Bouma Wobbe, Koning Kor Johan, Suurmeijer Albert J H, Slebos Dirk Jan, Mariani Massimo A, Klinkenberg Theo J
Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Department of Pulmonary Diseases, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
J Cardiothorac Surg. 2019 Feb 28;14(1):48. doi: 10.1186/s13019-019-0861-7.
Angiomatoid fibrous histiocytoma (AFH) is a soft-tissue tumor that generally affects the extremities of children and young adults. AFH overlaps with primary pulmonary myxoid sarcoma (PPMS) and can occur in unusual locations.
We present a case of a 22-year-old female with AFH in the distal trachea. In addition to describing the challenge in making a correct diagnosis of AFH, we describe the first case of successful hybrid bronchoscopic and surgical resection of endotracheal AFH. A staged removal procedure was required to quickly secure the airway, allowing a lower-risk elective distal tracheal resection through a cervical approach for complete resection. A more conventional, but more invasive, more painful and cosmetically less satisfying thoracotomy was avoided.
A distal tracheal resection for AFH can be safely performed in young adults through a cervical approach with excellent follow-up results.
血管样纤维组织细胞瘤（AFH）是一种软组织肿瘤，通常累及儿童和年轻成人的四肢。AFH与原发性肺黏液样肉瘤（PPMS）有重叠，且可发生于不寻常的部位。
我们报告一例22岁女性，其远端气管患有AFH。除了描述正确诊断AFH所面临的挑战外，我们还描述了首例成功通过混合支气管镜和手术切除气管内AFH的病例。需要采用分期切除手术来迅速确保气道安全，从而能够通过颈部入路进行风险较低的选择性远端气管切除以实现完整切除。避免了采用更传统但更具侵入性、更痛苦且美容效果较差的开胸手术。
对于年轻成人的AFH，通过颈部入路进行远端气管切除可安全实施，且随访结果良好。
