Huynh Tridu R, Lu Conrad, Drazin Doniel, Lekovic Gregory
Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA.
Department of Pathology, St. Vincent Medical Center, Los Angeles, California, USA.
Surg Neurol Int. 2018 Sep 20;9:191. doi: 10.4103/sni.sni_422_17. eCollection 2018.
Myxopapillary ependymoma (MPE) with anaplastic features is extremely rare, with only three case reports in the literature.
We report the case of a MPE with anaplastic features in a 24-year-old female who presented with a dominant lumbar mass along with intracranial and sacral metastases. Upon gross total resection of the dominant tumor located at L2-L3, it appeared to arise from the filum terminale, and had a solid component in addition to soft or necrotic areas. Histologically, the tumor was composed of the two classic components of MPE: (1) low-grade ependymal cells surrounding blood vessels, producing the papillary appearance and (2) perivascular myxoid material between blood vessels and ependymal cells, creating the myxopapillary appearance. The high-grade anaplastic component showed hypercellularity, brisk mitotic rate, and vascular proliferation, with frequent pleomorphic cells and atypical mitotic figures. It was positive for vimentin and glial fibrillary acidic protein (GFAP); negative for epithelial membrane antigen (EMA), CAM5.2, creatine kinase 7 (CK7), CK20; and the MIB-1 index (Ki-67) was 8-38%. Ten months after initial resection, follow-up magnetic resonance imaging revealed new lesions in (1) the hypothalamus, (2) the left pons, and (3) the left medial temporal lobe, which were treated with radiosurgery. Eight months later (18 months from initial surgery), the patient underwent thoracic laminectomy for a large leptomeningeal metastasis at T6 and T8.
The present case of MPE with anaplastic features is the fourth case on record in the medical literature.
具有间变特征的黏液乳头型室管膜瘤（MPE）极为罕见，文献中仅有3例病例报告。
我们报告了1例24岁女性的具有间变特征的MPE病例，该患者表现为以腰椎肿块为主，伴有颅内和骶骨转移。在对位于L2 - L3的主要肿瘤进行全切除后，发现肿瘤似乎起源于终丝，除了柔软或坏死区域外还有实性成分。组织学上，肿瘤由MPE的两个经典成分组成：（1）围绕血管的低级别室管膜细胞，形成乳头状外观；（2）血管和室管膜细胞之间的血管周围黏液样物质，形成黏液乳头外观。高级别间变成分表现为细胞增多、有丝分裂活跃和血管增生，伴有频繁的多形性细胞和非典型有丝分裂象。波形蛋白和胶质纤维酸性蛋白（GFAP）呈阳性；上皮膜抗原（EMA）、CAM5.2、细胞角蛋白7（CK7）、CK20呈阴性；MIB - 1指数（Ki - 67）为8% - 38%。初次切除术后10个月，随访磁共振成像显示（1）下丘脑、（2）左侧脑桥和（3）左侧颞叶内侧出现新病变，对这些病变进行了放射外科治疗。8个月后（初次手术后18个月），患者因T6和T8处的大片软脑膜转移接受了胸椎椎板切除术。
本例具有间变特征的MPE是医学文献中记录的第4例病例。
