Ampntin Kleanthi-Christina, Tasis Nikolaos, Arnaouti Maria, Chrysi Maria, Korkolis Dimitrios P, Plastiras Aris
Department of Surgical Oncology, General Anticancer and Oncology Hospital of Athens "Saint Savvas", Athens, GRC.
Department of Pathology, General Anticancer and Oncology Hospital of Athens "Saint Savvas", Athens, GRC.
Cureus. 2024 Dec 5;16(12):e75189. doi: 10.7759/cureus.75189. eCollection 2024 Dec.
Malignant peripheral nerve sheath tumor (MPNST) is an uncommon type of sarcoma that arises from a peripheral nerve or any tissue with nerve sheath differentiation. It does not have any specific symptoms and shows great variability in clinical and radiological findings. It is usually associated with neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumors mostly originate from soft tissue organs and rarely originate from the small bowel. Less than 20 cases of intestinal MPNSTs are recorded worldwide. Therefore, we present this unique case of a 78-year-old woman, with no history of NF1, who presented with abdominal pain and vomiting and was diagnosed postoperatively with MPNST in the small bowel. Prompted by this case, we reviewed the literature for cases of intestinal MPNSTs.
恶性外周神经鞘瘤（MPNST）是一种罕见的肉瘤类型，起源于外周神经或任何具有神经鞘分化的组织。它没有任何特异性症状，在临床和影像学表现上差异很大。它通常与1型神经纤维瘤病（NF1）相关。恶性外周神经鞘瘤大多起源于软组织器官，很少起源于小肠。全球记录的肠道MPNST病例不到20例。因此，我们报告了这例独特的病例，一名78岁女性，无NF1病史，出现腹痛和呕吐，术后被诊断为小肠MPNST。受此病例启发，我们查阅了有关肠道MPNST病例的文献。
