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Epithelioid sarcoma in children and adolescents. An immunohistochemical study
Insights
Epithelioid sarcoma is a rare soft tissue tumor. Immunohistochemistry reveals its potential for multidirectional differentiation, supporting a multipotent mesenchymal stem cell origin.
Area of Science:
- Oncology
- Pathology
- Immunohistochemistry
Background:
- Epithelioid sarcoma is a rare soft tissue sarcoma.
- This study analyzed six pediatric cases.
Purpose of the Study:
- To characterize epithelioid sarcoma using light microscopy and immunohistochemistry.
- To investigate the differentiation potential and cellular origin of epithelioid sarcoma.
Main Methods:
- Conventional light microscopy was used.
- Immunohistochemistry was performed using a panel of antibodies.
- Analysis included vimentin, cytokeratin, EMA, HMFG-2, NSE, S-100, and alpha-1-antichymotrypsin.
Main Results:
- Four of six tumors showed multinucleated giant cells.
- All cases were positive for vimentin, cytokeratin, EMA, and HMFG-2.
- Five cases were positive for neuron specific enolase (NSE), three for S-100, and two for alpha-1-antichymotrypsin.
Conclusions:
- Immunohistochemical findings support multidirectional differentiation capabilities of epithelioid sarcoma.
- Results suggest derivation from a multipotent mesenchymal stem cell.
Abstract:
Six cases of epithelioid sarcoma were studied by conventional light microscopy and immunohistochemistry. The six cases account for 1.4% of the 417 cases of soft tissue sarcoma collected at the Paediatric Tumor Registry, Kiel. The average age of the five male and one female patient was 10.8 years (median: 13 years). Particular clinical findings included the location of the tumours; three were found in the pelvis, two in the head and neck, and one in the hand. Four patients are living without disease, and one patient died of disease three years after diagnosis. Histologically, four of the six tumours revealed multinucleated giant cells. Immunohistochemically using a panel of mono- and polyclonal antibodies all cases stained positively for vimentin, cytokeratin, epithelial membrane antigen (EMA), and human milk fat globulin (HMFG-2). Five cases were positive for neuron specific enolase (NSE), and three stained positively for protein S-100. A positive reaction for alpha-1-antichymotrypsin was noted in two cases. These immunohistochemical findings attest to the multidirectional differentiating capabilities of epithelioid sarcoma and support the concept of derivation from a multipotent mesenchymal stem cell.