中耳侵袭性乳头状肿瘤1例报道及文献复习

Aggressive papillary tumor of the middle ear: a case report and review of literature

目的探讨中耳侵袭性乳头状肿瘤(aggressive papillary tumour,APT)的临床病理和生物学特点。方法对1例中耳APT进行组织形态学、影像学和免疫组化研究,并复习相关文献。结果患者,女性,51岁,临床表现为左侧间断性耳鸣、突发耳聋。CT和MRI示左侧中耳鼓室后下方4cm×3cm肿块,颞骨岩部不规则骨质破坏。大体为灰红灰白色碎组织一堆,与骨组织紧密粘连。组织学表现为复杂的指突状乳头、类似于甲状腺滤泡的腺管样或片状实性的结构,局部侵犯骨组织,肿瘤细胞形态较温和。免疫表型:CKpan、EMA、vimentin+,S-100大部分+,Ki-67增殖指数<5%。结论中耳APT是一种少见的独特的肿瘤,确诊依赖于病理形态学及免疫组化标记,有复发倾向,罕见转移。

Purpose To investigate the clinicopathological, and biological characteristics of aggressive papillary tumour in the middle ear. Methods The clinical presentation, histological observation, radiologic examination and immunohistochemical staining were analyzed in one case of aggressive papillary tumour of the middle ear and related literatures were reviewed. Results A 51-year-old woman complained of intermittent episodes of left-sided tinnitus and sudden deafness. CT and MRI identified a solid mass measuring 4 cm ×3 cm in the hypotympanum, which led to temporal bone destruction. Grossly, the tumor showed pieces of gray-white tissues attached to the petrous bone. Microscopically, it consisted of complex, interdigitating papillae and follicular pattern, reminiscent of thyroid parenchyma lined by uniform, cuboidal to low columnar cells. The cells were typically positive for CKpan, vimentin and EMA, and most of them positive for S-100 protein as well. The tumor exhibited low proliferative indices of less than 5%. Conclusions Aggressive papillary tumour of the middle ear is unique and uncommon, whose definite diagnosis should be based on the histopathological and immunohistochemical features to exclude other tumors. After removed by surgery the tumor can recur locally while metastasis has not yet been documented.