Epithelioid Sarcoma Follow-up and Early Detection With CA-125 and PET-CT Scan

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Epithelioid sarcoma (ES) is believed to be derived from immature mesenchymal cells. It is most prevalent in adolescents and young adults with a propensity for the extremities distal > proximal. In addition to histologic confusion with benign necrotizing and necrobiotic histiocytic conditions, ES has a high local recurrence and metastasis.
We present 2 young adults with distal ESs that showed diffuse positive immunohistochemical staining for cytokeratin AE1/AE3, vimentin, epithelial membrane antigen, and the tumor antigen CA-125. Both patients were followed up after primary excision/ amputation with serologic CA-125 levels. At different times after primary excision, both patients showed increasing CA-125 levels. They then underwent positron emission tomography (PET)-computed tomography (CT) imaging studies for detection and localization of a satellite and metastatic focus in 1 and 3 metastatic foci in the other.
The new foci of ES were primarily excised without further amputations, and both patients have shown no further evidence of disease 4 years and 32 months later. Thus, CA-125 follow-up with localization of the tumor with PET/CT scan may decrease the long-term morbidity and mortality in patients with ESs.

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