Undifferentiated embryonal sarcoma of the liver mimicking acute appendicitis. Case report and review of the literature
© Sakellaridis et al; licensee BioMed Central Ltd. 2006
Received: 30 July 2005
Accepted: 20 February 2006
Published: 20 February 2006
Undifferentiated embryonal sarcoma (UES) of liver is a rare malignant neoplasm, which affects mostly the pediatric population accounting for 13% of pediatric hepatic malignancies, a few cases has been reported in adults.
We report a case of undifferentiated embryonal sarcoma of the liver in a 20-year-old Caucasian male. The patient was referred to us for further investigation after a laparotomy in a district hospital for spontaneous abdominal hemorrhage, which was due to a liver mass. After a through evaluation with computed tomography scan and magnetic resonance imaging of the liver and taking into consideration the previous history of the patient, it was decided to surgically explore the patient. Resection of I–IV and VIII hepatic lobe. Patient developed disseminated intravascular coagulation one day after the surgery and died the next day.
It is a rare, highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with or without postoperative chemotherapy.
Stocker et al, named undifferentiated embryonal sarcoma of the liver, as an entity in 1978 on the basis of an Armed Forces Institute of Pathology (AFIP) series . It is a rare, highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with or without postoperative chemotherapy. We report our experience treating a 20-year-old Caucasian male with undifferentiated embryonal sarcoma of the liver.
Undifferentiated embryonal sarcoma (UES) of the liver was first described as a clinicopathologic entity in 1978. Before that, the nomenclature was variable and included mesenchymoma, primary sarcoma of the liver, fibromyxosarcoma and malignant mesenchymoma .
After neuroblastoma and Wilm's tumor, primary hepatic neoplasms are the third most common solid malignant tumors found in children accounting for 2% of the total of childhood cancers. Although rare, malignant mesenchymal hepatic tumors are the fourth most common malignant tumors of the liver in the children, with hepatoblastoma and hepatocellular carcinoma accounting for the majority of primary hepatic malignancies in children [2, 3].
The UES of the liver is typically diagnosed after 6 years of age with a decline in incidence after 10 years of age and 50% of the patients were between the above ages in the longest reported series . In children, there is a slight male predominance (1.0:0.65) . In 1978, 31 patients were reported with embryonal sarcoma with a median survival time of less than 1 year . A recent review of published cases revealed a better outlook with 37.5% of patients alive without disease for an average of 37.5 months . Two further patients are reported alive and free of disease more than 5 years after complete surgical excision of tumor and chemotherapy .
There are no specific clinical features. Tumor related symptoms might be regarded as abdominal mass with or without upper abdominal pain or swelling. Fever is probably related to the hemorrhage and necrosis found in the majority of these tumors, as happened in our patient. Of possible significance is the absence of jaundice. Rupture into the tumor or into the peritoneal cavity due to rapid growth of the tumor is not uncommon . Review of the literature did not reveal a case of undifferentiated embryonal sarcoma mimicking acute appendicitis. Different from primary carcinoma of the liver, UESL has no relation to hepatitis or liver cirrhosis, no disturbance of hepatic function. Laboratory studies are non-specific, and the α-fetoprotein is not increased [1, 2, 7].
Radiographs of the abdomen are usually normal. The lesion can be detected by ultrasound, CT and MRI. MRI localizes the lesion more accurately than the other methods, with good resectability correlation. It also can detect vascular invasion, biliary obstruction and hilar adenopathies .
UES of the liver is a neoplasm with primitive mesenchymal phenotype. Tumor size often exceeds 10 cm and can be as large as 30 cm. Macroscopic examination shows a single, well-demarcated, soft, globular mass that frequently has cystic, gelatinous, hemorrhagic and necrotic foci. Microscopic examination reveals a pseudocapsule surrounding a neoplasm, composed predominately of spindle, oval, or stellate cells with ill-defined cell borders. The tumor cells are embedded in an abundant myxoid stroma that contains many thin-walled veins. The bile duct-like structures occasionally appear hyperplastic or reactive, although they, too, may show degenerative changes . Immunohistochemical studies have indicated variable immunoreactivity with antibodies to desmin, muscle-specific actin, and cytokeratin, but not myoglobin . Vimentin and the "histiocytic" determinants (alpha-1-antitrypsin and alpha-1-antichymotrypsin) have been the only consistent immunohistochemical markers expressed by this tumor. However, it is known that the latter two markers are not specific for histiocytes, but are expressed in a range of tissue types including epithelium . In our case the immunohistochemical studies revealed expression to vimentin, desmin, but not to muscle-specific actin, S-100-protein and cytokeratin.
The prognosis for UESL has been poor to until recently and majority of the patients died of tumor recurrence or metastasis within two years after the initial operation . The major impediments in achieving long-term, disease-free intervals, is local recurrence in the upper abdomen and distant metastases . Metastases to lung, pleura and peritoneum are common; invasion of the vena cava with extension into the right atrium rarely occurs . Recent researchers have shown that pre- and/or post-operative systemic chemotherapy (with cisplatin, andriamycin, cyclophosphamide) and/or radiotherapy, when necessary, can remarkably improve patient's survival [15, 16]. Because the tumor does not produce any characteristic serum markers to permit monitoring of subclinical recurrences, the second-look laparotomy after the completion of chemotherapy should be considered . Once there is an evidence of recurrence, resection of the tumor wherever feasible should be performed .
The written permission was obtained from the patients/ next of kin for publication of this case report.
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