Iida Atsushi, Imamura Yoshiaki, Katayama Kanji, Hirose Kazuo, Yamaguchi Akio
First Department of Surgery, Fukui Medical University, 23-3 Shimoaizuki, Matsuoka, Yoshida-gun,Fukui, 910-1193, Japan.
Surg Today. 2003;33(12):940-3. doi: 10.1007/s00595-003-2610-7.
Plexiform schwannoma is a benign peripheral nerve sheath tumor composed exclusively of schwann cells arranged in a plexiform pattern. Most plexiform schwannomas are skin tumors and there has been only one case report of this tumor originating in the colon. We describe herein the first known case of plexiform schwannoma of the small intestine occurring without any relationship to schwannomatosis or neurofibromatosis. A 57-year-old man presented with a short history of abdominal pain, vomiting, and bloody stool after each meal. Jejunography demonstrated multiple nodular tumors in the small intestine. We resected the small intestine laparoscopically. The tumors consisted of multiple white nodules in the submucosal and subserosal layers. Microscopic examination revealed that each tumor was composed mainly of Antony A tissue, compatible with conventional schwannoma. Immunohistochemically, the tumors were positive for S-100, vimentin, and neuron-specific enolase, and negative for HHF35, Alpha-SMA, and c-kit. No evidence of recurrence has been found in 38 months of follow-up.
丛状神经鞘瘤是一种良性周围神经鞘膜肿瘤,仅由呈丛状排列的施万细胞组成。大多数丛状神经鞘瘤是皮肤肿瘤,仅有一例该肿瘤起源于结肠的病例报告。我们在此描述首例已知的小肠丛状神经鞘瘤病例,该病例与神经鞘瘤病或神经纤维瘤病无关。一名57岁男性患者,餐后出现腹痛、呕吐和便血的病史较短。空肠造影显示小肠内有多个结节状肿瘤。我们通过腹腔镜切除了小肠。肿瘤由黏膜下层和浆膜下层的多个白色结节组成。显微镜检查显示,每个肿瘤主要由与传统神经鞘瘤相符的Antony A组织构成。免疫组化结果显示,肿瘤对S-100、波形蛋白和神经元特异性烯醇化酶呈阳性反应,对HHF35、α-平滑肌肌动蛋白和c-kit呈阴性反应。随访38个月未发现复发迹象。