Abstract
BackgroundPrimary malignant tracheal tumors account for only 0.2 % of all malignancies of the respiratory tract. Tracheal chondrosarcoma is a rare condition and only 17 cases have been described in the literature from 1965 to date. Herein we report the very unusual case of a patient with a tracheal chondrosarcoma, electively treated by curative surgery despite the virtually complete obstruction of the airway.Case presentationWe present the case of a 79-year old Caucasian man with long-lasting wheezing misdiagnosed as asthma and affected by a tracheal chondrosarcoma almost completely obstructing the airway. Videobronchoscopy and imaging investigations revealed a well-circumscribed mass arising from the cartilaginous rings of the cervical trachea with a posterior residual respiratory space of about 1 mm. Because of the mobility and flaccidity of the uninvolved pars membranacea, the tiny respiratory space slightly expanded during inspiration and expiration allowing the patient to be treated without an essential emergency procedure. Standard tracheal intubation was impossible. Rigid bronchoscopy enabled placement of a small tracheal tube distally to the tumor. Successful cervical tracheal resection and reconstruction was then performed, achieving complete tumor excision. Histologically, the mass was characterized as a low-grade tracheal chondrosarcoma. Videobronchoscopy performed 9 months after surgery showed a wide, well healed tracheal anastomosis. Ten months after surgery, the patient is alive and disease free.ConclusionComplete surgical resection is the treatment of choice for tracheal chondrosarcoma. Rigid bronchoscopy is an essential tool for diagnostic and therapeutic purposes. It allows the palliative maneuvers for obstruction relief but also, in resectable patients, the intraoperative safe and straightforward management of the obstructed airway.Electronic supplementary materialThe online version of this article (doi:10.1186/s13019-016-0498-8) contains supplementary material, which is available to authorized users.
Highlights
Primary malignant tracheal tumors account for only 0.2 % of all malignancies of the respiratory tract
Complete surgical resection is the treatment of choice for tracheal chondrosarcoma
In all previously reported cases, Tracheal chondrosarcoma (TCS) appeared as a bulky tracheal tumor, with variable airway obstruction: 16 patients presented with a long clinical history [1,2,3] and one with past history of radioiodine therapy for thyroid cancer [4]
Summary
The slow tumor evolution, along with the integrity of the flaccid, membranous pars of the trachea, can explain why an emergency procedure was not needed in the reported case despite the almost absolute airway obstruction. Complete surgical resection is the treatment that allows the best oncologic results for tracheal chondrosarcomas. Curative surgery is facilitated by the limited submucosal spread of TCS which can be radically resected with a minimum clear resection margin. Rigid bronchoscopy is fundamental for surgical planning and for airway management, especially for those patients not amenable to standard tracheal intubation. Elective management in referring centers improves the outcome, because emergency treatments can be just palliative
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