Patel Mohit, Li Luyuan, Nguyen Ha Son, Doan Ninh, Sinson Grant, Mueller Wade
Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Case Rep Neurol Med. 2016;2016:5608315. doi: 10.1155/2016/5608315. Epub 2016 May 9.
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.
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。