Maschek H, Schrader B, Werner M, Franke K D, Georgii A
Pathologisches Institut, Medizinische Hochschule Hannover.
HNO. 1992 Oct;40(10):405-9.
Progressive unilateral sensorineural deafness and tinnitus developed in a 59-year-old woman over a 1-year period. Clinical examination showed a tumor mass which almost completely filled the tympanic cavity, and grew around the auditory ossicular chain. Histological findings revealed the tumor to be a primary carcinoid of the middle ear. Neuro-endocrine differentiation was confirmed immunohistochemically by positivity for neuron-specific enolase, chromogranin, pancreatic polypeptide and synaptophysin. Using electron microscopy, neuroendocrine granules could be visualized. In addition, both light and electron microscopy revealed that cells had an epithelial differentiation with mucin granules while immunohistochemistry showed a positivity for cytokeratins. The detection of intermediary filaments (immunohistochemically with vimentin and under electron microscopy) was unique to this neoplasm and has to be considered in distinguishing the carcinoid tumor from the papillary adenoma of the middle ear. Tumor prognosis is excellent with radical extirpation from the middle ear. In the case presented, there has been no evidence for either recurrence or metastases 10 months after surgical resection.
一名59岁女性在1年时间里逐渐出现单侧进行性感音神经性耳聋和耳鸣。临床检查发现一个肿瘤块，几乎完全充满鼓室，并围绕听骨链生长。组织学检查结果显示该肿瘤为中耳原发性类癌。通过神经元特异性烯醇化酶、嗜铬粒蛋白、胰多肽和突触素免疫组化阳性证实了神经内分泌分化。利用电子显微镜可观察到神经内分泌颗粒。此外，光镜和电镜均显示细胞具有上皮分化且含有粘蛋白颗粒，而免疫组化显示细胞角蛋白呈阳性。中间丝的检测（免疫组化检测波形蛋白以及电镜观察）是该肿瘤所特有的，在鉴别中耳类癌与中耳乳头状腺瘤时必须予以考虑。中耳根治性切除后肿瘤预后良好。在本病例中，手术切除10个月后未发现复发或转移迹象。
