Filisetti Claudia, Russo Tiziana, Pansini Andrea, Vella Claudio, Viglio Camilla, Riccipetitoni Giovanna
Department of Pediatric Surgery, "V.Buzzi" Children Hospital, University of Pavia, Milano, Italy.
European J Pediatr Surg Rep. 2020 Jan;8(1):e52-e55. doi: 10.1055/s-0040-1713766. Epub 2020 Aug 19.
Primary myoepithelial carcinoma of the lung (PMC-L) arising from the bronchial glands in lower respiratory tract is exceedingly rare. Thus far, few cases in adults and only one in a pediatric patient have been recorded. To our knowledge, this is the first report of PMC-L successfully removed in a child, focusing on the importance of multidisciplinary primary surgery for the treatment of this tumor. A 7-year-old girl was admitted for persistent cough and fever; she was unresponsive to oral antibiotics. Chest radiography showed loss of volume of left lung sustained by almost total atelectasis. After routine clinical investigations, she was referred for computed tomography scan and magnetic resonance imaging that documented the presence of a mass occupying the entire left upper lobe, infiltrating the pulmonary hilum (main bronchus, pulmonary artery, superior pulmonary vein, and pericardium). After multidisciplinary evaluation, the histopathologic diagnosis of PMC-L was established using ultrasonography-guided transthoracic core needle biopsy and bronchoscopic biopsies. She was then subjected to left pneumonectomy under extracorporeal circulation and positioning of a thoracic expander filled with 200 mL of saline solution. The postoperative course was uneventful. With TREP (very Rare Tumor in Pediatric Age) consent radiotherapy was performed (61.2 Gy). At the 10-month follow-up, the patient was alive, breathing normally without any oxygen support, without recurrence of PMC-L or metastasis, and without any chest deformity. To our knowledge, this is the first case where a pediatric patient was successfully operated for PMC-L involving the whole lung. Extracorporeal circulation enabled us to perform radical primary surgery. Prosthesis implant not only maintained normal chest expansion but also allowed focused radiotherapy, thus enabling us to prevent damage to vital organs.
起源于下呼吸道支气管腺体的原发性肺肌上皮癌（PMC-L）极为罕见。迄今为止，仅记录到少数成人病例，儿科患者仅有1例。据我们所知，这是首例成功为儿童切除PMC-L的报告，重点强调了多学科原发性手术治疗该肿瘤的重要性。一名7岁女孩因持续咳嗽和发热入院；口服抗生素治疗无效。胸部X线检查显示左肺容积减小，几乎完全肺不张。经过常规临床检查后，她接受了计算机断层扫描和磁共振成像检查，结果显示有一个肿块占据整个左上叶，浸润肺门（主支气管、肺动脉、肺上静脉和心包）。经过多学科评估，通过超声引导经胸芯针活检和支气管镜活检确立了PMC-L的组织病理学诊断。随后，她在体外循环下接受了左肺切除术，并植入了一个填充200毫升生理盐水的胸腔扩张器。术后过程顺利。在获得TREP（儿童罕见肿瘤）同意后进行了放疗（61.2 Gy）。在10个月的随访中，患者存活，呼吸正常，无需任何氧气支持，无PMC-L复发或转移，也无任何胸部畸形。据我们所知，这是首例成功为累及全肺的PMC-L患儿进行手术的病例。体外循环使我们能够进行根治性原发性手术。假体植入不仅维持了正常的胸部扩张，还允许进行聚焦放疗，从而使我们能够避免对重要器官造成损害。
