■滑膜细胞肉瘤(SS)是一种极其罕见的间充质恶性肿瘤,占所有软组织肉瘤的近10%。这些高级软组织肉瘤通常出现在下肢的关节旁区域。然而,已在异常位置描述了15%的滑膜肉瘤,包括头部,脖子,和树干。在这里,我们描述了第十二例原发性甲状腺滑膜细胞肉瘤并进行了文献复习。
方法:一名43岁女性患者在过去5个月中一直表现为进行性颈部肿块。近2个月前她出现吞咽困难和呼吸困难,没有声音嘶哑的迹象,和减肥。超声检查显示异质性,高血管化甲状腺肿块。甲状腺全切除术后,免疫组织化学有利于甲状腺原发性滑膜细胞肉瘤。通过使用RT-聚合酶链反应方法对SYT-SSX融合基因转录物进行分子遗传学分析来确认诊断。临床讨论:原发性甲状腺SVS是一种极其罕见的恶性肿瘤,生物学行为较差。SVS以其在治疗几年后局部和远端复发的趋势而闻名。基于间充质和/或上皮组分的存在,SS可以分为单相或双相的两种亚型。因此,SS最准确的诊断工具被认为是SYT/SSX融合转录物的分子遗传分析。
结论:此处,我们报道了一例极为罕见的甲状腺SVS病例。这些高级软组织肉瘤主要表现为无症状的快速增长的颈部肿块。这种疾病的非特异性临床表现和极端罕见,使甲状腺SVS的诊断非常具有挑战性。由于数据匮乏,没有足够的证据来确定可靠的死亡率。然而,甲状腺SVS的预后似乎不利。
UNASSIGNED: Synovial cell sarcoma (SS) is an extremely rare mesenchymal malignancy, representing nearly 10% of all soft-tissue sarcomas. These high-grade soft tissue sarcomas commonly arise in the para-articular regions of lower extremities. However, 15% of Synovial sarcomas has been described at Unusual locations, including head, neck, and trunk. Herein, we describe the twelfth case of primary synovial cell sarcoma of thyroid with a literature review.
METHODS: A 43-year-old woman presented with complaint of a progressive neck mass for the last five-months. She developed with dysphagia and dyspnea nearly 2 months prior, without signs of hoarseness, and weight loss. Ultrasonography in which revealed a heterogeneous, hypervascularized thyroid mass. After total thyroidectomy immunohistochemistry was in favor of primary synovial cell sarcoma of thyroid. The diagnosis was confirmed via Molecular genetic analysis of the SYT-SSX fusion gene transcript using the RT- polymerase chain reaction method. Clinical Discussion: Primary thyroid SVS is an extremely rare malignancy with poor biological behavior. SVS has been known for its tendency to local and distal re-occurrence after a few years of treatment. SS can be classified into two subtypes of monophasic or biphasic based on the presence of mesenchymal and/or epithelial components. Accordingly, the most accurate diagnostic tool for SS is considered to be molecular genetic analysis for SYT/SSX fusion transcript.
CONCLUSIONS: Herein, we reported an extremely rare case of SVS of thyroid gland. These high-grade soft tissue sarcomas mainly present with an asymptomatic rapid growing neck mass. Unspecific clinical presentations and extreme rarity of this disorder, make the diagnosis of thyroid SVS very challenging. Due to paucity of data, there is not enough evidence to establish a reliable mortality rate. However, the prognosis of thyroid SVS seems unfavorable.