Case Presentation: Chondrosarcoma is a tumor of malignant chondrocytes that commonly arises in the proximal femur, pelvis, and hip. We report this very unusual case of a patient presenting initially with chronic voice hoarseness, who was found to have a growing mass in the larynx with features of a chondrosarcoma.A 45-year-old male with a past medical history of former tobacco use presented for worsening chronic voice hoarseness. This was present for 3 years prior to presentation and was misdiagnosed as GERD. The patient was treated on proton pump inhibitor therapy without improvement. Later, a CT neck with contrast showed a well-defined lesion centered in the right posterior cricoid cartilage measuring 2.0 x 2.0 x 2.3 cm, suspected to be a chondrosarcoma. Laryngoscopy confirmed a non-obstructive submucosal smooth mass along the right posterior subglottis. The patient did not follow up further until 5 years later. He began to develop new complaints including dysphagia, dyspnea on exertion, wheezing, and significant weight loss of 35 lbs in 2 years. A CT chest now revealed a 4.1 x 4.6 x 4.1 cm mass centered on the right larynx, causing mass effect and severe airway narrowing. Laryngoscopy discovered a mass characterized as a grade 1 T3 chondrosarcoma. He underwent an elective tracheostomy followed by a partial laryngectomy and right thyroid lobectomy with cartilage resection. A reconstruction of his larynx was done using an osteocutaneous radial forearm free flap and this was finally followed by a percutaneous endoscopic gastrostomy (PEG) tube placement. More recently, he has now had removal of the PEG tube and placement of a smaller tracheal tube. He has also undergone multiple laser resections for recurrent benign vocal cord polyps and is currently being managed with chronic steroids.

Discussion: Chondrosarcomas within the head and neck are rare, and represent only 0.1% of all tumors[1]. The subset of tracheal chondrosarcomas are itself an extremely rare malignancy and comprise only 0.2% of malignancies of the respiratory tract[2]. Indeed, in a systematic review using a literature search, only thirty-five patients have been identified with tracheal chondrosarcoma in the past 60 years[3]. Patients with tracheal chondrosarcoma are often asymptomatic until the tumor occludes greater than 75% of the tracheal lumen[4]. Another systematic review of sixteen patients with tracheal chondrosarcoma reported the most commonly reported symptoms include dyspnea (occurring in 100% of these patients), nonproductive cough, and wheeze[4]. The above combination of symptoms can often lead to misdiagnosis of this condition as an acute exacerbation of asthma or chronic obstructive pulmonary disease (COPD)[4]. Our patient’s presenting complaint of hoarseness, which led to a misdiagnosis of GERD, was appreciated in only 1/16 patients from this literature review.[4]

Conclusions: Formal treatment guidelines have not been established due to the lack of high quality studies and best evidence at this time is limited to case reports and systematic reviews.Given the rarity of this malignancy and case, It is crucial that future cases of tracheal chondrosarcoma are reported to allow for better characterization of its presentation, treatment, and outcomes.