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目的探讨鼻腔平滑肌源性肉瘤的临床及病理形态特征、治疗与预后。方法对鼻腔平滑肌源性肉瘤病例进行临床、组织学形态和免疫组化染色观察并文献复习。结果患者双侧间断交替鼻塞伴流清涕1年。鼻窦冠状位CT示:右侧鼻窦炎、鼻中隔偏曲,慢性鼻炎。查体见右侧中鼻道荔枝样新生物,周围可见大量黄白色脓性分泌物,右侧后鼻孔可见暗红色新生物。术中见右侧鼻腔上部呈裂隙状,被淡红色新生物堵塞,新生物经鼻腔上部垂吊于总鼻道后部。组织学结构见组织表面被覆上皮呈原位鳞癌图像,黏膜下梭形细胞肿瘤。免疫组化PCK(部分+)、CK5/6(上皮+)、P63(上皮+)、HMB45(-)、S-100(散在+)、CD34(-)、Calponin(+)、Vimentin(+)、Myogenin(-)、Myo D1(-)、Desmin(-)、SMA部分(+)、Ki67(Li:80%)。结论鼻腔及鼻窦平滑肌肿瘤极罕见,诊断主要根据组织病理和免疫组化标记。目前认为最重要的预后因素是肿瘤部位和大小,治疗方法除手术切除外可辅助化疗。
Objective To investigate the clinical and pathological features, treatment and prognosis of nasal smooth muscle sarcoma. Methods The clinical, histological and immunohistochemical staining of nasal smooth muscle sarcoma were observed and reviewed. Results The patient was interrupted on both sides of the nasal plug with a clear runny nose 1 year. Coronal sinus CT showed: right sinusitis, nasal septum deviation, chronic rhinitis. See the right side of the middle nasal cavity lychee-like organisms, visible a large number of yellow-white purulent discharge, the right nostril visible dark red new creatures. Surgery, see the right nasal cavity was the upper part of the flesh was blocked by the new reddish creature, the new organ is suspended by the upper part of the nasal cavity in the nose. Histological structure See the tissue surface epithelium was squamous cell carcinoma in situ image, submucosal spindle cell tumor. Immunohistochemistry PCK (partial +), CK5 / 6 (epithelial +), P63 (epithelial +), HMB45 (-), S-100 , Myogenin (-), Myo D1 (-), Desmin (-), SMA part (+), Ki67 (Li: 80%). Conclusions Nasal and sinus smooth muscle tumors are extremely rare. The diagnosis is mainly based on histopathology and immunohistochemistry. Currently considered the most important prognostic factor is the size and location of the tumor. In addition to surgical resection, adjuvant chemotherapy can be used.