滑膜肉瘤(SS)是一种侵袭性软组织肿瘤,主要发生在年轻人身上。SS几乎可以出现在身体的任何部位,尤其是下肢。头颈部SS占所有SS的1.9-3.5%,然而,这种肿块在喉部非常罕见。我们报告了一例喉部双相SS。一个14岁的男孩出现声音嘶哑,没有明显的诱因,呼吸困难,发烧,咳嗽,咯血,咽部异物感,吞咽困难,食欲不振等症状近一个月前。该患者接受了部分喉切除术,并对有自由边缘的肿瘤进行了广泛的手术切除。肿瘤标本的病理学检查显示出包裹且牢固的肿瘤病变。灰白色至深褐色肿块8×6×4cm,产生于阿利会厌褶皱的左边。组织学检查显示了短梭形细胞和上皮样细胞的双相模式等特征性组织形态学特征,包括腺体分化。免疫组织化学,波形蛋白染色,bcl-2和钙蛋白在梭形肿瘤细胞中通常呈阳性。CK染色在上皮样肿瘤细胞中通常是阳性的。EMA染色,CD99和TLE-1在上皮样和梭形肿瘤细胞中通常是阳性的。在这种情况下,通过FISH检测到来自染色体易位的SYT-SSX融合基因的存在。术后两个月,患者接受了局部放疗。联合治疗可能是有效的,并且在18个月的随访后,患者在放射学和临床检查中存活,没有肿瘤复发。
Synovial sarcoma (SS) is an aggressive soft tissue tumor, which occurs predominantly in young adults. The SS can arise in almost any part of the body, especially in the lower extremity. The SS of head and neck accounts for 1.9-3.5% of all SS, however, the mass appears very extremely rare in the larynx. We report a
case of a
biphasic SS in the larynx. A 14-year-old boy appeared without apparent inducement with hoarseness, dyspnea, fever, cough, hemoptysis, pharyngeal foreign body sensation, dysphagia, odynophagia and other symptoms since nearly one month ago. The patient underwent partial laryngectomy and performed a wide surgical excision of the tumor with a free margin. Pathological examinations of the tumor specimen revealed an encapsulated and firm tumor lesion. The grayish-white to dark-brown mass was 8×6×4 cm, arising from the left of aryepiglottic fold. Histological examination showed the characteristic histomorphological features such as
biphasic pattern of short spindle cells and epithelioid cells, including glandular differentiation. Immunohistochemically, staining for vimentin, bcl-2 and calponin were typically positive in the spindle tumor cells. Staining for CK was typically positive in the epithelioidtumor cells. Staining for EMA, CD99 and TLE-1 were typically positive in both the epithelioid and spindle tumor cells. The presence of SYT-SSX fusion gene from chromosomal translocation was detected by FISH in this
case. Two months postoperatively, the patient received local radiotherapy. Combined treatment may be effective and the patient is alive without tumor recurrence in radiological and clinical examination after 18 months of follow-up.