Background: Tracheal chondrosarcoma is a rare malignancy, and formal treatment guidelines have not been established due to the lack of high quality studies. Best evidence at this time is limited to case reports.Aim: Explore the role of surgical intervention, radiation therapy, and chemotherapy, and the long-term outcomes for these interventions for tracheal chondrosarcoma.Methods and Results: A literature search was performed using PubMed and ResearchGate (1959-2020) using medical subject heading terms "tracheal chondrosarcoma" OR "trachea chondrosarcoma." Additional reports were identified within reviewed articles and included for review. Articles pertaining to chondrosarcomas of the lung, bronchus, larynx, or other head and neck subsites were excluded. Cases of chondromas were excluded. Thirty-five patients with tracheal chondrosarcoma were identified in the literature since 1959. Advanced age was significantly associated with recurrent or persistent disease (p = .003). The majority (77%) of cases were treated with open surgical resection, with an open approach and negative surgical margins being significantly associated with being disease-free after treatment (p = .001 and p < .001, respectively). Adjuvant radiotherapy was reserved for those unfit for surgery or for recurrent disease. Tumor size, extra-tracheal extension, tumor calcification, location, and initial diagnosis were not associated with tumor recurrence.
Conclusion:Non-metastatic tracheal chondrosarcoma can be treated by adequate surgical resection, with little to no role for adjuvant radiotherapy or chemotherapy.Open surgery and negative margins were associated with oncologic control, while advanced age was associated with recurrent or persistent disease.