[Clear cell chondrosarcoma of the larynx. A rare tumor in an uncommon location].

Clear cell chondrosarcoma is a rare bone tumor of low malignancy. It constitutes only 2% of all chondrosarcomas and shows a particular predilection for the epiphyses of long bones. So far, there have only been three cases of clear cell chondrosarcoma of the larynx published in the literature. We report the case of a 46 year old man who suffered from slowly worsening dyspnoea. Diagnostic evaluation revealed a directly subglottic stenosis of the larynx, narrowing the lumen to 1/3. When we tried to resect the stenosis via splitting the ring cartilage from outside, tumorous infiltration of the whole ring cartilage was found. Extended biopsies were taken and histology revealed a clear cell chondrosarcoma. The therapy of choice for this tumor is wide surgical resection. Additional radio- and/or chemotherapy are not recommended. Therefore, the patient underwent laryngectomy. Histologically, the clear cell chondrosarcoma can be easily confused with the highly malignant osteosarcoma or the conventional chondrosarcoma, both requiring more aggressive treatment. Thus, clear cell chondrosarcoma should be kept in mind and, when necessary, a reference histology by a bone tumor register should be requested.