Amirtham Usha, Manohar Vidhya, Kamath Mangesh Padmanabha, Srinivasamurthy Premalatha Chennagiri, Chennagiriyappa Lakshmaiah Kuntegowdenahalli, Shenoy Ashok Mohan, Renuka Pramod Kallur Paramesh, Kumar Rekha Vijay
Associate Professor, Department of Pathology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
Fellow, Department of Pathology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
J Clin Diagn Res. 2016 Aug;10(8):XC08-XC11. doi: 10.7860/JCDR/2016/19763.8386. Epub 2016 Aug 1.
Follicular Dendritic Cell Sarcomas (FDCS) are rare disorders of the lymph node and soft tissues. Accurate characterization of these neoplasms is important in planning optimal treatment given its potential for recurrence and metastasis.
To analyse the clinicopathological profile and outcomes of a series of 10 cases of follicular dendritic cell sarcoma arising in the head and neck region diagnosed at our regional cancer centre.
A series of 10 cases of FDCS of the head and neck region from the hospital registry of head and neck cancers diagnosed between 2007 and 2013 were collected and analysed retrospectively. Clinical details, pathologic features, immuno-phenotypic profile, treatment approach and outcomes over a period of 5 years were noted. The Recurrence Free Survivals (RFS) of all the patients were recorded.
The median age of the patients was 57 years and the mean age was 50.9 years. Male: female ratio was 7:3. In eight of the 10 patients, the tumours were located in the tonsils. All the tumour cells showed diffuse cytoplasmic CD21 and CD23 positivity by IHC. All the 10 cases underwent surgical excision of the tumour and three cases underwent additional neck dissection for cervical lymph node enlargement. All the patients with high grade tumours were uniformly managed with Tri-modality treatment (Surgery followed by radiotherapy and chemotherapy). The overall recurrence rate was 70%. The mean and median recurrence free survival was 39.6 and 44 months, respectively. Two of the three patients who remained recurrence free at the end of the 60 months had low grade tumours.
Early recognition of follicular dendritic cell sarcomas requires a high index of suspicion and bi-modality or tri-modality treatment may cure a subset of low and high grade tumours respectively and prolong recurrence in a large subset of patients. Surgery is the mainstay and the definitive modality of treatment; the advantages and benefit of adjuvant radiotherapy and chemotherapy are yet to be established. Tri-modality management may have a role in high grade patients which needs to be substantiated in future studies.
滤泡树突状细胞肉瘤（FDCS）是淋巴结和软组织的罕见疾病。鉴于这些肿瘤有复发和转移的可能性，准确地对其进行特征描述对于规划最佳治疗方案很重要。
分析在我们地区癌症中心诊断出的10例头颈部滤泡树突状细胞肉瘤患者的临床病理特征及预后。
收集并回顾性分析了2007年至2013年间在头颈癌医院登记处登记的10例头颈部FDCS病例。记录了临床细节、病理特征、免疫表型特征、治疗方法及5年期间的预后情况。记录了所有患者的无复发生存期（RFS）。
患者的中位年龄为57岁，平均年龄为50.9岁。男女比例为7:3。10例患者中有8例肿瘤位于扁桃体。所有肿瘤细胞经免疫组化显示弥漫性细胞质CD21和CD23阳性。所有10例患者均接受了肿瘤手术切除，3例因颈部淋巴结肿大接受了额外的颈部清扫术。所有高级别肿瘤患者均采用三联疗法（手术，随后进行放疗和化疗）。总体复发率为70%。无复发生存的平均和中位生存期分别为39.6个月和44个月。在60个月结束时仍无复发的3例患者中有2例为低级别肿瘤。
早期识别滤泡树突状细胞肉瘤需要高度的怀疑指数，双模式或三模式治疗可能分别治愈一部分低级别和高级别肿瘤，并延长大部分患者的复发时间。手术是主要的治疗方式；辅助放疗和化疗的优势和益处尚未确定。三联疗法可能对高级别患者有作用，这需要在未来的研究中得到证实。
