Chondrosarcomas of the head and neck

Chondrosarcoma represents approximately 11% of all primary malignant bone tumors. It is the second most common sarcoma arising in bone after osteosarcoma. Chondrosarcomas of the head and neck are rare and may involve the sinonasal tract, jaws, larynx or skull base. Depending on the anatomical location, the tumor can produce a variety of symptoms. Computed tomography and magnetic resonance imaging are the preferred imaging modalities. The histology of conventional chondrosarcoma is relatively straightforward; major challenges are the distinction between grade I chondrosarcomas and chondromas, and the differential diagnosis with chondroblastic osteosarcoma and chondroid chordoma. Surgery alone or followed by adjuvant radiotherapy is the treatment of choice. Radiotherapy alone has also been reported to be effective and can be considered if mutilating radical surgery is the only curative alternative. The 5-year survival for chondrosarcoma reaches 80%; distant metastases and/or local recurrences significantly worsen prognosis. The present review aims to summarize the current state of information about the biology, diagnosis and management of these rare tumors.