Malignant peripheral nerve sheath tumor arising from the greater omentum: case report.
Malignant peripheral nerve sheath tumor arising from the greater omentum: case report.
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DOI：
10.1186/1477-7819-9-33
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发表时间：
2011-03-21
影响因子：
3.2
通讯作者：
Ohdan H
中科院分区：
文献类型：
--
作者：
Miguchi M;Takakura Y;Egi H;Hinoi T;Adachi T;Kawaguchi Y;Shinomura M;Tokunaga M;Okajima M;Ohdan H
恶性周围神经鞘瘤（MPNST）是一种罕见的软组织肿瘤，起源于周围神经或表现出神经鞘分化。这些肿瘤大多发生在躯干，四肢或头颈部;它们很少位于腹腔。患者为71岁男性，因右下腹肿块和疼痛转诊至我院。腹部计算机断层扫描显示骨盆内有一个大的（9 × 9 cm）、边界清楚、分叶状、不均匀强化的肿块。剖腹探查术发现大网膜有一个大肿块，肿瘤被完全切除。组织学分析显示肿瘤由具有高分裂活性的梭形细胞组成。在肿瘤染色中，S-100获得阳性结果，但c-kit、分化簇（CD）34、α-平滑肌肌动蛋白和结蛋白获得阴性结果。这些发现强烈支持MPNST主要起源于大网膜的诊断。据我们所知，这是第一次报告的情况下，MPNST产生的大网膜。在这份报告中，我们描述了一个病人的MPNST来自大网膜，并讨论了MPNST的临床特点和管理。
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue tumors that arise from a peripheral nerve or exhibit nerve sheath differentiation. Most of these tumors arise on the trunk, extremities, or head and neck regions; they are very rarely located in the abdominal cavity. The patient was a 71-year-old man who was referred to our hospital for a mass and pain in the right lower abdomen. Abdominal computed tomography revealed a large (9 × 9 cm), well-circumscribed, lobulated, heterogeneously enhanced mass in the pelvis. Exploratory laparotomy revealed a large mass in the greater omentum, and the tumor was completely excised. Histopathological analysis revealed that the tumor was composed of spindle cells with high mitotic activity. On staining the tumor, positive results were obtained for S-100 but negative results were obtained for c-kit, cluster of differentiation (CD)34, α-smooth muscle actin, and desmin. These findings strongly supported a diagnosis of MPNST primarily arising from the greater omentum. To the best of our knowledge, this is the first reported case of an MPNST arising from the greater omentum. In this report, we have described the case of a patient with an MPNST arising from the greater omentum and have discussed the clinical characteristics and management of MPNSTs.
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