PDF(Pubmed)
Abstract：
Primary intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are rare yet highly aggressive neoplasms originating from peripheral nerves. Typically manifesting as soft tissue masses accompanied by pain or functional impairment, these tumors pose significant challenges in management. Surgical intervention remains the cornerstone of treatment for patients with MPNST lacking distant metastasis, with generally modest success rates. In cases of recurrence and metastasis, the pursuit of effective systemic therapies has been a focus of clinical investigation. Herein, we present a case study involving an elderly female patient with refractory MPNST. In light of surgical limitations, a multimodal therapeutic approach combining chemotherapy, denosumab, and subsequent administration of anlotinib was pursued following collaborative consultation. This regimen yielded noteworthy clinical benefits, exemplifying a promising avenue in the management of challenging MPNST cases.
摘要：
原发性骨内恶性周围神经鞘瘤（MPNSTs）是罕见的，但起源于周围神经的高度侵袭性肿瘤。通常表现为伴有疼痛或功能障碍的软组织肿块，这些肿瘤在管理方面构成了重大挑战.手术干预仍然是治疗缺乏远处转移的MPNST患者的基石。成功率一般不高。在复发和转移的情况下，寻求有效的系统治疗一直是临床研究的重点.在这里，我们提供了一项涉及难治性MPNST老年女性患者的病例研究.鉴于手术的局限性，结合化疗的多模式治疗方法，denosumab,随后的安洛替尼的给药是在合作协商后进行的.该方案产生了值得注意的临床益处，为处理具有挑战性的MPNST病例提供了一条有希望的途径。
