颌下滤泡树突状细胞肉瘤病例

Jae Ho Yoo, Dong Won Lee, Jeong-Kyu Kim
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摘要

滤泡树突状细胞肉瘤(Follicular dendritic cell sarcoma,FDCS)是一种罕见的淋巴肉瘤,可发生在身体的任何部位,多见于淋巴结。六十二岁的男性左侧颌下腺区肿块已有五个月。患者接受了肿块切除术和颌下腺切除术。组织病理学显示,颌下腺淋巴结内有纺锤形和卵圆形细胞增生,呈星状排列,CD21、CD23、Vimentin、Ki-67均呈阳性,提示为FDCS。肿瘤大小为 3 厘米,没有累及切除边缘,也没有细胞不典型性和坏死,因此进行了定期随访。4 年后,发现左侧颌下腺区域有新的增大肿块。医生对肿块进行了大范围切除,并在左侧Ⅰ-Ⅲ水平进行了颈部解剖。组织病理学证实 FDCS 复发。患者接受了从左下颌骨到舌骨区的放射治疗。两年后,在左肺上叶发现新的结节,楔形切除证实 FDCS 转移。患者正在接受辅助化疗。
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A Case of Follicular Dendritic Cell Sarcoma in Submandibular Region
Follicular dendritic cell sarcoma (FDCS) is rare lymphoid sarcoma occurs anywhere in body, mostly in lymph nodes. Sixty-two-year-old man presented left submandibular gland region mass for 5 months. Mass excision with submandibular gland resection was performed. Histopathology showed proliferation of spindle and ovoid cells with storiform arrangement which were positive for CD21, CD23, Vimentin, Ki-67, suggested FDCS in submandibular gland region lymph node. Tumor size was 3cm with no involvement of resection margin, nor cellular atypia and necrosis, so regular follow up was performed. After 4 years, new enhancing mass in left submandibular area was found. Wide excision of mass with neck dissection on left level I-III was performed. Histopathology confirmed recurrence of FDCS. The patient underwent radiation therapy from left mandible to hyoid area. After 2 years, new nodule was found in left lung upper lobe, and wedge resection confirmed metastasis of FDCS. The patient is on adjuvant chemotherapy.
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