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目的探讨内淋巴囊肿瘤的临床病理学特征、诊断要点及肿瘤的预后情况。方法对7例内淋巴囊肿瘤进行临床、影像及病理分析,并进行随访,观察肿瘤预后及患者生存情况。结果内淋巴囊肿瘤的临床症状包括进行性加重的感音神经性耳聋、耳鸣、眩晕、颅神经受累等症状。镜下瘤组织主要呈乳头状结构,局部可见腺样及囊性结构,部分腺腔及囊腔内可见粉染胶样物质,肿瘤细胞为圆形或立方形,部分瘤细胞的胞质透明,核形态及大小比较一致,异型性不明显。肿瘤间质血管较丰富,且胶原及纤维结缔组织增生,可见出血、噬含铁血黄素细胞、异物巨细胞及胆固醇结晶,部分病例出现骨质破坏及坏死。免疫表型:CK和NSE(+),部分病例CD56和S-100(+),GFAP、TTF-1、CgA、TG和Syn均(-)。7例患者中,1例肿瘤局部切除后行放射治疗,另1例术后2年复发,其余患者均未见复发、转移。结论内淋巴囊肿瘤是罕见的肿瘤,其诊断和鉴别诊断主要靠病理组织学和免疫组织化学,肿瘤的预后情况较好。
Objective To investigate the clinicopathological features, diagnosis points and prognosis of endolymphatic sac tumors. Methods Seven cases of lymphatic sac tumor were analyzed by clinical, imaging and pathology. The patients were followed up to observe the prognosis of the tumor and the survival of the patients. Results The clinical manifestations of endolymphatic sac tumors included progressive sensorineural hearing loss, tinnitus, dizziness and cranial nerve involvement. Microscope tumor tissue was mainly papillary structure, local adenoid and cystic structures can be seen, part of the glandular cavity and cysts visible powder dye-like substance, the tumor cells were round or cubic, part of the tumor cells of the cytoplasm transparent, Nuclear morphology and size are more consistent, atypia is not obvious. Tumor stroma blood vessels are more abundant, and collagen and fibrous connective tissue hyperplasia, we can see bleeding, hemosiderin cells, foreign body giant cells and cholesterol crystallization, bone destruction and necrosis in some cases. Immunophenotypes: CK and NSE (+), CD56 and S-100 (+), GFAP, TTF-1, CgA, TG and Syn in some cases (-). Of the 7 patients, one patient underwent radiotherapy after local excision, and the other one recurred 2 years after operation. No recurrence or metastasis was observed in the remaining patients. Conclusions Endolymphatic sac tumor is a rare tumor. The diagnosis and differential diagnosis of endolymphatic sac tumor are mainly based on histopathology and immunohistochemistry. The prognosis of the tumor is good.