Murase K, Hino A, Ozeki Y, Karagiri Y, Onitsuka A, Sugie S
Department of Surgery, Seki Chuo Hospital, Japan.
J Gastroenterol. 2001 Nov;36(11):772-7. doi: 10.1007/s005350170020.
An extremely rare case of malignant schwannoma of the esophagus with lymph node metastasis is reported. A 49-year-old woman was found to have an abnormal shadow on a chest X-ray film taken during an annual checkup. Upper gastrointestinal series showed extrinsic pressure on the middle thoracic esophagus, without a mucosal lesion. An exploratory operation was performed, with a tentative diagnosis of esophageal leiomyoma. The tumor was enucleated with part of the esophageal mucosa, and a few enlarged lymph nodes around the tumor were dissected. The resected tumor was an elastic firm mass, measuring 8.2 x 5.8 x 3.7 cm, and had a smooth surface. Histological examination of the tumor revealed the proliferation of spindle-shaped cells with chromatin-rich nuclei. The nuclei were variable in size and showed remarkable atypia. A paraesophageal lymph node had same findings as the main tumor. Immunohistochemically, the tumor cells were diffusely positive for S-100 protein and neuron-specific enolase. The pathological diagnosis of this tumor was malignant esophageal schwannoma with lymph node metastasis. Esophageal schwannoma is extremely rare. We reviewed the literature on 19 cases of esophageal schwannoma, including that in our patient. The majority of the tumors were benign. Only three cases of schwannoma were malignant, and this is the first reported case of malignant schwannoma with lymph node metastasis.
报告了一例极为罕见的伴有淋巴结转移的食管恶性神经鞘瘤。一名49岁女性在年度体检时拍摄的胸部X光片上发现有异常阴影。上消化道造影显示胸段食管中段有外在压迫,无黏膜病变。进行了探查手术,初步诊断为食管平滑肌瘤。肿瘤连同部分食管黏膜被摘除,并切除了肿瘤周围的一些肿大淋巴结。切除的肿瘤为质地坚韧的弹性肿块,大小为8.2×5.8×3.7厘米,表面光滑。肿瘤组织学检查显示梭形细胞增殖,细胞核富含染色质。细胞核大小不一,显示出明显的异型性。食管旁淋巴结与主肿瘤有相同表现。免疫组化显示,肿瘤细胞S-100蛋白和神经元特异性烯醇化酶弥漫性阳性。该肿瘤的病理诊断为伴有淋巴结转移的食管恶性神经鞘瘤。食管神经鞘瘤极为罕见。我们回顾了包括本例患者在内的19例食管神经鞘瘤的文献。大多数肿瘤为良性。只有3例神经鞘瘤为恶性,这是首例报告的伴有淋巴结转移的恶性神经鞘瘤。