Lynch syndrome is a hereditary cancer syndrome caused by germline variants in DNA mismatch repair genes, most commonly associated with colorectal and endometrial cancer. Recent literature has also shown that Lynch syndrome may rarely manifest with sarcomas. To our knowledge, tracheal chondrosarcoma has never been reported as a manifestation of Lynch syndrome. Herein we demonstrate a unique case of a patient with regionally metastatic tracheal chondrosarcoma as a presentation of Lynch syndrome.

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