Abstract
Tumors of the follicular dendritic cells (FDC-Sarcoma) represent a rare entity with only about 200 cases reported worldwide. The majority (60%) of cases arise primarily in cervical, abdominal or axillar lymph nodes, but extra nodal origin from secondary lymphatic tissue like the tonsils, Waldeyer's ring or MALT is also common (40%). The current report presents a characteristic course of a cervical FDC-Sarcoma, with its challenges in establishing the initial diagnosis and the struggle for therapeutic options. The FDC-Sarcoma presented recurrently for four times. Three different university hospitals in Germany were involved in the patients' treatment. Due to the patients' refusal, no adjuvant therapy was applied. In the end, a neck dissection was performed. The patient was closely followed up and has been recurrence-free for 10 years. This case suggests operative resection in combination with a neck dissection as a curative therapy for FDC-Sarcoma of the head and neck.