Ampollini Luca, Ventura Luigi, Gnetti Letizia, Carbognani Paolo, Silini Enrico Maria, Rusca Michele
1 Thoracic Surgery, Department of Medicine and Surgery, University Hospital of Parma, Parma, Italy.
2 Pathology Unit, Department of Medicine and Surgery, University Hospital of Parma, Parma, Italy.
Tumori. 2018 Dec;104(6):NP17-NP21. doi: 10.1177/0300891618778950. Epub 2018 Jul 9.
To describe a case of giant intercostal nerve schwannoma successfully resected in a patient with neurofibromatosis type 2.
A 44-year-old woman, with a history of neurofibromatosis type 2, presented with chest discomfort and mild dyspnea. She had undergone a recent resection of a large frontal parasagittal benign meningioma. Radiologic examinations showed a large lesion (9×12×9 cm) of the left hemithorax causing a complete atelectasis of left upper lobe. Bronchoscopy did not show any endobronchial alterations, apart from an ab estriseco compression of the left upper bronchial tree. A transthoracic needle biopsy was then performed and microscopic examination revealed a mesenchymal tumor composed of spindle-like cells.
A video-assisted thoracoscopic surgery procedure was proposed. The tumor mass appeared to be tenaciously adherent to the parietal pleura in its anterolateral aspect, confirming the radiologic appearance. No invasion of the lung parenchyma or parietal pleural metastases were visible. Therefore, a left posterolateral thoracotomy at the fifth intercostal space was performed and a macroscopic complete resection was carried out. The recovery was uneventful and the patient was discharged on postoperative day 5. The histologic examination revealed a moderate cellular proliferation of spindle-shaped and oval to polygonal cells with frequent Verocay bodies; mitotic figures were rare. The tumor cells were strongly S-100 positive. The microscopic features were consistent with benign intercostal schwannoma. Eight years later, the patient is disease-free and asymptomatic.
An unusual case of giant intercostal nerve schwannoma successfully resected in a patient with neurofibromatosis type 2 is described.
描述1例2型神经纤维瘤病患者成功切除巨大肋间神经鞘瘤的病例。
一名44岁患有2型神经纤维瘤病的女性，出现胸部不适和轻度呼吸困难。她近期接受了大型额部矢旁良性脑膜瘤切除术。影像学检查显示左半胸有一个大的病变（9×12×9厘米），导致左上叶完全肺不张。支气管镜检查未发现任何支气管内改变，仅见左上支气管树受外压。随后进行了经胸针吸活检，显微镜检查显示为一个由梭形细胞组成的间叶性肿瘤。
建议行电视辅助胸腔镜手术。肿瘤块在其前外侧似乎与壁层胸膜紧密粘连，与影像学表现相符。未见肺实质侵犯或壁层胸膜转移。因此，在第五肋间间隙行左后外侧开胸手术，并进行了宏观完整切除。恢复顺利，患者术后第5天出院。组织学检查显示梭形、椭圆形至多边形细胞呈中度细胞增殖，可见频繁的Verocay小体；有丝分裂象罕见。肿瘤细胞S-100强阳性。显微镜下特征与良性肋间神经鞘瘤一致。8年后，患者无病且无症状。
描述了1例2型神经纤维瘤病患者成功切除巨大肋间神经鞘瘤的罕见病例。
