Pecorella I, Okello T R, Ciardi G, Ochola E, Ogwang M D
Department of Radiological, Oncological and Anatomic Pathology Sciences University of Rome "Sapienza", Rome (Italy).
Department of Surgery of Gulu University Medical School, St. Mary's Hospital Lacor, Gulu (Uganda).
Pathologica. 2017 Jun;109(2):120-125.
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.
我们报告一例60岁乌干达女性的滤泡性树突状细胞肉瘤（FDCS），该患者有6年左侧扁桃体进行性肿物病史。全身检查未发现异常，患者接受了左侧扁桃体切除术。随后她失访了。大体上，肿物直径6cm，因组织微出血而呈斑驳外观。免疫组化分析显示滤泡性树突状细胞分化特异性标志物（CD21、CD35和CD23）、p53和表皮生长因子受体（EGFR）均有表达。对已发表的49篇扁桃体FDCS报告的回顾显示，该肿瘤在女性中发病年龄（平均44.5岁）往往比男性（平均49.4岁）小。肿瘤最大直径范围为0.8至5cm（平均2.9cm）。仅12.2%的患者在初诊时出现转移性疾病，均局限于颈部淋巴结。局部或远处复发平均发生在72.5个月后。总之，尽管相关文献提示FDCS至少应被视为中级肿瘤，但我们的回顾表明扁桃体区域的FDCS表现为低度肉瘤。
