头颈部低度恶性肌纤维母细胞肉瘤二例分析

doi:10.6040/j.issn.1673-3770.0.2017.068

摘要/Abstract

摘要： 目的探讨头颈部低度恶性肌纤维母细胞肉瘤(LGMS)的临床表现、影像学表现、病理表现、治疗方法及预后。方法回顾性分析2例头颈部LGMS患者的临床资料并复习相关文献,总结其临床表现、影像学表现、病理表现及治疗方法、预后情况。结果头颈部LGMS的临床表现取决于肿瘤的部位,声门区的LGMS以声音嘶哑为首发症状,随着肿瘤增大可出现呼吸困难,颈部的LGMS表现为无痛性肿块。肿瘤表面光滑,似有被膜,界不清,基底广泛,表面可有坏死。CT表现为均匀或不均匀的中等密度肿瘤,肿瘤内可见钙化,偶可破坏骨质,增强后呈现轻度均匀强化或环形强化。MRI扫描T₁W2呈低信号或等限号,T₂W1呈均匀或不均匀高信号,肿瘤内有条索信号影,增强后肿瘤实质呈周边明显强化表现。光镜下见肿瘤细胞由梭形细胞和小多角形细胞组成,呈束状、疏松状、编织状排列,弥漫性浸润性生长,可侵犯黏膜鳞状上皮、肌肉,肿瘤细胞界限不清,纺锤形,核为细长型,核增大、深染、不规则。免疫组化见Vimentin、SMA、desmin、actin等阳性表达,S-100等阴性表达。治疗方法以广泛手术切除为主,术后随访12~23个月,均无复发及转移。结论头颈部LGMS临床及影像学表现无特异性,病理诊断必须结合免疫组织化学检查,广泛手术切除是最有效的治疗方法,肿瘤对放化疗不敏感,肿瘤的复发与是否切除彻底有关。

关键词: 肿瘤, 低度恶性肌纤维母细胞肉瘤, 临床病理, 影像学, 头颈

Abstract: Objective To investigate the clinical characteristics, imaging features, diagnosis, and treatment of low-grade myofibroblastic sarcoma(LGMS)of the head and neck. Methods A retrospective study of 2 patients with LGMS of the head and neck was performed, and previous cases of LGMS affecting the head and neck reported in the literature were reviewed. The clinic-pathological characteristics, imaging features, diagnosis and treatment were recorded. Results The symptoms of LGMS depend on the location of the tumor. Hoarseness is the first symptom of LGMS affecting vocal cord, along with an increase in dyspnea. LGMS of the neck manifests as a painless mass. It has been observed that LGMS of neck manifests as a painless mass with smooth surgace, capsule, and wide base, while its interface is unclear. Computed tomography demonstrated moderate or inhomogeneous density of tumor necrosis. Moreover, the tumor is characterized by calcification, or even bone destruction, and slight uniform ring enhancement. T₁W2 weighted MRI showed the tumor with low signal or limited numbers, while in T₂W1 weighted phase, homogeneous or inhomogeneous high signal was observed. The tumor consisted of spindle cells and small polygonal cells arranged in bundles, loose, and braided arrangement. The neoplasm showed diffuse infiltrative growth, and could invade the mucosa squamous epithelium. Tumor cells had unclear, fusiform shape, and the nucleus was slender, enlarged, irregular, and hyperchromatic. On immunohistochemistry analysis, the tumor cells were positive for vimentin, SMA, desmin, and actin, but negative for S-100. Treatment methods mainly focus on extensive surgical resection, and in the 2 cases that were followed up for 12-23 months, no recurrence and metastasis were observed. Conclusion LGMS of the head and neck is a very rare tumor, which lacks easily recognizable clinical and imaging manifestations. Pathological diagnosis must be combined with immunohistochemical examination, while extensive resection is the most effective method of treatment. Specifically, a thoroughgoing resection and negative margin are the keys to success of surgery. LGMS of the head and neck is not sensitive to chemotherapy and radiotherapy. Disease prognosis is good, while recurrence depends on complete resection.

Key words: Neoplasm, Pathology, Head and neck, Low-grade myofibroblastic sarcoma, Iconography

中图分类号:

R767.04

白永,尹迎春,韩学锋,尹兆富,李学忠. 头颈部低度恶性肌纤维母细胞肉瘤二例分析[J]. 山东大学耳鼻喉眼学报, 2018, 32(4): 58-61.

BAI Yong, YIN Yingchun, HAN Xuefeng, YIN Zhaofu, LI Xuezhong. Clinical analysis of low-grade myofibroblastic sarcoma of the head and neck[J]. J Otolaryngol Ophthalmol Shandong Univ, 2018, 32(4): 58-61.

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