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Abstract：
Histiocytic sarcoma (HS) is an extremely rare but aggressive hematopoietic malignancy, and the prognosis has been reported to be rather unfavorable with a median overall survival of merely 6 months. We presented a 58-year-old female patient complaining of abdominal pain and fever, who was admitted to our institution in September 2021. Fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET/CT) scan showed enlargement of generalized multiple lymph nodes. Subsequently, laparoscopic retroperitoneal lesion biopsy and bone marrow aspiration were performed. The pathological findings indicated the diagnosis of HS concurrent with follicular lymphoma. The immunohistochemistry (IHC) staining of the tumor lesion revealed a high expression of CD38 and PD-L1 proteins. Furthermore, KRAS gene mutation was identified by means of next-generation sequencing. The patient exhibited poor treatment response to both first- and second-line cytotoxic chemotherapies. Therefore, she underwent six cycles of Daratumumab (anti-CD38 monoclonal antibody), Pazopanib (multi-target receptor tyrosine kinases inhibitor) combined with third-line chemotherapy, followed by involved-site radiotherapy and maintenance therapy with the PD-1 inhibitor Tislelizumab. Long-term partial remission was finally achieved after multi-modality treatment. Duration of remission and overall survival reached 22 and 32 months, respectively. Our case indicated that immuno-targeted treatment coupled with chemotherapy and radiotherapy might constitute a potential therapeutic option for HS.
摘要：
组织细胞肉瘤（HS）是一种极为罕见但侵袭性的造血系统恶性肿瘤，据报道，预后相当不利，中位总生存期仅为6个月。我们介绍了一名58岁的女性患者，抱怨腹痛和发烧，他在2021年9月被我们的机构录取。氟-18-氟脱氧葡萄糖（FDG）正电子发射断层扫描-计算机断层扫描（PET/CT）扫描显示广泛的多个淋巴结肿大。随后，行腹腔镜腹膜后病变活检和骨髓抽吸术。病理提示HS合并滤泡性淋巴瘤。肿瘤病变的免疫组织化学（IHC）染色显示CD38和PD-L1蛋白的高表达。此外，通过下一代测序鉴定KRAS基因突变。患者对一线和二线细胞毒性化疗均表现出较差的治疗反应。因此,她接受了六个周期的Daratumumab（抗CD38单克隆抗体），帕唑帕尼（多靶点受体酪氨酸激酶抑制剂）联合三线化疗，随后是受累部位放疗和PD-1抑制剂Tislelizumab维持治疗.经过多模式治疗，最终实现了长期部分缓解。缓解时间和总生存期达到22和32个月，分别。我们的病例表明，免疫靶向治疗与化疗和放疗相结合可能是HS的潜在治疗选择。
