Successful Treatment of Interdigitating Dendritic Cell Sarcoma Presenting as Multiple Parotid Tumors

Abstract Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm derived from antigen-presenting cells of the immune system. It mostly occurs in the lymph node of the neck or axilla. We report a case of IDCS occurring in an 82-year-old female who presented with multiple masses in her right parotid gland. The patient was successfully treated with conservative surgery preserving the facial nerve followed by radiotherapy for the macroscopic lesion remnants. Most localized diseases were treated by surgery with or without irradiation, while advanced diseases were treated with systematic chemotherapy, such as CHOP. Radiotherapy may be an effective alternative to complete resection in patients with localized IDCS that involves functional and/or vital structures. The present case demonstrated that conservative surgery preserving the facial nerve followed by radiotherapy is an effective alternative option for the treatment of IDCS.