Epithelioid neoplasms are generally carcinomas. As confirmation that every rule is meant to be broken, some sarcomas demonstrate epithelioid morphology, and can even express cytokeratins. These sarcomas have unique behavior, for example, a much higher rate of lymph node metastasis than other sarcomas. This group of sarcomas also presents diagnostic and therapeutic challenges to those clinicians who help patients contend with these difficult tumors. In this review, some of the major categories of epithelioid soft tissue tumors are described, with clinical data reported as available. Some of these tumors provide excellent opportunities to examine newer protein-targeted agents in investigational settings.
aDepartment of Anatomic Pathology, General Hospital of Treviso, Treviso, Italy
bCenter for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Boston, MA
cUnit of Molecular Cytogenetics–UO5, Fondazione IRCCS, Istituto Nazionale dei Tumori, Milano, Italy
eCentre Pluridisciplinaire d'Oncologie, University Hospital, Lausanne, Switzerland
Address correspondence to Angelo Paolo Dei Tos, MD, Director of Anatomic Pathology, General Hospital of Treviso, Piazza Ospedale 1, 31100 Treviso, Italy
1,2 These authors contributed equally to this work.
ABSTRACT