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Paper text:
we came across 3 cases of sarcomas , other than leiomyosarcomas , reported to arise from the broad ligament . to the best of our knowledge ,
no case of an embryonal rhabdomyosarcoma ( erms ) of the broad ligament has been reported in literature .
we report about a 9-month - old female child with an erms of the broad ligament .
the case we present here is a 9-month - old female child , presented to us with the complaints of straining at micturition and defecation since the age of 5 months .
the patient was diagnosed with benign partial epilepsy of infancy and was under treatment for the same .
no other comorbidities were present . on examination , the patient had multiple caf au lait spots over her body ( more than 5 in number ) .
however , there were no other features to suggest neurofibromatosis , like , family history , lisch nodules , axillary or inguinal freckling and osseous lesions .
abdominal examination revealed an abdominopelvic mass , in the midline , extending superiorly to just above the umbilicus .
a contrast enhanced computed tomography ( cect ) scan of the abdomen and pelvis confirmed the clinical findings of an abdominopelvic mass of about 9.8 cm  6 cm  5 cm extending from the level of acetabulum in the pelvis , to the level of l3 vertebra [ figure 1 ] .
the mass was isodense to hypodense on a plain scan with heterogenous enhancement on post - contrast scan .
the urinary bladder was pushed to the right side due to mass effect but planes were maintained .
the uterus and adnexa were not visualized separately . a percutaneous biopsy done from the mass revealed erms . the metastatic work - up , including a bone scan , cect thorax and bone marrow examination , was normal .
contrast enhanced computed tomography scan showing the heterogenously enhancing mass arising from pelvis , with contrast in urinary bladder ( a and b ) .
intra - operatively ( c ) , the mass seen arising from the broad ligament , the uterus ( a ) and urinary bladder ( b ) separated the patient received neoadjuvant chemotherapy ( nact ) , as per intergroup rhabdomyosarcoma study group ( irs ) iv protocol ( vincristine , actinomycin and cyclophosphamide ) .
an exploratory laparotomy performed revealed a lobulated mass of 10 cm  5 cm  5 cm [ figure 1 ] .
the mass had a pedicle and was seen to be arising from the left broad ligament .
the uterus and adnexa were separate from the mass , pushed to the right side .
the mass was densely adhered to the urinary bladder but could be separated by dissection .
as per the tnm and irs staging , our patient was staged as stage i , group iii , with embryonal histology and thus classified as low risk subgroup .
rhabdomyosarcoma is the most common soft - tissue sarcoma of children accounting for 3 - 4% of all the childhood cancers .
rhabdomyosarcoma is known to be associated with caf au lait spots and neurofibromatosis type - i .
our patient though had caf au lait spots , did not have other features suggestive of neurofibromatosis . on reviewing the literature on broad ligament sarcomas ( excluding leiomyosarcomas ) , we came across only 3 such cases .
have reported the first of those in 1985 in their series of eight patients of female genital tract sarcomas .
the patient was 16 years of age with an alveolar rhabdomyosarcoma of the broad ligament .
the second case was a 55-year - old female diagnosed and treated for an undifferentiated pleomorphic sarcoma of the broad ligament reported by bouraoui et al .
the third patient , reported by diaz - murillo et al . was a 27-year female with an undifferentiated round cell sarcoma of the broad ligament .
our patient is a 9-month - old child with an erms of the broad ligament .
the patient reported by diaz - murillo was followed - up for 12 years without any recurrence .
the treatment is decided after a tissue diagnosis and metastatic work - up of the patient .
our patient after appropriate investigations was diagnosed as a non - metastatic pelvic erms . on the basis of cect scan findings of an abutting urinary bladder and non - visualization of the left ureter
, it was decided to start nact ( irs iv protocol ) and reassess for the response .
on repeating a cect scan after nact , it was a stable disease ; hence , it was decided to go ahead with surgery for appropriate local control of the tumor .
adjuvant radiation therapy was considered in view of the mass adherent to the urinary bladder , but withheld considering the age and low risk group of the patient .
broad ligament rhabdomyosarcomas are an extremely rare entity . to the best of our knowledge ,
this is the first case of an erms arising from the broad ligament in an infant .
broad ligament tumors are uncommon . 
 sarcomas rarely arise from the broad ligament , leiomyosarcomas being the most common . 
 rhabdomyosarcomas of the broad ligament are very rare . to the best of our knowledge , no case of an embryonal rhabdomyosarcoma of the broad ligament has been reported in literature .