Frank T C, Anand V K, Subramony C
Department of Surgery, University of Mississippi Medical Center, Jackson 39216, USA.
Ear Nose Throat J. 2000 Mar;79(3):183, 187-8, 191-2 passim.
Yolk sac tumor (endodermal sinus tumor) is rarely encountered in the temporal bone. Facial nerve paralysis can be a primary manifestation of this condition. Histologically, the tumor can be difficult to diagnose, although elevated levels of alpha fetoprotein can facilitate its identification. In this report, we describe the case of an 18-month-old girl who developed peripheral VIIth nerve palsy and a polypoid mass in the left external ear canal 3 months following myringotomy. Computed tomography and magnetic resonance imaging revealed that the tumor involved the left external ear canal, middle ear space, and mastoid air cells. Biopsies were consistent with a yolk sac tumor. Special staining demonstrated that only a very few tumor cells were positive for alpha fetoprotein, despite the markedly elevated level of alpha fetoprotein in her serum. The patient was treated with chemotherapy, which included cisplatin, etoposide, and bleomycin. Within a period of weeks, she experienced a complete reversal of her left VIIth nerve palsy, a marked decrease in her serum alpha fetoprotein levels, and a dramatic resolution of the tumor as demonstrated radiographically. Such a successful chemotherapeutic response in this case argues against surgical intervention in other cases, particularly in view of the risk of serious complications with surgery.
卵黄囊瘤（内胚窦瘤）在颞骨中很少见。面神经麻痹可能是这种疾病的主要表现。从组织学上看，该肿瘤可能难以诊断，尽管甲胎蛋白水平升高有助于其识别。在本报告中，我们描述了一名18个月大女孩的病例，她在鼓膜切开术后3个月出现左侧周围性VII面神经麻痹和左侧外耳道息肉样肿物。计算机断层扫描和磁共振成像显示肿瘤累及左侧外耳道、中耳腔和乳突气房。活检结果与卵黄囊瘤一致。特殊染色显示，尽管她血清中甲胎蛋白水平明显升高，但只有极少数肿瘤细胞甲胎蛋白呈阳性。该患者接受了化疗，化疗药物包括顺铂、依托泊苷和博来霉素。几周内，她左侧VII面神经麻痹完全恢复，血清甲胎蛋白水平显著下降，影像学显示肿瘤显著消退。该病例如此成功的化疗反应表明其他病例不宜进行手术干预，特别是考虑到手术存在严重并发症的风险。
