First Reported Implementation of Lenalidomide as Maintenance Therapy for Metastatic Follicular Dendritic Cell Sarcoma

Follicular dendritic cell sarcoma (FDCS) is an exceptionally rare, low- to intermediate-grade malignancy arising from mesenchymal-derived follicular dendritic cells, but can present with high-grade pathological features and may rapidly become fatal. Conventional therapeutic strategies, primarily consisting of surgical resection with adjunctive systemic chemotherapy, such as gemcitabine-docetaxel, ifosfamide, doxorubicin, or other regimens, remain inconsistently effective, with median survival notably reduced in advanced or recurrent disease. Given the limited therapeutic options and frequent relapse, there is a critical need for well-tolerated treatments that can improve long term outcomes for patients with metastatic FDCS. Here, we report the first documented clinical use of the immunomodulatory agent Lenalidomide (Revlimid) as maintenance therapy following chemotherapy. Remarkably, the patient achieved nearly total sustained remission with exceptional disease stabilization for approximately six years, experiencing only one manageable recurrence. This clinical response points to lenalidomide as a promising maintenance therapy for metastatic FDCS and may provide further basis for consideration of lenalidomide as a potential first line treatment.