leiomyosarcoma is a rare gynecologic malignancy that accounts for less than 1% of gynecological malignancies . leiomyosarcoma of the broad ligament is an even rarer condition . according to gardner s criteria , the diagnosis is made when the mass is completely separated from the uterus and adnexa . so far , 23 cases of primary leiomyosarcoma of the broad ligament have been reported in the literature published in english . in september 2014 , a 55-year - old , gravida 3 , para 3 woman with a bmi of 30 and a chief complaint of fever and dizziness was admitted to the infectious - diseases ward of the pars general hospital affiliated with iran university of medical sciences in tehran , iran . the results of a fever workup and examination for infectious , metabolic , and immunologic problems were all negative . imaging modalities revealed an endometrial polyp , two calcified myoma in the body of the uterus , and a solid , heterogeneous 70-mm mass in the right para - cervical space , posterior to the broad ligament , and far from the ovary . although a leiomyosarcoma of the broad ligament is rare , practitioners should consider it when dealing with masses in the region of the broad ligament . if there is any suspicion of malignancy , especially in the presence of fever , it is recommended to avoid morcellation during laparoscopy . leiomyosarcoma accounts for 3.7% of uterine malignancies and less than 1% of gynecological malignancies ( 1 ) . the most frequent solid tumor of the broad ligament is leiomyoma , and leiomyosarcoma of this region is very rare . it seems that the microscopic pattern plays an important role in the prognosis of this disease . according to gardner s criteria ( 2 ) , to make a diagnosis of broad ligament tumor , the mass should be completely separated from the uterus and adnexa . since very few cases have been reported , treatment of broad ligament leiomyosarcoma is based on the criteria used for uterine leiomyosarcoma . the first sarcomas of this region were reported in the latter part of the nineteenth century and were not diagnosed using the criteria of gardner et al . ( 2 , 3 ) . since then , only 23 cases of primary leiomyosarcoma of the broad ligament have been reported in the literature published in english , with the most - recent case reported in 2015 by gupta et al . , primary leiomyosarcoma of the broad ligament occurs in or on the broad ligament , with no connection to the uterus and ovaries ( 3 ) . this neoplasm occurs mainly among postmenopausal women , but a few reports show that it also may also occur in women of reproductive age . it often has an insidious course and a poor prognosis , similar to ovarian cancer ( 1 ) . since primary leiomyosarcoma of the broad ligament is very rare , its treatment is based on the criteria used for the uterine leiomyosarcoma ( 1 , 2 ) . none of the previous cases of leiomyosarcoma of the board ligament reported in the literature were correctly diagnosed before surgery and the final histopathology report , which indicates the importance of considering this rare disease . here , we describe the 24th case , that of a postmenopausal woman who presented with the unusual manifestation of fever , and the management of the case . in september 2014 , a 55-year - old , gravida 3 , para 3 woman , 5 years postmenopausal , with chief complaints of fever and dizziness , was admitted to the infectious disease ward of pars general hospital , a private hospital affiliated with iran university of medical sciences in tehran , iran . her past history included cardiac surgery at age 8 , and diabetes and hypertension beginning 12 years before . she was under 60 u of nph , 14 u of regular insulin , 10 mg of enalapril , and 6.25 mg of oral carvedilol daily . physical exam results were as follows : head , neck , chest , and cardiac exams were normal . after gynecologic consultation and bimanual exam , we detected a top normal and myomatous uterus , normal left adnexa , and a big , firm mass in the right adnexa . the lab results were as follows : hb : 7.4 gm / dl , wbc : 13600/mcl , platelet : 929000/mcl , esr : 130 mm / hour . tumor markers , such as ldh , afp , hcg , ca125 , ca19 - 9 , ca15 - 3 and cea , were all normal . the result of a fever workup were negative , as were all examinations for infectious , metabolic , and immunologic problems , including malaria , brucellosis , 2me , wright , ana , ds dna , blood and urine cultures , gamma glutamyl transferase , c3 , c4 , crp , sgot , and sgpt . a pelvic ultrasound showed an endometrial polyp , two calcified myomas with diameters of 27 and 24 mm in the body of the uterus , and a 60  65 hypo - echoic mass with areas of necrosis adjacent to the right aspect of the uterus . an axial ct scan of the abdomen and pelvis with iv and oral contrast showed a 9-mm , calcified gallstone and a 70-mm solid , heterogeneous mass in the right para - cervical space , posterior to the broad ligament , and far from the ovary . the differential diagnoses were subserosal myoma and right ovarian mass . magnetic resonance imaging ( mri ) showed a broad ligament mass ( figure 1 ) . as her general condition did not change , insulin , empirical antibiotics , and four units of packed cells were administered . a laparotomy confirmed a 7 7-cm right broad - ligament mass , adhering to the right pelvic side - wall , closely attached to the bladder and the right ureter . the uterus , tubes , and both ovaries were grossly normal and completely separated from the mass . the liver , sub - diaphragmatic area , para - aortic lymph nodes , gut , omentum , and peritoneum were all normal . pelvic and para - colic washings were taken for cytological assessment . after the mass was separated from the attached structures , it was entirely excised . total abdominal hysterectomy and bilateral salpingo - oophorectomy were performed , as were multiple lymph node , omental , and peritoneal biopsies . the uterus , adnexa , lymph nodes , omentum , and peritoneum revealed no metastasis , but the pathology of the mass showed a highly atypical neoplastic growth , composed of spindle and epithelioid cells with highly pleomorphic nuclei and large foci of necrosis ( figure 2 ) . there was mass effect on the bladder and uterus , without obvious invasion , and internal , non - enhancing parts in favor of necrosis . ( arrows ) : fascicles of spindle and devoid cells with large pleomorphic nuclei and abnormal mitotic shapes . in immunohistochemistry , the neoplastic cells showed positive staining for smooth muscle actin ( sma ) antibodies ( figure 3 ) . in the aftermath , the patient received 25 courses of radiotherapy and 5 courses of adjuvant chemotherapy . at the end of 12 months of leiomyosarcoma of the broad ligament is a very rare tumor and mainly involves postmenopausal women . leiomyosarcoma behaves unpredictably and has non - specific clinical manifestations , including abdominal pain , mass , distension , and anorexia ( 2 , 3 ) . the present case was admitted to the infectious ward due to fever and sweating . after a comprehensive literature review on medline and a search for hard copies of older articles in the oregon health and sciences university , we found the full text of 23 previously reported cases of leiomyosarcoma , none of which indicated fever without pain as the main presentation ( tables 2 , 3 ) . abbreviations : bso , bilateral salpingo - oophrectomy ; ct , chemotherapy , dod : dead of disease ; hpf , high power - field ; lso , left salpingo - oophrectomy ; n / s , not specified ; rt : radiotherapy ; tah , total abdominal hysterectomy . modified from : shah et al . abbreviations : bso , bilateral salpingo - oophrectomy ; ct , chemotherapy , dod : dead of disease ; hpf , high power - field ; lso , left salpingo - oophrectomy ; n / s , not specified ; rt : radiotherapy ; tah , total abdominal hysterectomy . leiomyoma is the most prevalent primary tumor of the broad ligament , and myolipoma , angioleiomyoma , and solitary , fibrous tumors are rare , benign tumors of this region ( 5 - 7 ) . malignant fibrous histiocytomas , alveolar soft - part sarcomas , and hyalinizing spindle - cell tumors are other examples of rare malignant tumors of this region . the histological differential diagnoses are endometrial stromal sarcomas , uterine carcinosarcomas , and gastrointestinal stromal tumors ( 4 ) . none of the previous cases of leiomyosarcoma of the broad ligament reported in the literature were correctly diagnosed before surgery and the final histopathology report . schmidt reported the first sarcoma of this region in 1887 ( 8) , followed by a few cases documented by german and french authors around the turn of the twentieth century ( 9 ) , which were not diagnosed using the criteria of gardner et al . , primary leiomyosarcoma of the broad ligament occurs in or on the broad ligament , with no connection to the uterus and ovary ( 2 ) . but the reliance on mitotic counts has been questioned due to adjacent necrosis , the influence of hormones , and an increased appreciation for the lack of reproducibility among observers ( 9 ) . the criteria used by zaloudek and norris are mitotic figures (  5 versus < 5 per 10 high - power field ( hpf ) ) , cellularity ( hyper - cellular versus normo - cellular ) , and nuclear atypia ( yes - no ) ( 10 ) . coindre et al . advocated a three - grade system , using mitotic figures , tumor differentiation , and necrosis ; this is very important , because of the prognostic and therapeutic implications ( 11 ) . immunohistochemically , these tumors have significant reactivity for actin , myosin , desmin , and estrogen receptors , which increase the possibility of the hypothesis of tumor hormonal responsiveness ( 12 ) . since very few cases have been reported , treatment of broad