Li Ting, Xiao Shengda, Sun Mingfang, Wu Zhiwei, Guo Jing, Xing Yun, He Zheng, Wang Yibao, Wang Yong
Department of Neurosurgery, The First Hospital of China Medical University, Shenyang, Liaoning Province, China.
Department of Pathology, The First Hospital of China Medical University, Shenyang, Liaoning Province, China.
J Neurosurg Case Lessons. 2025 Mar 3;9(9). doi: 10.3171/CASE24437.
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon types of soft tissue sarcomas. The occurrence of intracranial MPNSTs in the sellar region is exceedingly rare.
A 37-year-old female, who had undergone transnasal surgery for a pituitary adenoma followed by adjuvant stereotactic radiotherapy 10 years earlier, was diagnosed with an intracranial MPNST in the sellar region. A second transnasal procedure successfully removed the lesion and was followed by proton therapy. Three months postoperatively, spontaneous cerebrospinal fluid (CSF) rhinorrhea occurred and was repaired through a third transnasal surgery. A CSF test revealed the presence of suspicious tumor cells. Subsequently, acute hydrocephalus developed and was urgently managed with a ventriculoperitoneal shunt. About 2 months later, the patient died due to intraspinal metastasis.
Pathologically ambiguous lesions in the sellar region require meticulous observation, as MPNST can be erroneously diagnosed as a pituitary adenoma. Prompt surgical intervention provides the best opportunity for achieving complete resection, which remains the most effective treatment for MPNST. In addition to hematogenous metastasis, MPNST can also disseminate and implant via CSF, leading to recurrence. Acute hydrocephalus or intraspinal metastasis can significantly reduce overall survival. https://thejns.org/doi/10.3171/CASE24437.
恶性外周神经鞘瘤（MPNSTs）是软组织肉瘤中较为罕见的类型。鞍区发生颅内MPNSTs极为罕见。
一名37岁女性，10年前因垂体腺瘤接受了经鼻手术并辅助立体定向放疗，现被诊断为鞍区颅内MPNST。第二次经鼻手术成功切除了病变，随后进行了质子治疗。术后3个月，出现自发性脑脊液鼻漏，通过第三次经鼻手术修复。脑脊液检查发现可疑肿瘤细胞。随后，急性脑积水发生，紧急进行了脑室腹腔分流术。约2个月后，患者因脊髓转移死亡。
鞍区病理诊断不明确的病变需要仔细观察，因为MPNST可能被误诊为垂体腺瘤。及时的手术干预为实现完全切除提供了最佳机会，而完全切除仍是MPNST最有效的治疗方法。除血行转移外，MPNST还可通过脑脊液播散和种植，导致复发。急性脑积水或脊髓转移可显著降低总体生存率。https://thejns.org/doi/10.3171/CASE24437
