Objective: Follicular dendritic cell sarcoma is a rare disease with a non-specific and insidious presentation that is further complicated by difficult diagnostic and therapeutic assessment.
Methods: The database PubMed was searched for reports of follicular dendritic cell sarcoma between 1986 (first case published) and 2012. All of the articles presenting informations regarding one or more cases of follicular dendritic cell sarcoma of the head and neck region, in patients less than 18 years of age, were included. The reference lists for pertinent reports were also scanned to ensure that all relevant literature was included.
Results: We present a case of a 14 year-old girl, with a 2-month history of a right-sided level II neck mass. After a carefull radiologic evaluation the mass was resected combined with a right selective neck dissection. Histology with immunohistochemical staining was positive for follicular dendritic cell sarcoma. No recurrence was seen after 31 months follow-up. The literature search identified six more cases of pediatric follicular dendritic cell sarcoma of the head and neck. This is the first female patient with follicular dendritic cell sarcoma in the pediatric population.
Conclusions: Current treatment of head and neck follicular dendritic cell sarcoma consists of wide radical resection, with associated radiotherapy or chemotherapy only for cases with aggressive disease such as extracapsular invasion, tumor size ≥6 cm or after failure of the first-line surgical treatment.
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