cases reported in the literature till date . other hypotheses include undifferentiated mesenchyme having the capacity for rhabdomyoblastic differentiation , or perhaps from embryonal muscle tissue that has been displaced during the early stages of tissue development . trauma , cryptorchidism , and exogenous maternal estrogen ( in utero ) have all been associated with its development . mean age of primary intratesticular sarcoma at presentation is 32 years ( range 386 years ) . in our case it presents as a slowly - growing , painless , firm to hard , testicular mass . rhabdomyosarcoma is regarded as a highly malignant tumor with frequent recurrence and dissemination via the bloodstream and lymphatics . metastasis of rhabdomyosarcoma is common to the lung , bone marrow , brain , liver , omentum and lymph nodes . primary intratesticular rhabdomyosarcoma should be differentiated from germ cell tumor with sarcomatous component , other intratesticular spindle cell sarcomas and paratesticular rhabdomyosarcoma . diagnosis of primary intratesticular rhabdomyosarcoma should be made only after meticulous examination of specimen to exclude any germ cell component as striped muscle elements are frequent findings in testicular teratoma . the accurate diagnosis of the sarcomatous component in germ cell tumor is very important because of the implications for therapy . leiomyosarcoma shows strong immune reactivity for muscle - specific actin , smooth muscle actin , vimentin , calponin and alpha - l - chymotrypsin and negative reaction for desmin , cytokeratin , s-100 protein and leu 7 . fibrosarcoma shows positive result for vimentin , type i collagen and negative result for smooth muscle marker and histiocytic markers . a diagnosis of primary intratesticular sarcoma should only be made after exclusion of the more commonly seen paratesticular sarcomas . scrotal ultrasound and abdominal computed tomography imaging were used to exclude paratesticular and retroperitoneal pathology . the tumor epicenter was found within the testis and no paratesticular pathology was seen in our case . gross examination of the resected specimen showed tumor mass arising from the testis primarily , and tumor was not adherent to the scrotal wall or adjacent tissues . however , to determine the specific site of origin of paratesticular and testicular sarcoma is not always possible because these structures are in continuity with each other . the intergroup rhabdomyosarcoma study ( irs ) group management guidelines for the pediatric population have resulted in reducing morbidity and increasing survival from 2570% over 20 years since 1970 . post - orchidectomy rplnd used to be carried out as a routine staging procedure , more commonly for a therapeutic purpose . patients with unresectable tumors who undergo treatment with chemotherapy should be considered for surgery after downgrading . radiotherapy is recommended more commonly to control local recurrence , metastasis or for unfavorable histology , such as alveolar rhabdomyosarcoma . recurrence may occur within two years so intensive surveillance and follow - up is necessary . primary intratesticular sarcoma that is neither associated with germ cell ele - ments nor paratesticular elements is a unique subset of intrascrotal sarcoma . germ cell tumor component and paratesticular sarcoma must be excluded carefully before establishing the diagnosis . radical orchidectomy with negative surgical margins followed by rplnd and chemotherapy is the recommended treatment .
testicular sarcomas constitute only 12% of all testicular tumors and are mostly associated with germ cell tumor . 
 primary intratesticular rhabdomyosarcoma is rare and only 14 cases have been reported in the literature till date . 
 it should be differentiated from germ cell tumor with sarcomatous component , other intratesticular spindle - cell sarcomas and paratesticular rhabdomyosarcoma . 
 accurate diagnosis and early treatment is essential as it is an aggressive tumor with high metastatic potential and poor prognosis . 
 orchidectomy is the treatment of choice . 
 chemo - radiotherapy is recommended in case of recurrence and metastasis .