Watanabe Shin-Nosuke, Kimura Daisuke, Tani Kengo, Sasaki Takahiro, Kimura Shuta, Muto Chisaki, Kato Tsubasa, Minakawa Masahito
Department of Thoracic and Cardiovascular Surgery, Hirosaki University, Graduate School of Medicine, Hirosaki, Aomori, Japan.
Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.25-0150. Epub 2025 Aug 22.
Neurogenic tumors commonly develop in the posterior mediastinum in both pediatric and adult patients. In patients with neurofibromatosis type 1, distinguishing benign schwannomas from malignant peripheral nerve sheath tumors is challenging. In this study, we aimed to present the surgical management of a giant schwannoma that required differentiation from a malignant peripheral nerve sheath tumor.
A 15-year-old boy presented with a large right mediastinal mass discovered on chest radiography at high school admission. Subsequent contrast-enhanced CT of the chest showed the development of a large tumor (16.0 × 12.5 × 11.8 cm) occupying approximately two-thirds of the right thoracic cavity, with atelectasis of the lower lobe of the right lung. The patient was histopathologically diagnosed with a benign schwannoma associated with neurofibromatosis type 1 through a thoracoscopic biopsy of the tumor and had received oral selumetinib at 50-70 mg/day for 11 months. Surgical excision was performed because of tumor progression and suspected malignant transformation. Right posterolateral thoracotomy with the opening of the 6th intercostal space was performed by extending the anterior skin incision along the abdominal rectus muscle and separating the 6th costal cartilage and diaphragmatic margin along the chest wall. The tumor was completely removed by resecting numerous adhesions between the tumor and the surrounding tissues and coagulating several nutrient vessels that flowed into the tumor, while resecting the lower lobe of the lung. The postoperative course was uneventful. The pathological examination revealed no malignancy. Subsequent contrast-enhanced CT of the chest revealed no residual tumors.
Posterolateral thoracotomy with the separation of the costal cartilage and diaphragmatic margin along the chest wall could achieve safe surgery for a giant mediastinal schwannoma.
神经源性肿瘤常见于儿童和成人患者的后纵隔。对于1型神经纤维瘤病患者，区分良性神经鞘瘤与恶性外周神经鞘膜瘤具有挑战性。在本研究中，我们旨在介绍一例需要与恶性外周神经鞘膜瘤进行鉴别诊断的巨大神经鞘瘤的外科治疗。
一名15岁男孩在高中入学时胸部X线检查发现右侧纵隔有一巨大肿块。随后的胸部增强CT显示有一个大肿瘤（16.0×12.5×11.8 cm），占据了右侧胸腔约三分之二的空间，右肺下叶肺不张。通过肿瘤的胸腔镜活检，患者经组织病理学诊断为与1型神经纤维瘤病相关的良性神经鞘瘤，并接受了每天50 - 70 mg的口服司美替尼治疗11个月。由于肿瘤进展及疑似恶变，遂行手术切除。沿腹直肌延长前皮肤切口并沿胸壁分离第6肋软骨和膈肌边缘，行右侧后外侧开胸术，打开第6肋间间隙。通过切除肿瘤与周围组织之间的大量粘连并凝固数条流入肿瘤的滋养血管，同时切除肺下叶，将肿瘤完全切除。术后病程顺利。病理检查未发现恶性病变。随后的胸部增强CT显示无残留肿瘤。
沿胸壁分离肋软骨和膈肌边缘的后外侧开胸术可为巨大纵隔神经鞘瘤实现安全手术。
