Wigand Marlene C, Hoffmann Thomas K, Barth Thomas F E, Veit Johannes
Department of Otolaryngology, Head and Neck Surgery, University Hospital Ulm, Frauensteige 12, 89075 Ulm, Germany.
Department of Otolaryngology, Head and Neck Surgery, University Hospital Ulm, Frauensteige 12, 89075 Ulm, Germany.
Auris Nasus Larynx. 2018 Jun;45(3):617-621. doi: 10.1016/j.anl.2017.06.007. Epub 2017 Jul 6.
Synovial sarcomas are rare malignant tumors supposed to arise from pluripotent mesenchymal stem cells predominantly affecting the deep soft tissue of the lower and upper extremities in young adults. The occurrence of this tumor entity in the head and neck is very uncommon and hence, timely diagnosis and treatment of synovial sarcoma in this region remain a challenge. We describe the clinical and molecular pathological features of a biphasic synovial sarcoma of the epiglottis, a site where the primary manifestation of this tumor entity has not been documented to date. With this background the existing literature is being reviewed. Whilst wide excision of synovial sarcoma is considered as the primary mainstay of therapy, (neo)adjuvant treatment strategies have been proposed to increase local control and prolong disease-free survival.
滑膜肉瘤是一种罕见的恶性肿瘤,被认为起源于多能间充质干细胞,主要影响年轻成年人的上下肢深部软组织。这种肿瘤实体在头颈部的发生非常罕见,因此,该区域滑膜肉瘤的及时诊断和治疗仍然是一个挑战。我们描述了一例会厌双相滑膜肉瘤的临床和分子病理特征,该部位迄今为止尚未见该肿瘤实体的主要表现的报道。在此背景下,对现有文献进行了综述。虽然广泛切除滑膜肉瘤被认为是主要的治疗方法,但也有人提出(新)辅助治疗策略以提高局部控制率并延长无病生存期。