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颅内骨外硬脑膜尤文肉瘤伴液血比水平:一种罕见肿瘤的影像学表现。

Intracranial extraosseous dural-based Ewing's sarcoma with fluid-haematocrit levels: imaging findings of a rare tumour.

机构信息

Radiodiagnosis, All India Institute of Medical Sciences Bhubaneswar, Bhubaneswar, Odisha, India

Radiodiagnosis, All India Institute of Medical Sciences Bhubaneswar, Bhubaneswar, Odisha, India.

出版信息

BMJ Case Rep. 2022 Jun 14;15(6):e247056. doi: 10.1136/bcr-2021-247056.

DOI:10.1136/bcr-2021-247056
PMID:35701017
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9198795/
Abstract

Extraosseous dural-based primary Ewing's sarcoma of the central nervous system is a rare tumour posing a diagnostic challenge. On cross-sectional radiological imaging, the lesion has an extra-axial location with heterogeneous appearance. These lesions are usually multicystic with internal haemorrhage causing fluid-haematocrit levels. It mimics conditions like an aneurysmal bone cyst, microcystic meningioma, telangiectatic osteosarcoma or cystic metastasis. Exclusion of primary Ewing's sarcoma or malignancy elsewhere in the body is required to rule out a secondary. Surgery along with adjuvant chemotherapy and focal radiotherapy is the preferred mode of treatment. Due to the presence of non-specific small round blue cells on H&E stain, these tumours are also confused with lymphoma, osteosarcoma, rhabdomyosarcoma, Merkel cell carcinoma, ependymoblastoma and neuroendocrine carcinoma. Immunohistochemistry provides a definitive diagnosis. A high degree of suspicion in the preoperative scans is crucial for prognostication and early management of this aggressive tumour leading to improved patient survival.

摘要

中枢神经系统骨外硬脑膜原发性尤文肉瘤是一种罕见的肿瘤,具有诊断挑战性。在横断面影像学检查中,病变具有颅外位置和异质性表现。这些病变通常为多房性,伴有内部出血导致液体-血细胞比容水平升高。它类似于动脉瘤样骨囊肿、微囊脑膜瘤、毛细血管扩张性骨肉瘤或囊性转移瘤等疾病。为了排除继发性疾病,需要排除原发性尤文肉瘤或身体其他部位的恶性肿瘤。手术联合辅助化疗和局部放疗是首选的治疗方式。由于 H&E 染色存在非特异性小圆蓝细胞,这些肿瘤也与淋巴瘤、骨肉瘤、横纹肌肉瘤、默克尔细胞癌、室管膜母细胞瘤和神经内分泌癌混淆。免疫组织化学提供了明确的诊断。术前扫描中高度怀疑对于这种侵袭性肿瘤的预后和早期管理至关重要,可提高患者的生存率。