Follicular dendritic cell sarcoma of the head and neck. Literature review and report of the tonsil occurrence in a Ugandan patient

Abstract

We report a case of follicular dendritic cell sarcoma (FDCS) in a 60-year-old Ugandan female who presented with a 6-year history of a progressive left sided tonsillary mass. General systemic examination was unrevealing and the patient underwent left tonsillectomy. She was subsequently lost to follow-up. Grossly, the mass measured 6 cm in diameter and had a mottled appearance due to tissue microhaemorrhages. Markers specific for follicular dendritic cell differentiation (CD21, CD35 and CD23), p53 and EGFR were expressed on immunohistochemical analysis. Review of all of the 49 published reports of tonsil FDCS showed that this entity tended to occur at younger age (mean: 44.5 yrs) in women than in men (mean: 49.4 yrs). Tumour size ranged from 0.8 to 5 cm in maximum dimension (mean 2.9 cm). Only 12.2% of the patients presented with metastatic disease at initial diagnosis, all localised in the cervical lymph nodes. Local or distant recurrences occurred after a mean period of 72.5 months. In conclusion, although the pertinent literature suggests that FDCS should be considered at least of intermediate grade, our review indicates that FDCS of the tonsil region behaves as a low-grade sarcoma.

Keywords: Extranodal haematological malignancy; Follicular dendritic cell sarcoma; Immunohistochemistry; Palatine tonsil.