中耳胚胎型横纹肌肉瘤1例

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患者男性,14岁,因左侧面瘫2月余伴左耳痛、流脓、发热3天于1980年8月7日入院。检查 体温38.3℃,左侧周围性面瘫,张口受限,下颌偏向右侧。左侧乳突区红肿,压痛明显。左侧外耳道内充满粉红色息肉样肿物。音叉试验系左耳传导性聋。血白细胞13,000中性粒细胞85%,淋巴细胞15%,血沉99mm。乳突X线照片:两侧乳突均为气化型,左侧气房模糊,房隔不清,左侧颈颌关节破坏。于8月8日在局麻下行左侧单纯乳突凿开术。术中见气房内粘膜充血,鼓窦入口及中耳腔内充满肉芽样组织,触之易出血。外耳道内有粉红色质松而脆的肿物。鼓室天盖部分骨质破坏。术中取中耳腔及外耳道内肿物活检,病理诊断:中耳胚胎型横纹肌肉瘤。术后第4天开始见左外耳道内有息肉样肿物迅速生长,再次病检诊断同前。入院后一月余出现 The patient was male, 14 years old. He was admitted to hospital on August 7, 1980 due to left facial paralysis for more than 2 months with left ear pain, pus, and fever. Check the temperature of 38.3 °C, the left side of the facial paralysis, mouth opening is limited, the jaw biased to the right. Left mastoid area was swollen and tender. The left ear canal is filled with pink polypoid tumors. The tuning fork test is conducted on the left ear. White blood cells 13,000 neutrophils 85%, lymphocytes 15%, ESR 99mm. The mastoid X-ray photos: Both mastoids are gasified, the left side of the gas room is blurred, the room is unclear, the left side of the jaw joint destruction. On August 8th, under the local anesthesia, a simple mastoidectomy was performed on the left side. During the operation, the mucous membranes in the air chamber were congested, and the entrance of the sinus tympani and the middle ear cavity were filled with granulation-like tissue, which caused easy bleeding. There is a pink loose and brittle mass in the external auditory canal. The tympanic cavity covers part of the bone destruction. The middle ear cavity and external auditory canal biopsy were performed during operation. The pathological diagnosis was: middle ear embryonal rhabdomyosarcoma. On the 4th day after operation, polypoid tumors in the left external auditory canal began to grow rapidly. The diagnosis of the disease was again the same as before. More than one month after admission
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