Interdigitating dendritic cell sarcoma: case report with review of the literature

Abstract

Background: Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare tumor. The characteristics of IDCS and its optimal therapeutic approach have not been fully clarified.

Case report: We report the case of a 53-year-old Chinese male patient presenting with a subcutaneous nodule in the right chest wall. The histological and immunohistochemical features of the nodule confirmed the diagnosis of IDCS. Complementary examination excluded other involvement of the tumor. The patient was alive without evidence of disease 1 year after tumor resection followed by radiotherapy.

Conclusion: With regard to the literature, IDCS presents with a wide spectrum of clinical manifestations, and its correct diagnosis requires awareness of this rare disease and the use of appropriate markers. Surgery with curative potential might remain the first treatment option, and current data do not support adjuvant therapy. Systemic chemotherapy is mainly suggested for extensive disease while the long-term efficacy is unsatisfactory. The prognosis of IDCS seems to be associated with the initial stage of disease.