alveolar soft part sarcoma is a rare type of soft tissue sarcoma affecting female more than male and clinically mimics hemangioma or arterio - venous malformation . alveolar soft - part sarcoma ( asps ) is an extremely rare disease arising from connective tissues . it occurs commonly in the lower extremities ( 44% ) and the head and neck ( 27% ) , of which 25% occur in the tongue . although the origin of asps is still unknown , recent cytogenetic studies revealed some chromosomal translocation , which may have a role . the tumor runs an indolent course , but may recur and metastasize , leading to high mortality . a 32-years - old female from a remote village presented with multiple painless soft globular swellings all over the body including face , tongue , neck , chest , abdomen , back , genitalia , and all the limbs for a duration of 3 months [ figures 1 and 2 ] . most of the lesions were skin - colored , but few of the lesions over forehead , neck , back , and genitalia showed reddish to bluish discoloration while few showed crusting over the surface . the patient gave history of similar type of lesion in right thigh 3 years back , for which she underwent surgical excision , but no histopathological examination , was done at that time . there was associated history of anorexia , weight loss , and fatigue . on examination , apart from the aforesaid lesions , a nodule was palpable in the posterior fornix of vagina and another in the anterior rectal wall 6 cm from anal verge . after thorough investigations , all the blood parameters were found to be normal , except moderate degree of anemia . fortunately , her chest x - ray , usg of whole abdomen , ct scan of brain revealed no abnormality . the most symptomatic swelling which were present over right thigh ( at the site of previous excision ) and back were excised followed by biopsy . histopathology showed large polygonal cells with eccentrically - placed prominent nucleus and abundant eosinophilic cytoplasm arranged in an alveolar pattern separated by fibrous septae and occasional mitotic figures [ figure 3 ] . following surgery , the patient was referred to radiotherapy department and was put on chemotherapy regimen comprising of vincristine ( 2 mg ) , adriamycin ( 60 mg ) , and cyclophosphamide ( 90 mg ) , given at an interval of 3 weeks . there is mild reduction of the lesions with moderate symptomatic improvement and without any features of metastasis . multiple globular swellings over the back of trunk similar swellings over face and neck characterisistic histopathology of asps the tumor occurs most commonly in the soft tissues of the extremities , rarely in arm , chest , and retro - peritoneal tissues , even stomach and female genital tract has been reported . the tumor occurs predominantly in females , with a male : female ratio of 1:2 . the most probable genetic theory of origin is chromosomal re - arrangements at t ( x;17)(p11;q25 ) , resulting in the aspl - tfe3 fusion gene . since women have an extra x - chromosome , they are likely to develop an x autosomal translocation , which is theoretically doubles that of men . histo - pathologically , asps needs to be differentiated from paraganglioma , rhabdomyosarcoma , metastatic adrenal carcinoma and melanoma by demonstrating cytological uniformity , lack of nuclear atypia , and paucity of mitotic figures in asps . the outcomes depend on prognostic factors such as a patient 's age , tumor size , recurrence ( which has been reported to range from 11 - 50% ) , and the presence of metastasis at the time of diagnosis . it usually metastasizes late to the lungs , bones , lymph nodes , and brain . accurate diagnosis and treatment of this unusual tumor requires clinical suspicion and clinico - pathological correlation with appropriate radiological studies . as treatment options are limited when lesions are multiple , surgical resection of the symptomatic lesions followed by chemotherapy was given in our patient . in spite of extensive involvement , this female with multiple lesions involving unusual sites but without any features of metastasis is uncommon of asps . however , a classical histopathological feature from the biopsy of the lesions favors the diagnosis . because of the rarity of occurrence of the tumor with uncommon presentation , the case has been reported . 1 . occurrence of multiple lesions in asps , astonishingly without any metastasis in the present case . when lesions are multiple , surgical excision of the symptomatic lesions followed by chemotherapy is a good treatment option .
alveolar soft - part sarcoma ( asps ) is an extremely rare disease arising from connective tissues with a propensity for recurrence and metastasis . 
 clinically , it can be confused with hemangioma or arterio - venous malformations . 
 thus , a high index of suspicion and histopathological examination are required to make a definitive diagnosis . 
 we report a case of recurrent asps in a young female with multiple sites involvement without any features of metastasis who has been treated with excision of the symptomatic lesions followed by chemotherapy .