Immunohistochemical Analysis of Embryonal Sarcoma of the Liver

Abstract

Embryonal sarcoma of the liver is a rare, aggressive malignant tumor that typically occurs in children and teenagers. Microscopic features include spindle, oval, or stellate cells with poorly defined cell borders, nuclear pleomorphism and multinucleation, and variable immunoreactivity to cytokeratin, vimentin, and alpha-1-antitrypsin. Intracellular and extracellular PAS-positive, diastase-resistant hyaline globules are commonly present. The authors evaluated a panel of IHC stains to better define the pattern of immunoreactivity in this tumor. Embryonal sarcomas of the liver were identified from archival files and were immunostained with antibodies: cytokeratin AE1/3, hepatocyte, SMMS, myogenin, calponin, h-caldesmon, desmin, S100, vimentin, CD34, C-kit (CD117), CD10, ALK-1, PE10, Bcl2, p53, and Ki-67. Six cases were identified. Patient age ranged from 6 to 24 years. Tumors ranged from 10 to 20 cm and contained spindled and epithelioid areas with PAS-positive, diastase-resistant globules and atypical cells with focal multinucleation. All cases showed immunoreactivity with vimentin and five showed immunoreactivity with Bcl2. Focal immunoreactivity was seen with cytokeratin AE1/3 in three cases, CD10 in four, calponin in two, desmin in one, and p53 in four. All tumors were negative with hepatocyte, myogenin, CD34, SMMS, h-caldesmon, PE10, ALK-1, and S100. No cytoplasmic staining was seen with C-kit. The proliferation index ranged from 30% to 95%. The diagnosis of embryonal sarcoma is based on typical morphologic features in a large liver tumor occurring in a young patient. The most useful IHC stains help to exclude tumors such as hepatoblastoma, hepatocellular carcinoma, embryonal rhabdomyosarcoma, and other sarcomas.

Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis

“…Pleomorphic multinucleated giant cells are relatively frequent. In addition, as in our report, there are prominent eosinophilic, PAS+, and diastase-resistant globules in the tumor cell cytoplasm and extracellular matrix [7,[29][30][31].…”

Malignant Peripheral Nerve Sheath Tumor of the Liver: A Case Report

“…(double labeled fluorescence staining of laminin and S100, ×200). d Amorphous basement-membrane like material (arrow) and desmosome-like junctional complexes (arrowhead) were constant findings on ultrastructural level However, this tumor is reported to be S100 negative [6,7] and diastase resistant globules, which are characteristic for the embryonal sarcoma were missing from the presently examined tumor. There are two more options: both dendritic reticular cell sarcoma and MPNST are vimentin, S100 positive and constituted mostly by spindle cells.…”

Undifferentiated Embryonal Sarcoma of the Liver: A Concise Review

“…Furthermore, hepatocyte paraffin 1, myogenin, CD34, C-kit (CD117), surfactant (PE10), anaplastic lymphoma kinase 1 (ALK-1), and S100 are negative in most cases. 7,9 Individual markers are often not helpful in differentiating UESL from other liver tumors. Therefore, multiple immunostains are usually performed to help with the diagnosis.…”

“…Finally, negative staining for S100 and melanin markers is helpful to exclude melanoma and neural tumors. 7 The extensive panels of immunohistochemical markers are helpful in diagnosing UESL. However, the antibody selection has to be based on demographic information, clinical history, and histologic findings.…”

“…However, the diagnosis is often challenging because of the overlapping epidemiological, clinical, and radiological findings with those of other liver tumors. 7 …”