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目的探讨原发于甲状腺罕见的黏液纤维肉瘤(MFS)的临床病理特征、诊断及鉴别诊断。方法对1例甲状腺原发性黏液纤维肉瘤的临床表现、组织学形态及免疫组化进行分析,并复习相关文献。结果患者男性,71岁。颈部增强CT及彩超示双侧甲状腺内占位性病变。光镜下肿瘤由纤维结缔组织分隔呈多结节状,肿瘤细胞为梭形或星芒状,呈紊乱或条束状排列于黏液样间质中,可见具有形态学特征的多空泡假脂肪母细胞,以及间质中曲线状或弧线状血管。免疫组化示vimentin(+),CKpan、TG、TTF1、PAX-8、calcitonin、Syn、Cg A、CD34、S-100蛋白、SMA、desmin、EMA和CD68均(-),Ki-67阳性率10%~15%。结论甲状腺原发性黏液纤维肉瘤极其罕见,临床病理诊断较困难,应排除转移性软组织肿瘤、甲状腺常见肿瘤及其他类似肿瘤,需结合病理形态和免疫组化标记确诊。该肿瘤的治疗方式为手术扩大切除并术后辅助放疗。
Objective To investigate the clinical and pathological features, diagnosis and differential diagnosis of rare myofibrillar sarcoma (MFS) in thyroid. Methods The clinical manifestations, histological features and immunohistochemistry of one case of primary myxosarcoma of thyroid were analyzed and relevant literatures were reviewed. Results The patient was male, 71 years old. Neck enhanced CT and color Doppler ultrasound showed bilateral thyroid lesions. Under light microscopy, the tumors were separated by fibrous connective tissue with multiple nodules. The tumor cells were fusiform or stellate, and were arranged in mucin-like stroma in disorder or bundled form. The morphological characteristics of pseudolobular fats Blast cells, as well as the curved or arcuate blood vessels in the stroma. Immunohistochemistry showed that the expressions of vimentin, CKpan, TG, TTF1, PAX-8, calcitonin, Syn, CgA, CD34, S-100 protein, SMA, desmin, 10% ~ 15%. Conclusions Primary thyroid mucinous fibrosarcoma is extremely rare and difficult to diagnose clinically and pathologically. Metastatic soft tissue tumors, common thyroid tumors and other similar tumors should be excluded. Combined with pathological features and immunohistochemical markers, it should be confirmed. The tumor is treated by surgical excision and postoperative adjuvant radiotherapy.