adenomatous hyperplasia of gallbladder is the most common benign mesenchymal proliferation , accounting for more than 40% of tumor - like lesions of the organ .
malignant mesenchymal neoplasms of the gallbladder are extremely rare and only 105 isolated cases of primary gallbladder sarcoma have been reported .
a variety of tumor types have been reported ( such as leiomayosarcoma , rhadomyosarcom , angiosarcoma , kaposi 's sarcoma , malignant fibrous histiocytoma , and synovial sarcoma ) .
leiomyosarcomas are usually diagnosed at an advanced stage therefore surgical management is not a therapeutic option .
consensus about the management of leiomyosarcomas is limited due to limited experience with to this type of tumor .
we present a case of leiomyosarcoma of the gallbladder treated by radical cholecystectomy and small bowel resection .
a 54-year - old male patient presented to the outpatient clinic with complaints of a palpable mass in the right upper quadrant of the abdomen , nausea and some weight loss . on physical examination , a firm and fixed mass was found in the abdomen .
the tumor markers ( carcinoembyonic antigen , carbohydrate antigen 19 - 9 , alpha - fetoprotein ) were also normal .
endoscopic ultrasound examination revealed a gallbladder mass without direct invasion of liver parenchyma and duodenum ( fig .
1 ) . abdominal computed tomography ( ct ) and magnetic resonance cholangiopancreatography ( mrcp ) demonstrated a 9-cm mass lesion that was attached to the small bowel and abdominal wall , but had not invaded the liver parenchyma ( figs . 2 , 3 ) .
the stage of tumor using the standard tumor - node - metastasis staging system was t4n0m0 .
the 18f - fluorodeoxyglucose positron emission tomography / ct that was performed to check for distal metastasis revealed a hypermetabolic lesion ( maximum standardized uptake value [ suvmax ] 14.5 ) in the entire gallbladder ( fig .
we performed a simultaneous radical cholecystectomy ( s4b + s5 segmentectomy ) , omentectomyand small bowel segmental resection .
we also did dissection of lymph nodes around the hepatoduodenal ligaments and the celiac trunk .
histopathology showed a malignant spindle cell tumor consisting of leiomyosarcoma with marked nuclear atypia and mitosis ( 62/10 high - power fields [ hpfs ] ) ( fig .
the neoplastic cells infiltrated the muscularispropia layers of the small bowel and the soft tissue of the abdominal wall .
the tumor cells showed positive immunoreactivity for smooth muscle actin ( sma ) ( fig .
there was negative immunoreativities for the following markers ; caldesmon , cytokeratin ( ck ) 7 , ck19 , ck20 , cd31 , cd34 , c - kit , calponin , desmin , myoglobin , hmb45 , high molecular weight cytokeratin ( hmwck ) , panck , and s100 .
a pulmonary thromboemolism in the upper left anterior lobe 's segmental branch was revealed in a chest ct therefore , the patients received thrombolysis followed by heparin anticoagulation therapy .
multiple liver metastasis and seeding metasitasis on the peritoneum were found in an abdominal ct 1 month postoperatively .
the patient subsequently underwent adjuvant chemotherapy composed of mesna , adriamycin , ifosfamide and dacarbazine ( maid ) for 3 months .
sarcomas of the gallbladder are rare and represent about 1.5% of all malignant gallbladder diseases .
a variety of tumor types have been described including leiomyosarcoma , rhabdomyosarcoma , angiomyosarcoma , kaposi 's sarcoma , malignant fibrous histiyocytoma , and synovial sarcoma .
leiomyosarcoma of the gallbladder is an especially rare malignant tumor . by 1984 , 105 cases of primary sarcomas of the gallbladder had been reported , with primary leiomyosarcomas accounting for 7% of them .
the diagnoses were established in accordance with the new world health organization classifications for soft tissue tumors and the most recent soft tissue criteria published .
leiomyosarcoma is defined as a malignant tumor composed of cells showing distinct smooth muscle features . in the macroscopy ,
leiomyosarcoma typically forms a fleshy mass , with colors varying from grey to white to tan .
the typical histopathologic pattern of leiomyosarcoma is that of intersecting , sharply marginated groups of spindle cells . in the immunophenotype , desmin , h - caldesmon , and sma
none of these are absolutely specific to smooth muscle and positivity for two of these markers were more supportive of leiomyosarcoma than positivity for one alone .
immunostains may be focally positive on cd34 , epithelial membrane antigen ( ema ) , keratin and s100 .
a diagnosis should be made on the appropriate morphologic features , not only on the immunostains . in this case , immunopositive staining was strong for sma and vimentin but the typical histopathologic pattern of leiomyosarcoma had appeared in the hematoxylin and eosin stain .
leiomyosarcoma is more frequent in women between the ages of 50 and 75 years and usually has a poor prognosis .
the presence of gallstones are invariable and the symptoms presented are those of chronic cholecystitis .
histopathologically , the majority of these tumors are - high grade and display an epithelioid morphology ; but cases with features of well - differentiated leiomyosarcoma have been described . according to the national comprehensive cancer network clinical practice guideline in oncology ver .
i. 2011 , patients with a resectable intraabdominal sarcoma should undergo immediate surgical treatment with a grossly negative margin and and possible interoperative radiation therapy .
the postoperative margin status was the most important factor contributing to long - term disease free survival .
postoperative treatment options were dependent on the surgical outcomes and clinical , or pathological finding following surgery .
postoperative radiation therapy should be considered in patients with pathological findings of high grade disease following a negative margin resection ( r0 resection ) or for microscopic positive margins ( r1 resection ) .
for patients with unresectable or disseminated recurrences , preoperative rt and/or chemotherapy should be considered after a biopsy .
combination regimens with activity in soft tissue sarcoma include ad ( doxorubicin , dacarbazine ) , aim ( doxorubicin , ifosfamide , mesna ) , maid , and so on .
the single agents include dacarbazine , doxorubicin , epirubicin , gemcitabine , ifosfamide , liposomal doxorubicin and temozolomide .
the prognosis of sarcoma and leiomyosarcomas of the gallbladder is dismal , the five year survival rate being less than 5% .
this is due to the fact that at the time of the diagnosis or surgery .
our patient was also diagnosed at an advanced stage , but he had no distant organ metastasis . because r0 resection is expected in this case , an aggressive surgical approach was attempted .
however , soon after , multiple liver metastasis and peritoneal seeding metastasis were detected in postoperative evaluations . therefore additional aggressive multimodality treatments such as surgery with chemotherapy are the only way to increase the survival rate . in conclusion , the five year survival rate of leiomyosarcoas of the gallbladder is less than 5% .
however for young and healthy patients with leiomyosarcomas of the gallbladder , aggressive surgical treatment followed by adjuvant chemotherapy should increase the survival rate despite high mortality and morbidity .
further studies are needed to confirm the benefit of aggressive treatment for patients with leiomyosarcoma of the gallbladder .
also surgeons will have to tread very carefully in selection of candidates for surgical treatments .
 malignant mesenchymalneoplasms of the gallbladder are extremely rare with only 105 cases of primary gallbladder sarcoma having been described . 
 it has a very aggressive behavior and is usually diagnosed at advanced stages . 
 therefore , curative surgical management may not be possible . 
 we performed a radical cholecystectomy ( s4b + s5 segmentectomy ) , omentectomy and small bowel resection in a 54-year - old patient with locally invasive leiomyosarcoma of the gallbladder . 
 further studies are needed to confirm the benefit of aggressive treatment for patients with leiomyosarcoma of the gallbladder . 
INTRODUCTION
CASE REPORT
DISCUSSION