Al Hayek Mohammad, Yousfan Abdulmajeed
Faculty of Medicine, Damascus University, Damascus, Syrian Arab Republic.
Department of Otolaryngology, Al Mouwasat University Hospital, Damascus University, Damascus, Syrian Arab Republic.
Int J Surg Case Rep. 2023 Apr;105:107998. doi: 10.1016/j.ijscr.2023.107998. Epub 2023 Mar 21.
Synovial sarcoma is a malignant neoplasm that arises from soft tissue and makes up 5 % to 10 % of all types of sarcoma. It is most common between the ages of 15 and 40; it typically develops in the lower extremities; just 3 % to 10 % of cases arise in the head and neck. The usual main areas in the head and neck are the parapharyngeal, hypopharyngeal, and paraspinal.
An 18-year-old woman presented with a painful mass in the left pre-auricular area.
Magnetic resonance imaging showed a well-defined lobular mass localized superior and anterior to the left ear. Incisional biopsy showed spindle cell sarcoma. A preauricular incision was done to remove the tumor with the superficial lobe of the parotid gland during the procedure, and histological examination revealed a high-grade spindle cell sarcoma the differential diagnosis included a monophasic synovial sarcoma. Immunohistochemistry was done for a full evaluation and the panel supported diagnosing a monophasic synovial sarcoma.
Synovial sarcoma is a malignant tumor that is rare to develop in the temporomandibular region, which makes it a significant challenge to diagnose and differentiate it from other lesions, it should be considered in all patients with a mass in this region. The cornerstone to identify synovial sarcoma is Immunohistochemistry (IHC), and molecular genetic analyses. Total surgical excision, with or without radiation and chemotherapy, is currently the best option for treatment. We present a review of the literature after the case presentation.
滑膜肉瘤是一种起源于软组织的恶性肿瘤,占所有肉瘤类型的5%至10%。它在15岁至40岁之间最为常见;通常发生在下肢;仅3%至10%的病例发生在头颈部。头颈部常见的主要部位是咽旁、下咽和椎旁。
一名18岁女性,左耳前区出现疼痛性肿块。
磁共振成像显示左耳上方和前方有一个边界清晰的小叶状肿块。切开活检显示为梭形细胞肉瘤。手术过程中通过耳前切口切除肿瘤及腮腺浅叶,组织学检查显示为高级别梭形细胞肉瘤,鉴别诊断包括单相滑膜肉瘤。进行免疫组织化学检查以全面评估,结果支持诊断为单相滑膜肉瘤。
滑膜肉瘤是一种罕见于颞下颌区域发生的恶性肿瘤,这使其诊断及与其他病变的鉴别成为一项重大挑战,对于该区域有肿块的所有患者均应考虑此病。识别滑膜肉瘤的基石是免疫组织化学(IHC)和分子遗传学分析。目前,无论是否进行放疗和化疗,手术全切是最佳治疗选择。病例介绍后我们对文献进行了综述。