Epithelioid sarcoma: a review and update

Abstract

Epithelioid sarcoma is a rare, highgrade, soft tissue tumor that has a known propensity for local recurrence, regional lymph node involvement, and distant metastases. We review the clinical and histological presentations of epithelioid sarcoma. Because epithelioid sarcoma presents innocuously, it is often mistaken as a benign process, which can result in insufficient treatment. Therefore, we emphasize the need for clinicians to consider this diagnosis when a slowgrowing tumor is found on the distal extremity of a young male as the malignancy inherently portends a poor prognosis. Prognostic factors, such as local recurrence, regional metastatic disease, and tumor width, are discussed along with current treatment modalities, which include radical excision, sentinel lymph node biopsy, and radiation.

Figure 1

Fungating, ulcerated, epithelioid sarcoma of the thumb. Photo courtesy of Elena Kret-Sudjian, MD, PhD, Department of Internal Medicine, UC Davis Medical Center

Figures 2a–2c

(a) Medium power view of a granulomalike growth pattern with central necrosis and a mixture of spindled and epithelioid cells surrounding necrotic areas (10x); (b) The tumor is composed of sheets of large round or polygonal epithelioid cells in a pseudogranulomatous pattern as well as spindled cells with areas of necrosis and central hyalinization (20x); (c) Tumor cells have abundant ill-defined eosinophilic cytoplasm, pleomorphic hyperchromatic irregularly shaped nuclei of varying sizes with open chromatin and prominent central nucleoli (20x). Images courtesy of Mary Sidawy, MD, and Saadiya Haque, MD, Department of Pathology, Georgetown University Hospital