Association of Clinicopathological Features With Outcome in Chondrosarcomas of the Head and Neck.

Abstract

The aim of this study is to assess the association between clinical and radiological features as well as of isocitrate dehydrogenase 1 and 2 (<i>IDH</i> 1,2) mutations with outcome in head and neck chondrosarcomas. Retrospective study. Tertiary referral center. Clinical, histological, and molecular data of patients with head and neck chondrosarcomas treated by surgery were collected. Forty-six patients were included. The mean age at diagnosis was 56 years (range, 17-78). The tumor originated from the skull base (52.2%), facial bones (28.2%), or laryngotracheal area (19.6%). At last follow-up (median 52.5 months), 38 patients were alive, 30 of which were disease free, whereas 8 had died, 4 of disease progression and 4 of other causes. Fourteen (30.4%) had local recurrence and 2 (4.3%) had lung metastasis. All cases were negative for cytokeratin AE1/AE3, brachyury, and IDH1 at immunohistochemistry, while Sanger sequencing identified IDH1/2 point mutations, typically IDH1 R132C, in 9 (37.5%) tumors arising from the skull base. Margin infiltration on the surgical specimen negatively affected the outcome, whereas no correlation was identified with <i>IDH</i> mutation status. An adequate margin positively affects survival. <i>IDH</i> mutation status does not affect patient outcome.