Department of Thoracic Surgery, The First Hospital of Jilin University, 71 Xinmin Street, Changchun, 130021, Jilin, China.
BMC Surg. 2021 Mar 10;21(1):128. doi: 10.1186/s12893-021-01122-5.
Neurofibromatosis comprises neurofibromatosis type 1 (NF1) and type 2 (NF2). Major tumor type of NF1 are neurofibroma recognized as benign peripheral nerve tumor, malignant peripheral nerve sheath tumor (MPNST), and glioma.
We report a woman with a special condition, whose tumors in body surfaces were benign neurofibroma and tumors in posterior mediastinum are MPNST. The chest-enhanced CT suggested a round soft tissue density in posteriormediastium. The diagnosis was established by pathology and immunohistochemistry. A single-stage thoracoscopic mediastinal mass resection was performed. The whole operation went smoothly and the CT scan of lungs did not show relapse of tumor three months later.
The appearance of neurofibroma should draw particular attention to the possibility of developing MPNST. More careful imaging examinations should be carried out, and pathological examination could diagnose it.
神经纤维瘤病包括神经纤维瘤病 1 型（NF1）和 2 型（NF2）。NF1 的主要肿瘤类型为神经纤维瘤，被认为是良性周围神经肿瘤，恶性周围神经鞘瘤（MPNST）和神经胶质瘤。
我们报告了一名女性的特殊情况，其体表肿瘤为良性神经纤维瘤，而后纵隔肿瘤为 MPNST。胸部增强 CT 提示后纵隔有一个圆形软组织密度影。通过病理和免疫组织化学检查确诊。行一期胸腔镜纵隔肿块切除术。整个手术过程顺利，三个月后肺部 CT 扫描未见肿瘤复发。
神经纤维瘤的出现应特别注意发生 MPNST 的可能性。应进行更仔细的影像学检查，病理检查可明确诊断。
