骨内梭形细胞横纹肌肉瘤伴::融合——附大量临床随访和文献复习的病例报告。
Intraosseous Spindle Cell Rhabdomyosarcoma with :: Fusion - Case Report with Substantial Clinical Follow-up and Review of the Literature.
机构信息
Department of Pathology and Laboratory Medicine, Oregon Health & Science University, Portland, OR, USA.
Department of Orthopaedics and Rehabilitation, Oregon Health & Science University, Portland, OR, USA.
出版信息
Cancer Invest. 2023 Sep;41(8):704-712. doi: 10.1080/07357907.2023.2255668. Epub 2023 Sep 6.
Spindle cell/sclerosing rhabdomyosarcoma (SSRMS) is a clinicopathologically and molecularly heterogeneous disease. Gene fusions have been identified in intraosseous SSRMS, consisting predominantly of /:: and ::. The former often follow an aggressive clinical course; there is limited clinical follow-up available for the latter. We report here a new case of the very rare intraosseous SSRMS with :: gene fusion and include the detailed treatment course and 52 months of clinical follow-up. SSRMS with :: gene fusion appears biologically distinct from other intraosseous SSRMS, following a course characterized by local recurrence with rare reports of metastasis to date.
纺锤形细胞/硬化性横纹肌肉瘤（SSRMS）是一种临床病理和分子上具有异质性的疾病。在骨内 SSRMS 中已经鉴定出基因融合，主要由 /:: 和 :: 组成。前者通常具有侵袭性的临床病程；后者的临床随访资料有限。我们在此报告一例非常罕见的伴有 :: 基因融合的骨内 SSRMS 新病例，并包括详细的治疗过程和 52 个月的临床随访。伴有 :: 基因融合的 SSRMS 在生物学上与其他骨内 SSRMS 不同，其特征为局部复发，迄今为止鲜有转移的报道。
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