Zenmyo M, Komiya S, Hamada T, Hiraoka K, Nagata K, Tsuji S, Hashimoto H, Inoue A
Department of Orthopaedic Surgery, Kurume University School of Medicine, Japan.
Spine (Phila Pa 1976). 2001 Feb 1;26(3):310-3. doi: 10.1097/00007632-200102010-00018.
A case report.
To illustrate a rare case of synovial sarcoma arising within a peripheral nerve.
A synovial sarcoma arising within a peripheral nerve is very unusual. Only five cases of primary synovial sarcoma within a peripheral nerve have been reported. This is the first case with involvement of the nerve root. The authors diagnosed the tumor arising within the S1 nerve root as synovial sarcoma using cytogenetic analysis that detected the chimeric SYT/SSX gene.
In addition to the immunohistochemical study, a reverse transcription-polymerase chain reaction (RT-PCR) assay was conducted for the SYT-SS10 fusion gene using archival formalin-fixed paraffin-embedded tumor specimens.
Computed tomography scan, magnetic resonance imaging performed before surgery, and the intraoperative findings showed that the tumor was embedded within the S1 nerve root. Although the histologic findings were suggestive of a malignant peripheral nerve sheath tumor, the results of the cytologic study confirmed its diagnosis of synovial sarcoma.
Primary intraneural synovial sarcoma, although rare, must be distinguished from malignant peripheral nerve sheath tumor. The molecular assay of the detection of the SYT/SSX fusion gene is useful to make a definite diagnosis of monophasic synovial sarcoma.
病例报告。
阐述1例发生于周围神经的滑膜肉瘤罕见病例。
发生于周围神经的滑膜肉瘤非常罕见。仅报道过5例原发性周围神经滑膜肉瘤。这是第1例累及神经根的病例。作者通过检测到嵌合性SYT/SSX基因的细胞遗传学分析,将起源于S1神经根的肿瘤诊断为滑膜肉瘤。
除免疫组化研究外,还使用存档的福尔马林固定石蜡包埋肿瘤标本对SYT-SS10融合基因进行逆转录聚合酶链反应(RT-PCR)检测。
术前计算机断层扫描、磁共振成像及术中所见显示肿瘤包埋于S1神经根内。尽管组织学表现提示为恶性周围神经鞘瘤,但细胞学研究结果证实其诊断为滑膜肉瘤。
原发性神经内滑膜肉瘤虽罕见,但必须与恶性周围神经鞘瘤相鉴别。检测SYT/SSX融合基因的分子检测方法有助于明确诊断单相性滑膜肉瘤。