BACKGROUND: 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.
CASE PRESENTATION: 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.
CONCLUSIONS: A distal tracheal resection for AFH can be safely performed in young adults through a cervical approach with excellent follow-up results.