Yoshiyama Tomomi, Munakata Wataru, Maeshima Akiko, Umesaki Arisa, Yamaga Hiroo, Nishiyama Akira, Nakajo Takato, Tanaka Yuko, Matsumoto Hiroaki, Terada Tomoaki
Department of Neurosurgery, Showa University Fujigaoka Hospital.
No Shinkei Geka. 2020 Sep;48(9):809-818. doi: 10.11477/mf.1436204277.
Erdheim-Chester disease(ECD)is a rare type of non-Langerhans histiocytosis. We report a surgical case of ECD with multiple lesions at the falx cerebri, tentorium cerebelli, and in the suprasellar region, with a literature review.
A 70-year-old woman presented with frequent falling and difficulty in standing. Her past medical history revealed ovarian cystectomy, transient thrombocytopenia, hypertension, left lower leg pain, and overactive bladder. Her head CT and MRI findings revealed well-defined mass lesions, suspected of meningioma, at the falx cerebri and tentorium cerebelli. Craniotomy and near total resection of the tumor at the falx cerebri was performed, leaving a hard portion of the tumor on the right falx. Intraoperative findings showed a solid and hard tumor, which was extremely difficult to decompress. Although the histopathological diagnosis was originally a metaplastic meningioma, considering her complaints of lower leg pain, we suspected ECD and performed a right tibial biopsy. The right tibial biopsy revealed ECD. Twenty-two months after the operation, the patient exhibited a marked enlargement of the tentorium lesion and a new lesion in the suprasellar region. Resection of the tentorial lesion was performed. The second intraoperative findings were similar to those of the first. The histopathological diagnosis of the tentorial lesion was ECD. After the surgeries, steroid therapy and radiation therapy were performed, but only with temporary improvement.
ECD is a rare disease; therefore, accumulation of clinical data to establish its treatment is necessary.
厄尔德海姆-切斯特病（ECD）是一种罕见的非朗格汉斯组织细胞增多症。我们报告一例ECD手术病例，病变位于大脑镰、小脑幕及鞍上区域，同时进行文献复习。
一名70岁女性，表现为频繁跌倒及站立困难。其既往病史包括卵巢囊肿切除术、短暂性血小板减少症、高血压、左下肢疼痛及膀胱过度活动症。头部CT和MRI检查发现大脑镰和小脑幕有边界清晰的肿块病变，怀疑为脑膜瘤。对大脑镰处肿瘤进行了开颅手术并近全切除，右侧大脑镰处残留部分坚硬肿瘤。术中发现肿瘤质地硬且实性，极难减压。虽然最初组织病理学诊断为化生型脑膜瘤，但考虑到患者有下肢疼痛症状，我们怀疑为ECD并进行了右胫骨活检。右胫骨活检结果显示为ECD。术后22个月，患者小脑幕病变明显增大，鞍上区域出现新病变。对小脑幕病变进行了切除。第二次术中所见与第一次相似。小脑幕病变的组织病理学诊断为ECD。手术后进行了类固醇治疗和放射治疗，但仅取得了暂时的改善。
ECD是一种罕见疾病；因此，积累临床数据以确定其治疗方法很有必要。
