Lymphotropic Sarcomas (Epithelioid Sarcoma and Clear Cell Sarcoma)

Lymphotropic sarcomas encompass a rare subset of soft tissue sarcoma histotypes known for their distinct ability to metastasize to locoregional lymph nodes. Epithelioid and clear cell sarcomas are the predominant lymphotropic subtypes and the focus of this review. The utility of lymph node assessment, whether clinical, radiographic, and/or surgical, in patients with these sarcomas is presented. The role of sentinel lymph node biopsy as well as completion lymphadenectomy in patients with these lymphotropic sarcoma histotypes is discussed. Surgery remains the only potential curative option for these tumors, and advancement in histology-specific chemotherapeutic regimens and biologic therapy options is necessary. This review contains 6 tables and 50 references. Key words: clear cell sarcoma, completion lymphadenectomy, epithelioid sarcoma, EWS-ATF1, limb-sparing surgery, lymph node metastasis, sentinel lymph node biopsy, soft tissue sarcoma