Yanchu Li, Li Zhang, Qiongwen Zhang, Jiayu Duan, Feng Wang
Head and Neck Oncology Ward, West China Hospital of Sichuan University, Chengdu, China.
Front Oncol. 2024 Dec 20;14:1398350. doi: 10.3389/fonc.2024.1398350. eCollection 2024.
Histiocytic sarcoma originates in various tissues, including the skin, lymph nodes, gastrointestinal tract, lungs, bone marrow, and central nervous system. Primary central nervous system histiocytic sarcoma (PCNSHS) is exceptionally rare, known for its aggressive behavior and poor prognosis. This report describes a case of PCNSHS in the cerebellum treated with surgery and radiotherapy.
A 30-year-old woman presented with progressive dizziness and headache. Magnetic resonance imaging scans showed right cerebellar neoplastic lesions approximately 3.6 cm*3.0 cm with cerebral edema and fourth ventricle and brainstem compression. The patient underwent surgical debulking, and the pathological diagnosis was PCNSHS. Two months after the surgery, the patient underwent adjuvant radiotherapy at a dose of 60 Gy. No tumor progression has been observed during the one-year follow-up period.
This case report provides an example of effective central nervous system control using resection and radiation therapy. A review of the literature confirms that surgery alone or combined concurrent or sequential treatment of radiotherapy and chemotherapy is often used; however, the best treatment plan remains unclear. Moreover, the prognosis is poor, with a median survival of six months. Thus, ongoing research aims to better understand the biology of histiocytic sarcomas and find more effective strategies.
组织细胞肉瘤起源于多种组织，包括皮肤、淋巴结、胃肠道、肺、骨髓和中枢神经系统。原发性中枢神经系统组织细胞肉瘤（PCNSHS）极为罕见，以其侵袭性生物学行为和不良预后而闻名。本报告描述了一例发生于小脑的PCNSHS病例，采用手术和放疗进行治疗。
一名30岁女性出现进行性头晕和头痛。磁共振成像扫描显示右侧小脑有一个大小约为3.6 cm×3.0 cm的肿瘤性病变，伴有脑水肿以及第四脑室和脑干受压。患者接受了手术减瘤，病理诊断为PCNSHS。术后两个月，患者接受了剂量为60 Gy的辅助放疗。在一年的随访期内未观察到肿瘤进展。
本病例报告提供了一个通过手术切除和放射治疗有效控制中枢神经系统肿瘤的实例。文献回顾证实，通常采用单纯手术或放疗与化疗的联合同步或序贯治疗；然而，最佳治疗方案仍不明确。此外，预后较差，中位生存期为6个月。因此，正在进行的研究旨在更好地了解组织细胞肉瘤的生物学特性并找到更有效的治疗策略。
