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原发性颅内滑膜肉瘤

Primary Intracranial Synovial Sarcoma.

作者信息

Patel Mohit, Li Luyuan, Nguyen Ha Son, Doan Ninh, Sinson Grant, Mueller Wade

机构信息

Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

出版信息

Case Rep Neurol Med. 2016;2016:5608315. doi: 10.1155/2016/5608315. Epub 2016 May 9.

Abstract

Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.

摘要

背景。滑膜肉瘤是一种侵袭性软组织肉瘤,其组织学起源尚不明确。病理学上常表现为局限性疾病,尤其在膝关节和大腿周围的大关节附近。颅内疾病较为罕见,已报道为由滑膜肉瘤转移所致。我们报告一例无明显颅外原发性病变的病例,提示原发性颅内疾病;文献中尚未有此报道。病例描述。一名21岁男性,曾因非典型梭形细胞瘤接受右颅骨病变切除术,现出现头痛、步态不稳、左臂无力和左侧同向性偏盲。头部CT显示右顶叶出血性病变并伴有占位效应,需进行手术减压。组织病理学检查显示为滑膜肉瘤。荧光原位杂交(FISH)分析发现存在t(X;18)(p11.2;q11.2)染色体易位。正电子发射断层扫描(PET)未显示其他转移性疾病。他接受了立体定向放射治疗和辅助化疗。在2年的随访中,他无局部症状且未复发。结论。我们报告了首例已知的原发性颅内滑膜肉瘤病例。此外,我们强调颅内病变可能有出血倾向,需要紧急进行挽救生命的减压治疗。

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