Department of Radiation Oncology, Albert-Einstein College of Medicine-Montefiore Medical Center, Bronx, NY.
Department of Pathology, Albert-Einstein College of Medicine-Montefiore Medical Center, Bronx, NY.
Int J Cancer. 2018 Feb 15;142(4):854-862. doi: 10.1002/ijc.31101. Epub 2017 Nov 14.
Myoepithelial tumors of the soft tissue are a rare tumor displaying myoepithelial elements and lacking obvious ductal differentiation. The rarity of these precludes any evidence-based consensus regarding optimal management. Nevertheless, the current approach to these lesions begins with amputation or complete excision. The efficacy of neoadjuvant or adjuvant radiation therapy or chemotherapy has not been established. Here, we present the first report to the authors' knowledge of neoadjuvant radiation therapy for the treatment of this rare soft tissue neoplasm and review the management and outcomes of published cases of myoepithelial carcinoma. A patient with a soft tissue myoepithelial carcinoma that declined both amputation and chemotherapy was treated with neoadjuvant radiation therapy and wide surgical excision followed by a brachytherapy boost to the resected tumor bed. Neoadjuvant radiation therapy resulted in an excellent response with extensive treatment-related changes consisting predominantly of fibrosis, hyalinization and hemorrhage and only 10% residual viable myoepithelial carcinoma present in the surgical specimen.
软组织肌上皮肿瘤是一种罕见的肿瘤，显示出肌上皮成分，缺乏明显的导管分化。这些肿瘤的罕见性排除了任何基于证据的共识，以确定最佳的管理方法。然而，目前对这些病变的处理方法是截肢或完全切除。新辅助或辅助放疗或化疗的疗效尚未确定。在这里，我们向作者报告了首例新辅助放疗治疗这种罕见的软组织肿瘤的病例，并回顾了已发表的肌上皮癌病例的治疗和结果。一名患有软组织肌上皮癌的患者拒绝截肢和化疗，接受了新辅助放疗和广泛的手术切除，然后对切除的肿瘤床进行近距离放射治疗。新辅助放疗产生了极好的反应，广泛的治疗相关变化主要包括纤维化、透明化和出血，在手术标本中仅残留 10%的存活肌上皮癌。
