Nagase Takayuki, Ishida Joji, Sasada Susumu, Sasaki Tatsuya, Otani Yoshihiro, Yabuno Satoru, Fujii Kentaro, Uneda Atsuhito, Yasuhara Takao, Date Isao
Department of Neurological Surgery, Okayama University Faculty of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Okayama, Japan.
Division of Brain Tumor Translational Research, National Cancer Center Research Institute, Tokyo, Japan.
NMC Case Rep J. 2023 Mar 24;10:75-80. doi: 10.2176/jns-nmc.2022-0159. eCollection 2023.
Diffusely infiltrative midline gliomas are known to have a poor prognosis. The standard treatment for typical diffuse midline glioma in the pons is local radiotherapy as surgical resection is inappropriate. This case reports a brainstem glioma in which stereotactic biopsy and foramen magnum decompression were concomitantly performed to confirm the diagnosis and improve symptoms. A 23-year-old woman was referred to our department with a chief complaint of headache for six months. Magnetic resonance imaging (MRI) showed diffuse T2 hyperintense swelling of the brainstem with the pons as the main locus. Enlargement of the lateral ventricles was observed because of cerebrospinal fluid obstruction out of the posterior fossa. This was atypical for a diffuse midline glioma in terms of the longstanding slow progression of symptoms and patient age. Stereotactic biopsy was performed for diagnosis, and foramen magnum decompression (FMD) was concomitantly performed to treat the obstructive hydrocephalus. The histological diagnosis was astrocytoma, IDH-mutant. Post-surgery, the patient's symptoms were relieved, and she was discharged on the fifth day after surgery. The hydrocephalus was resolved, and the patient returned to normal life without any symptoms. The tumor size follow-up with MRI demonstrated no marked change for 12 months. Even though diffuse midline glioma is considered to have a poor prognosis, clinicians should contemplate if it is atypical. In atypical cases like the one described herein, surgical treatment may contribute to pathological diagnosis and symptom improvement.
弥漫性浸润性中线胶质瘤预后较差。脑桥典型弥漫性中线胶质瘤的标准治疗方法是局部放疗,因为手术切除并不合适。本病例报告了一例脑干胶质瘤,同时进行了立体定向活检和枕骨大孔减压术以明确诊断并改善症状。一名23岁女性因头痛6个月为主诉转诊至我科。磁共振成像(MRI)显示脑干弥漫性T2高信号肿胀,主要部位在脑桥。由于后颅窝脑脊液梗阻,观察到侧脑室扩大。就症状长期缓慢进展和患者年龄而言,这对于弥漫性中线胶质瘤来说并不典型。进行立体定向活检以明确诊断,并同时进行枕骨大孔减压术(FMD)以治疗梗阻性脑积水。组织学诊断为异柠檬酸脱氢酶(IDH)突变型星形细胞瘤。术后患者症状缓解,术后第5天出院。脑积水得到解决,患者恢复正常生活,无任何症状。MRI随访肿瘤大小12个月无明显变化。尽管弥漫性中线胶质瘤被认为预后较差,但临床医生应考虑其是否不典型。在本文所述的非典型病例中,手术治疗可能有助于病理诊断和症状改善。