a 60-year - old lady presented to our sister department of medical gastroenterology at a tertiary health care institute with a history of an increasing mass in the right upper abdomen of one and a half year duration .
she also had a history of weight loss due to early satiety and decreasing appetite .
she had been evaluated with abdominal cect 14 months before by some local doctor which had shown a mass lesion in the right lobe of the liver .
all her systemic examinations were normal except a big nodular mass in the right hypochondrium merging with the liver .
the department of medical gastroenterology considered her for a repeat workup , with abdominal mri , rbc trigged nuclear scan of the liver , serum tumor markers and routine base investigations .
mri showed a nodular mass lesion arising from the right lobe of the liver , crossing towards the left lobe with involvement of the right portal vein , with areas of cystic changes and hemorrhage within the lesion ( fig .
her tumor markers , including -fetoprotein , cea , ca 19 - 9 and hcg , were all within normal limits .
she was also subjected to upper and lower gastrointestinal endoscopy to rule out any occult primary lesion in the gastrointestinal tract .
routine laboratory investigations including liver function tests , hemogram , kidney function , chest x - ray and ecg were within fairly normal limits , her coagulogram revealing pt elevated by 3 s. serology was negative for hepatitis b and c. she was also subjected to usg guided fnac which was inconclusive and revealed only blood cells .
after complete workup with a tentative diagnosis of fibrolamellar hepatocellular carcinoma the patient was referred to our department for surgical intervention . she was offered a diagnostic and if possible curative laparotomy after repeat imaging ( abdominal cect ) as she had again lapsed a period of 34 months after her workup . due to monitoring constraints
, her attenders did not agree to get an abdominal ct but were ready for any possible surgical intervention with the minimum of a biopsy to plan further treatment . on exploration
the patient was found to have nodular bilobular disease occupying almost all of the liver .
there were areas of boggy swelling in between firm nodules predominantly on the inferior surface of liver suggestive of bleeding within the tumor on aspiration .
the lesion involving both lobes of the liver was too big and multicentric for any resectional procedure and so a biopsy from the lesion was taken safely .
after getting her histopathological report the patient was referred to a medical oncologist for further treatment .
this is the first case of fibrosarcoma of the liver reported at our institute . on light
microscopy the tumor revealed homogeneous , spindle - shaped cells with abundant collagen fibers showing a classic herringbone pattern ( fig .
tissue samples were then sent to another referral cancer hospital for immunohistochemistry where immunoreactive vimentin was found in the tumor cells .
electron microscopically , the tumor cells were rich in rough endoplasmic reticulum without a basement membrane and were surrounded by large amounts of collagen fibers .
the purpose of this case report is to share information on a rare tumor of the liver , a primary fibrosarcoma , as up to the late seventies the literature about this entity was restricted to autopsy diagnosis . to our knowledge
there are case reports from various parts of the world [ 2 , 3 ] , but there are no series of cases of primary fibrosarcoma of the liver .
etiologically exposure to cadmium has been linked to carcinogenesis of this disease in animal models .
described a series of 20 cases of sarcomatous tumors of the liver , of which angiosarcoma was the commonest , and concluded that primary treatment is surgery with survival depending on the degree of differentiation . from the available literature , primary treatment to be considered for fibrosarcoma of the liver
is surgery with curative intent . in a recent article from sloan - kettering cancer center , between 1981 and 2004 they identified only 30 cases of primary liver sarcomas of which
most cases ( 10 ) were hemangioendotheliomas and only 3 were diagnosed to be primary fibrosarcoma .
only 11 of these patients underwent r0 resection and this group of resected tumors had a 5-year survival rate of 65% .
unfortunately our patient presented quite late in the course of disease even if we consider her first ct scan done one and a half year back .
there is a report of one patient with primary fibrosarcoma presenting with frequent episodes of hypoglycemia , where insulin - like growth factor ii was intensely stained in the golgi area of the tumor cells , suggesting its role in the mechanism of hypoglycemia .
on histopathological examination these neoplasms show typical light microscopic features like that of malignant rhabdoid tumor with filamentous cytoplasmic inclusions ( fig .
4 ) , and on electron microscopic examination show staining for both cytokeratin and vimentin by immunohistochemistry . after making a diagnosis of primary fibrosarcoma of the liver at an inoperable stage ,
an extensive review of the literature regarding the role of other treatment modalities was done which was not encouraging [ 5 , 8 ] . though few reports recommend chemotherapy   in liver metastasis of retroperitoneal fibrosarcoma consisting of cyclophosphamide , vincristine , farmorubicin , and dacarbazine , there is no definite role as treatment for primary lesion of liver .
overall the prognosis reported by most of the authors is dismal [ 1 , 2 , 3 , 10 ] .
we conclude by passing on the message that this rare entity does exist and is an area for workup to make guidelines for management of such tumors .
this may be one of the few reported cases of primary hepatic fibrosarcoma confirmed by immunohistochemistry and electron microscopy .
 a 60-year - old lady presented to us with a right upper abdominal mass . with a clinical diagnosis of liver tumor , she was evaluated with abdominal ct , mri , nuclear scan , tumor markers , usg guided fnac and other baseline investigations . 
 on evaluation she had a massive right lobe tumor crossing the midline . in view of the ambiguous diagnosis 
 she was subjected to laparotomy where the lesion was judged unresectable and a biopsy was taken . 
 histopathological examination showed the rare pathology of primary fibrosarcoma of the liver with features of homogeneous , spindle - shaped cells with abundant collagen fibers showing a classic herringbone pattern . 
 tissue samples were then sent to another referral cancer hospital for immunohistochemistry and immunoreactive vimentin was found in the tumor cells . 
 electron microscopically , the tumor cells were rich in rough endoplasmic reticulum without a basement membrane , and were surrounded by large amounts of collagen fibers . 
 the fibroblastic character of the tumor cells was suggested by light and electron microscopy . 