ligament leiomyosarcoma is based on the criteria used for uterine leiomyosarcoma . standard management for uterine sarcoma includes total abdominal hysterectomy and bilateral salpingo - oophorectomy , but pelvic and para - aortic lymphadenectomy is recommended only for carcinosarcoma and not for leiomyosarcoma , despite the fact that some cases of the latter show a higher incidence of nodal involvement ( 13 ) . occasionally , the tumor is large , adheres to surrounding structures , and has areas of hemorrhage or necrosis . the role of frozen section in the diagnosis can be omitted ( 13 ) , but in our case , frozen section revealed an accurate diagnosis . in nulliparous , pre - menopausal women or in the case of a tumor in early its stages , the ovaries should be conserved . adjuvant chemotherapy with or without radiotherapy can be used in selected cases ( 14 ) . some authors believe that when mitoses are less than 10 per 10 hpf , no further therapy is needed after surgical intervention ( 15 ) . reported a unique case : a 26-year old woman who , despite 30 - 40 mitosis in 10 hpf , was treated surgically without adjuvant treatment and remained disease free for more than 3 years . furthermore , she had a successful pregnancy , and the uterus and adnexa were found to be unremarkable during cesarean section ( 16 ) . it is well - known that malignancies can cause a fever . in some series , fever underlying malignancy accounts for up to 25% of cases of fever of underdetermined origin ( 17 ) . the pathophysiology of a tumor - induced fever may be caused by several mechanisms , including the release of cytokines ( tumor necrosis factor and interleukin-1 ) , tumor , or infiltrating mononuclear cells ( 18 ) . other causes include tumor necrosis or obstruction of a hollow duct or viscous , resulting in proximal infection ; drug fever ; ttp ; dvt ; and trousseau s syndrome . although ovarian cancers commonly cause a fever , there is no previous report of a fever being caused by a leiomyosarcoma of the broad ligament . our case indicates that fever might be present in patients with this very rare disease and should be considered in their diagnoses . a recent food and drug administration ( fda ) safety communication ( 19 ) discouraged the use of laparoscopic , uterine power morcellation in hysterectomy and myomectomy because of the chance of the presence of an underlying , unsuspected uterine sarcoma . in patients with such sarcomas , there is a risk that the procedure will spread cancerous tissue within the peritoneal cavity , significantly reducing the patient s long - term survival . our case shows that this consideration should be extended to cover patients who present with fevers and broad ligament masses . the most important point of this case is that a patient who presented with a chief complaint of fever actually had an unusual manifestation of leiomyosarcoma of the broad ligament . the patient s follow up was relatively short , 12 months , which might be considered a shortcoming of this presentation . although a leiomyosarcoma of the broad ligament is very rare , practitioners should consider it when dealing with tumoral masses in this region , particularly if the disease manifestation includes a fever . if there is any suspicion of malignancy , especially in the presence of fever , it is recommended to avoid power morcellation during laparoscopic treatment .
introductionleiomyosarcoma is a rare gynecologic malignancy that accounts for less than 1% of gynecological malignancies . 
 leiomyosarcoma of the broad ligament is an even rarer condition . according to gardner s criteria , 
 the diagnosis is made when the mass is completely separated from the uterus and adnexa . 
 so far , 23 cases of primary leiomyosarcoma of the broad ligament have been reported in the literature published in english.case presentationin september 2014 , a 55-year - old , gravida 3 , para 3 woman with a bmi of 30 and a chief complaint of fever and dizziness was admitted to the infectious - diseases ward of the pars general hospital affiliated with iran university of medical sciences in tehran , iran . 
 her symptoms had begun two weeks before . 
 the results of a fever workup and examination for infectious , metabolic , and immunologic problems were all negative . 
 imaging modalities revealed an endometrial polyp , two calcified myoma in the body of the uterus , and a solid , heterogeneous 70-mm mass in the right para - cervical space , posterior to the broad ligament , and far from the ovary . 
 after surgery , a histologic report revealed leiomyosarcoma.conclusionsalthough a leiomyosarcoma of the broad ligament is rare , practitioners should consider it when dealing with masses in the region of the broad ligament . 
 if there is any suspicion of malignancy , especially in the presence of fever , it is recommended to avoid morcellation during laparoscopy .