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a 48-year - old chinese female was admitted to department of oncological surgery ( zhejiang cancer hospital , hangzhou ) with left upper abdominal pain , accompanying with belching and weight loss of 2.5 kg over the previous 3 months .
prior to this presentation , she had been healthy , without any previous medical illness . on physical examination ,
the left upper quadrant was slight tender but no abdominal mass was detected by abdominal palpation .
laboratory data were all normal . computed tomography ( ct ) demonstrated a low - density mass with a ct value of 1 hounsfield unit ( hu ) , measuring about 3.2  2.2 cm in the right lobe of liver ( figure 1a ) , and a solid low - density mass with a ct value of 8hu , measur - ing about 5.2 cm  4.6 cm in the upper pole of the spleen ( figure 1b ) .
ct demonstrated a low - density mass measuring about 3.2 cm  2.2 cm in the right lobe of liver ( a ) , and a solid low - density mass measuring about 5.2 cm  4.6 cm in the upper pole of the spleen ( b ) .
based on the physical examination and ct , the preoperative diagnosis of the hepatic cyst was definite but the preoperative diagnosis of the splenic tumor was difficult .
then a laparo - scopic splenectomy was performed under a presumed diagnosis of a malignancy of the spleen .
intraoperatively , a solid mass ap - proximately 5 cm in diameter was found in the upper pole of the spleen .
there were not any signs of metasta - ses or any visible neoplastic tissue remnant at the operation site .
the re - sected spleen measured 11  10  8 cm , weighed 740 g. histopathology showed that tumor cells were pleomorphic and consisted of fibroblasts , histiocytes , and myofibroblast - like cells arranged in a storiform pattern ( figure 2a ) .
immunohistochemistry showed these tu - mor cells were positive for vimentin ( vim ) , -smooth muscle actin ( -act ) and cd68 ( figure 2b ) .
based on these results of histopathology and immunohistochemistry , the final diagnosis of mfh of the spleen was conformed .
the patient was discharged on the 7th day after the initial operation and was not given any additional treatment .
she was followed up for 13 months without recurrence or metastases after the initial surgery at the time of reporting .
histopathology showed tumor cells were pleomorphic and consisted of fibroblasts , histiocytes , and myofibroblast - like cells arranged in a storiform pattern ( a ) .
mfh is the most common soft tissue sarcoma mainly occurring in the extremities ( 70% ) , followed by the abdominal or retroperitoneum ( 16%).1 the origin of mfh is still controversial .
primary mfh of the spleen is extremely rare , although the incidence of mfh of the spleen remains undetermined . since the first description of primary splenic mfh reported by govoni et al.6 in 1982 ,
only 13 cases have been reported in the english language literature in medline.617 compared with the 13 previously reported cases of mfh of the spleen , our case was the first case of mfh of the spleen accompanied with other disease , and also the first case treated successfully by laparoscopic splenectomy .
the spleen , for poorly documented reasons , generally shows relative resistance to malignant tumors .
accordingly , the preoperative diagnosis of mfh of the spleen is difficult because the cases are extremely rare , the clinical symptoms are nonspecific , and the imaging patterns are not suggestive .
these tumor cells consist of histiocyte - like and fibroblast - like cells with other multinucleated giant cells.11318 there are positive stainings for lysozyme , vim , cd68 , and -1 antitrypsin.13 in our case , we could not get a definite preoperative diagnosis of the splenic tumor according to the physical examination and ct scanning .
a final diagnosis of mfh of the spleen was confirmed by the histopathology and immunohistochemistry . in review of the english language literature in medline
, we found 13 previously reported cases of the mfh of the spleen ( table 1 ) . among the 14 cases of mfh of the spleen , including our case , six cases were females and eight cases were males with the age from 35 to 82 years old , mainly in the fourth or fifth decade of life ( 9/14 )
the presenting symptoms were usually nonspecific , but the clinical characteristics such as abdominal pain , fever , weight loss , and splenomegaly were prominent features in about 70% of the patients.17 about 35.7% ( 5/14 ) of the cases with splenic mfh presented metastases at the initial surgery including liver ( 3 cases ) , stomach and pancreas ( 1 case ) , and omentum ( 1 case ) ; and the liver was the most common metastatic site .
mfh has been categorized into five types , based on the histopathologic subtype , including storiform - pleomorphic , myxoid , inflammatory , giant cell and angiomatoid variants.16913 however , only the subtype of storiformpleomorphic and inflammatory variants have been reported in the mfh of the spleen in the previously reported literature . in review of the 14 cases , although 4 cases were not mentioned , eight cases were storiform - pleomorphic types including our case and two cases were inflammatory types .
all of the 14 patients underwent the surgery of splenectomy , while our case was the first case treated successfully by laparoscopic splenectomy .
six patients died of local recurrence with multiple metastasis , 3 , 6 , 7 , 8 , 15 , and 19 months postoperatively .
one patient was dead after splenectomy with hepatic involvement and the other was alive at 18 months with multiple hepatic metastases at the time of reporting .
five cases , including our case , survived for 3 , 13 , 17 , 18 , and 46 months , without local recurrence and distant metastasis at the time of reporting . reported cases of malignant fibrous histiocytoma of the spleen in the english language literature in medline
the incidence of splenic mfh remains undetermined due to limited data on a rare malignancy .
the clinical symptoms of primary splenic mfh are usually nonspecific , but the characterics such as abdominal pain , fever , weight loss , and splenomegaly are prominent features .
chemotherapy and radiotherapy are other therapeutic modalities for patients with metastasis , but the therapeutic effect is poor .
jff carried out the design and coordinated this study , hl and jl collected and analyzed the clinicopathologic data .
primary malignant fibrous histiocytoma ( mfh ) of the spleen is extremely rare . since the first description of primary splenic mfh reported by govoni et al in 1982 , to the best of our knowledge , 
 only 13 cases of mfh of the spleen have been reported in the english language literature in medline . 
 we herein report a rare case of primary splenic mfh accompanying with hepatic cyst in a 48-year - old chinese female who treated successfully by laparoscopic splenectomy and fenestration , which has not yet been reported in the literature . compared with the 13 previously cases of mfh of the spleen , 
 our case is the first case accompanied with other disease , and also the first case treated successfully by laparoscopic splenectomy . 
 a literature review of mfh of the spleen previously reported in the english language literature in medline is also provided .