Abstract
Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare hematological neoplasm arising from dendritic cells that presents significant diagnostic and therapeutic challenges, particularly in cases of disseminated disease. Here, a 33-year-old woman presented with discomfort of the left pharynx accompanied by nasopharyngeal and cervical mass for 3 months. The histopathology confirmed the diagnosis of IDCS as the neoplastic cells were spindle or ovoid in shape, forming fascicles or whorls, and were positive for S-100, vimentin and CD163 but negative for CD21, CD23, CD35 and CD1a. The patient underwent autologous hematopoietic stem cell transplantation (auto-HSCT) after achieving partial remission (PR) from six courses of chemotherapy based on the ABVD regimen and one cycle of radiotherapy. Encouragingly, the mass disappeared after cladribine containing regimen pretreated auto-HSCT and the patient has been in complete remission (CR) state for over 5 years. Therefore, the long survival of this patient might suggest that ABVD regimen with a sequential cladribine-containing preparative regimen prior to auto-HSCT may improve the prognosis of disseminated IDCS. However, this represents only a single-case experience, and further studies with larger sample sizes are required for validation.