Tysome James R, Harcourt Jonny, Patel Manish C, Sandison Ann, Michaels Leslie
Department of Otolaryngology-Head and Neck Surgery, Charing Cross Hospital, London, SE10 8EX, UK.
Ear Nose Throat J. 2008 Jul;87(7):378-93.
Aggressive papillary tumors of the middle ear are rare, and their true origin is not clear. We describe the clinical, radiologic, genetic, and histopathologic features of a papillary epithelial tumor filling the middle ear of a 68-year-old woman. Imaging revealed no evidence of petrous temporal bone apex involvement, nor did genetic studies demonstrate the von Hippel-Lindau mutation. A literature search revealed 24 previously reported cases of such a papillary epithelial tumor of the middle ear. All except 2 cases demonstrated apical petrous temporal bone invasion on imaging, and it has been suggested that they arose from a primary endolymphatic sac tumor, which has a similar papillary epithelial histology. Substantial numbers of cases of papillary epithelial tumors involving the middle ear are reported to be associated with von Hippel-Lindau disease, as are known cases of endolymphatic sac tumor. This is the third reported case of papillary epithelial tumor of the middle ear that does not show apical petrous temporal bone invasion on imaging, suggesting that such neoplasms do not always arise from a primary in the endolymphatic sac.
中耳侵袭性乳头状瘤较为罕见，其真正起源尚不清楚。我们描述了一名68岁女性中耳内充满的乳头状上皮肿瘤的临床、放射学、遗传学和组织病理学特征。影像学检查未发现岩骨尖受累的证据，遗传学研究也未显示冯·希佩尔-林道基因突变。文献检索发现此前有24例关于这种中耳乳头状上皮肿瘤的报道。除2例病例外，所有病例影像学检查均显示岩骨尖侵犯，有人认为它们起源于原发性内淋巴囊肿瘤，后者具有相似的乳头状上皮组织学特征。据报道，大量累及中耳的乳头状上皮肿瘤病例与冯·希佩尔-林道病有关，内淋巴囊肿瘤的已知病例也是如此。这是第三例报道的中耳乳头状上皮肿瘤病例，其影像学检查未显示岩骨尖侵犯，提示此类肿瘤并非总是起源于内淋巴囊原发性肿瘤。
