Dedifferentiation of a Chondrosarcoma of the Larynx: A Case Report

Background: Chondrosarcomas of the larynx, relatively rare tumors with low grade pathology in approximately 95% of cases, can most often be managed with conservation laryngeal procedures. Dedifferentiated chondrosarcomas are much more rare and aggressive requiring more aggressive surgical extirpation.

Methods: A patient underwent three debulking procedures for a laryngeal chondrosarcoma Grade I/II histologically over a 2.5 year period of time during which slow growth was closely monitored. He then presented with relatively rapid onset of hoarseness and shortness of breath. Fiberoptic laryngoscopy demonstrated new bilateral vocal fold fixation with near occlusion of his subglottis by soft tissue mass.

Results: A total laryngectomy was performed that pathologically demonstrated a Grade III dedifferentiated chondrosarcoma. Shortly thereafter, the patient developed regional metastases, then distant metastases and then succumbed to the disease.

Conclusion: This first case of a Grade III dedifferentiating chondrosarcoma arising from closely monitored and multiple debulked previously Grade I/II tumor demonstrates the potential change in aggressiveness in low-grade chondrosarcomas of the larynx. Sudden increase in growth along with a soft tissue component to the tumor based on exam and/or imaging should heighten suspicion for dedifferentiation of the tumor and necessitates a more aggressive surgical resection. However, the chance of cure once dedifferentiation occurs is drastically reduced.