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甲状腺原发性滑膜肉瘤:一例报告并文献复习

Primary synovial sarcoma of thyroid gland: A case report and review of literature.

作者信息

Seyed-Alagheband Seyed-Ahmad, Sharifian Masoud, Adeli Omid-Ali, Sohooli Maryam, Shekouhi Ramin

机构信息

Department of Surgery, Lorestan University of Medical Sciences, Lorestan, Khorramabad, Iran.

Department of Pathology, Lorestan University of Medical Sciences, Lorestan, Khorramabad, Iran.

出版信息

Int J Surg Case Rep. 2021 Aug;85:106245. doi: 10.1016/j.ijscr.2021.106245. Epub 2021 Jul 26.

Abstract

INTRODUCTION AND IMPORTANCE

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.

CASE PRESENTATION

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.

CONCLUSION

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.

摘要

引言与重要性

滑膜肉瘤(SS)是一种极其罕见的间叶性恶性肿瘤,占所有软组织肉瘤的近10%。这些高级别软组织肉瘤通常发生在下肢的关节周围区域。然而,15%的滑膜肉瘤发生在不寻常的部位,包括头、颈和躯干。在此,我们报告第十二例原发性甲状腺滑膜肉瘤病例并进行文献复习。

病例介绍

一名43岁女性因过去五个月颈部肿块逐渐增大前来就诊。她在近2个月前出现吞咽困难和呼吸困难,无声音嘶哑及体重减轻症状。超声检查显示甲状腺肿块不均匀、血运丰富。全甲状腺切除术后免疫组化结果支持原发性甲状腺滑膜肉瘤。通过逆转录聚合酶链反应方法对SYT-SSX融合基因转录本进行分子遗传学分析确诊。

临床讨论

原发性甲状腺滑膜肉瘤是一种极其罕见的恶性肿瘤,生物学行为较差。滑膜肉瘤以治疗几年后局部和远处复发倾向而闻名。根据是否存在间叶和/或上皮成分,滑膜肉瘤可分为单相或双相两种亚型。因此,滑膜肉瘤最准确的诊断工具被认为是对SYT/SSX融合转录本进行分子遗传学分析。

结论

在此,我们报告了一例极其罕见的甲状腺滑膜肉瘤病例。这些高级别软组织肉瘤主要表现为无症状的颈部快速生长肿块。这种疾病非特异性的临床表现和极端罕见性使得甲状腺滑膜肉瘤的诊断极具挑战性。由于数据匮乏,没有足够证据确定可靠的死亡率。然而,甲状腺滑膜肉瘤的预后似乎不佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f9f6/8329501/0bb8bc4708d6/gr1.jpg

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