spermatocytic seminoma ( ss ) is an uncommon testicular germ cell neoplasm with distinct histopathological features . it usually occurs in older men with mean age of 53.6 years ( range 19 - 72 years ) . it bears an excellent prognosis but tends to behave aggressively and renders a poor prognosis if associated with sarcomatous transformation . patients with sarcomatous transformation at the time of presentation already have metastatic disease and this is almost fatal . the sarcoma component may present as rhabdomyosarcoma , angiosarcoma , leiomyosarcoma , myxoid liposarcoma or undifferentiated sarcoma . we describe a case of a young man with ss developing rhabdomyosarcomatous transformation . a 38-year - old man presented with long standing history of swelling in right hemiscrotum . he underwent right testicular biopsy in a local medical college , which was diagnosed as seminoma . there was a right testicular mass measuring 10  10 cm , with scrotal violation and left testis was normal . the serum tumor markers i.e. , alfa - fetoprotein ( afp ) and beta hcg were within normal . retroperitoneal lymph node dissection ( rplnd ) was not performed as radiologically there was no lymphadenopathy . in addition to local and abdominal radiotherapy , chemotherapy ( with vincristine , actinomycin d and cyclophosphamide ) was given . two months postoperatively , the patient is on follow - up and doing well . the right high inguinal orchidectomy specimen measured 7  6  4 cm with attached vas deferens and scrotal skin . most of the nodules were gelatinous with some appearing darker in color and firm in consistency than the rest [ figure 1a ] . microscopically , the spermatocytic component consisted of polymorphic cell population with large cells having characteristic  spermine chromatin ; intermediate - sized cells and small lymphocyte - like cells [ figure 1b ] . the cells were immunopositive with c - kit ( cd117 ) [ figure 1d ] but were negative with plap ( placental - like alkaline phosphatase).the other component of the tumor consisted of fascicles of spindle shaped cells with fusiform nuclei [ figures 2a and b ] . admixed with both the components were many multinucleated and bizarre tumor giant cells with hyperchromatic nuclei . few strap cells and globoid cells were identified within the fascicles of spindle cells [ figure 2c ] . no areas of intratubular germ cell neoplasia ( itgcn ) or other associated germ cell components were identified . immunohistochemically , the spermatcocytic component was negative for placental alkaline phosphatase ( plap ) , cd30 , smooth muscle - actin , and the spindle cells were strongly positive for desmin [ figure 2d ] and weakly for myoglobin . ( a ) lobulated , soft and homegenous cut surface of the tumor with few nodules appearing form and dark in color ( arrow ) . ( b ) spermatocytic seminoma with three types of cells ( small , intermediate and large ) with fine fibrovascular septa traversing between them ( h&e 400 ) . ( d ) spermatocytic seminoma cells show cytoplasmic positivity with c - kit ( immunoperoxidase 400 ) ( a ) long fascicles of spindle cells ( h&e 200 ) . ( b ) sarcomatous component in close apposition with the seminomatous component ( hxe 200 ) . a 38-year - old man presented with long standing history of swelling in right hemiscrotum . he underwent right testicular biopsy in a local medical college , which was diagnosed as seminoma . there was a right testicular mass measuring 10  10 cm , with scrotal violation and left testis was normal . the serum tumor markers i.e. , alfa - fetoprotein ( afp ) and beta hcg were within normal . retroperitoneal lymph node dissection ( rplnd ) was not performed as radiologically there was no lymphadenopathy . in addition to local and abdominal radiotherapy , chemotherapy ( with vincristine , actinomycin d and cyclophosphamide ) was given . two months postoperatively , the patient is on follow - up and doing well . the right high inguinal orchidectomy specimen measured 7  6  4 cm with attached vas deferens and scrotal skin most of the nodules were gelatinous with some appearing darker in color and firm in consistency than the rest [ figure 1a ] . microscopically , the spermatocytic component consisted of polymorphic cell population with large cells having characteristic  spermine chromatin ; intermediate - sized cells and small lymphocyte - like cells [ figure 1b ] . the cells were immunopositive with c - kit ( cd117 ) [ figure 1d ] but were negative with plap ( placental - like alkaline phosphatase).the other component of the tumor consisted of fascicles of spindle shaped cells with fusiform nuclei [ figures 2a and b ] . admixed with both the components were many multinucleated and bizarre tumor giant cells with hyperchromatic nuclei . few strap cells and globoid cells were identified within the fascicles of spindle cells [ figure 2c ] . no areas of intratubular germ cell neoplasia ( itgcn ) or other associated germ cell components were identified . immunohistochemically , the spermatcocytic component was negative for placental alkaline phosphatase ( plap ) , cd30 , smooth muscle - actin , and the spindle cells were strongly positive for desmin [ figure 2d ] and weakly for myoglobin . ( a ) lobulated , soft and homegenous cut surface of the tumor with few nodules appearing form and dark in color ( arrow ) . ( b ) spermatocytic seminoma with three types of cells ( small , intermediate and large ) with fine fibrovascular septa traversing between them ( h&e 400 ) . ( d ) spermatocytic seminoma cells show cytoplasmic positivity with c - kit ( immunoperoxidase 400 ) ( a ) long fascicles of spindle cells ( h&e 200 ) . ( b ) sarcomatous component in close apposition with the seminomatous component ( hxe 200 ) . a 38-year - old man presented with long standing history of swelling in right hemiscrotum . he underwent right testicular biopsy in a local medical college , which was diagnosed as seminoma . there was a right testicular mass measuring 10  10 cm , with scrotal violation and left testis was normal . the serum tumor markers i.e. , alfa - fetoprotein ( afp ) and beta hcg were within normal . retroperitoneal lymph node dissection ( rplnd ) was not performed as radiologically there was no lymphadenopathy . in addition to local and abdominal radiotherapy , chemotherapy ( with vincristine , actinomycin d and cyclophosphamide ) was given . the right high inguinal orchidectomy specimen measured 7  6  4 cm with attached vas deferens and scrotal skin . the testicular shape was maintained . on cut surface , most of the nodules were gelatinous with some appearing darker in color and firm in consistency than the rest [ figure 1a ] . microscopically , the spermatocytic component consisted of polymorphic cell population with large cells having characteristic  spermine chromatin ; intermediate - sized cells and small lymphocyte - like cells [ figure 1b ] . the cells were immunopositive with c - kit ( cd117 ) [ figure 1d ] but were negative with plap ( placental - like alkaline phosphatase).the other component of the tumor consisted of fascicles of spindle shaped cells with fusiform nuclei [ figures 2a and b ] . admixed with both the components were many multinucleated and bizarre tumor giant cells with hyperchromatic nuclei . few strap cells and globoid cells were identified within the fascicles of spindle cells [ figure 2c ] . no areas of intratubular germ cell neoplasia ( itgcn ) or other associated germ cell components were identified . immunohistochemically , the spermatcocytic component was negative for placental alkaline phosphatase ( plap ) , cd30 , smooth muscle - actin , and the spindle cells were strongly positive for desmin [ figure 2d ] and weakly for myoglobin . ( a ) lobulated , soft and homegenous cut surface of the tumor with few nodules appearing form and dark in color ( arrow ) . ( b ) spermatocytic seminoma with three types of cells ( small , intermediate and large ) with fine fibrovascular septa traversing between them ( h&e 400 ) . ( d ) spermatocytic seminoma cells show cytoplasmic positivity with c - kit ( immunoperoxidase 400 ) ( a ) long fascicles of spindle cells ( h&e 200 ) . ( b ) sarcomatous component in close apposition with the seminomatous component ( hxe 200 ) . ss is a relatively rare , well - defined pathological entity first described by masson comprising 3 - 7% of all seminomas . it is an indolent neoplasm with long duration of symptoms , early stage at presentation , absence of metastasis and bears an excellent prognosis . however , dedifferentiation or transformation to a sarcoma which occurs in approximately 6% of cases renders a poor prognosis with a metastatic potential . literature review cites small series and a few case reports[810 ] of occurrence of sarcomatous differentiation in ss . the occurrence of rhabdomyosarcomatous differentiation is rare and has been described in the literature in eight previous case reports [ table 1 ] . in the present case , the sarcomatous transformation occurred at a much early age of 38 years compared to the previous cases . the sudden increase in size however , it is important to be aware of misinterpreting the focal areas of atypical stromal overgrowth within conventional germ cell tumor . true sarcomatous component can be distinguished by its expansile growth pattern , infiltration into the surrounding elements and characteristic morphology of the distinct sarcomatous components . review of reported cases of spermatocytic seminoma with rhabdomyosarcomatous differentiation the seminomatous component was well circumscribed but the rhabdomyosarcomatous component was seen infiltrating and destroying the rete testis and epididymis . identification of epithelial structures of rete testis is important as these may be mistaken for epithelial component of a mixed nonseminomatous germ cell tumor . the origin of the sarcomatous elements of these tumors and the relation to the spermatocytic component is uncertain . few have implied this sarcoma component to be a teratomatous element of a mixed germ cell tumor as the occasional occurrence of sarcomatous elements ( chiefly rhabdomyosarcoma , angiosarcoma , and leiomyosarcoma ) in gonadal and extragonadal germ cell tumors is well known . the serum tumor markers were not elevated in the present case . however , other authors view the sarcomatous component as an the development of this tumor , first with slow enlargement of the testis followed by rapid increase in size tends to favor the explanation of sarcomatous differentiation occurring in ss . the treatment of choice in ss is orchidectomy , followed by surveillance to detect tumor in the contralateral testis . however , in the presence of sarcomatous differentiation , adjuvant chemotherapy or radiotherapy is beneficial . as the risk of metastases if remarkably increased with this transformation , a close follow - up is essential ; however , prognosis is dismal despite aggressive multimodality approach . in conclusion , this case describes the rare sarcomatous differentiation occurring in ss heralded by a rapid increase in size , which portends a poor prognosis to this indolent neoplasm .