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目的探讨原发于甲状腺的上皮样血管肉瘤的临床病理特点及鉴别诊断要点。方法报道1例甲状腺上皮样血管肉瘤,并结合文献对其临床病理特点及鉴别诊断和预后进行讨论。结果患者为老年女性,以颈部包块伴声音嘶哑就诊。CT显示颈部巨大软组织肿块伴气管受压。组织学显示,瘤组织大部分排列成实性片状,瘤细胞核大,空泡状,含有明显的核仁,部分瘤细胞胞质空泡化,内含红细胞。除实性区域外,还可见内衬不典型内皮细胞的不规则互相吻合的血管腔。周围淋巴结内见肿瘤转移。免疫组化:瘤细胞Ckpan(AE1/AE3)、CK19、CD31、CD34和FⅧRAg(+),TG、TTF-1、calcitonin、S-100、EMA和HMB45(-)。结论原发于甲状腺的上皮样血管肉瘤是一种罕见的肿瘤,恶性程度较高,确诊主要依靠组织学和免疫组化检查,临床主要应与甲状腺未分化癌鉴别。
Objective To investigate the clinicopathological features and differential diagnosis of primary epithelioid angiosarcoma in primary thyroid gland. Methods One case of thyroid epithelioid angiosarcoma was reported and its clinical and pathological characteristics, differential diagnosis and prognosis were discussed. Results The patient was an elderly woman who had hoarseness with a neck mass. CT showed a huge soft tissue mass with tracheal compression. Histology showed that most of the tumor tissue arranged in a solid sheet, tumor nuclei, vacuolar, with obvious nucleoli, some tumor cells cytoplasmic vacuolization, containing red blood cells. In addition to the real area, but also see the lining of atypical endothelial cells irregularly coincide with the vascular lumen. See around the lymph node metastasis. Immunohistochemistry: tumor cells Ckpan (AE1 / AE3), CK19, CD31, CD34 and FⅧRAg (+), TG, TTF-1, calcitonin, S-100, EMA and HMB45 (-). Conclusion The primary thyroid-derived epithelioid angiosarcoma is a rare tumor with a high degree of malignancy. The diagnosis mainly depends on histology and immunohistochemistry, and the clinical outcome should be mainly differentiated from undifferentiated thyroid carcinoma.