PDF(Pubmed)
Abstract：
Soft tissue myoepithelial carcinoma (MEC) is an extremely rare mesenchymal tumor that has a poor prognosis unless complete surgical resection is achieved. The present study reported a case of a 38-year-old woman with a tumor in the left paraspinal region at L2 to L3 with vertebral destruction. MEC was diagnosed based on molecular pathological examination of a biopsy specimen. Because curative surgery was expected to be difficult, a combination of chemotherapy with doxorubicin and ifosfamide and proton beam therapy as local therapy was performed, resulting in long-term survival for at least 7.8 years. To the best of our knowledge, this is the first case of soft tissue MEC for which classical cytotoxic chemotherapy and proton beam therapy were effective. Although surgical resection with negative margins is the mainstay of treatment for MEC, adequate doxorubicin-based systemic therapy and high-dose radiation therapy may be a feasible alternative in patients with unresectable or advanced MEC. Future studies on the relationship between molecular pathological features, including biomarkers, and the selection of therapeutic agents are warranted.
摘要：
软组织肌上皮癌（MEC）是一种极其罕见的间充质肿瘤，除非获得完整的手术切除，否则预后较差。本研究报告了一例38岁的女性，在L2至L3的左侧椎旁区域患有椎体破坏。根据活检标本的分子病理学检查诊断MEC。因为预期治愈性手术会很困难，进行了联合化疗与阿霉素和异环磷酰胺和质子束治疗作为局部治疗，导致至少7.8年的长期生存。据我们所知,这是第一例软组织MEC,经典细胞毒性化疗和质子束治疗有效.尽管切缘阴性的手术切除是MEC的主要治疗方法，对于不可切除或晚期MEC患者,适当的基于多柔比星的全身治疗和大剂量放射治疗可能是可行的替代方案.分子病理特征之间关系的未来研究，包括生物标志物,和治疗剂的选择是必要的。
