Association of Clinicopathological Features With Outcome in Chondrosarcomas of the Head and Neck. (April 2021)

• Record Type: Journal Article
• Authors: Asioli, Sofia; Ruengwanichayakun, Poosit; Zoli, Matteo; Guaraldi, Federica; Sollini, Giacomo; Greco, Paolo; Facco, Carla; Gibertoni, Dino; Jiménez, Brayan Vega; Benini, Stefania; Turri-Zanoni, Mario; Pasquini, Ernesto; Mazzatenta, Diego; Foschini, Maria Pia; Righi, Alberto
• Is Part Of: Otolaryngology--head and neck surgery. Volume 164:Number 4(2021)
• Page Start: 807
• Page End: 814
• Publication Date: 2021-04
• Subjects: isocitrate dehydrogenase -- chondrosarcoma -- head and neck -- cartilaginous tumor -- gene mutation; Head -- Surgery -- Periodicals; Neck -- Surgery -- Periodicals; Otolaryngology -- Periodicals; 617.51
• DOI: 10.1177/0194599820957271
• Languages: English
• ISSNs: 0194-5998

Abstract

Objective: The aim of this study is to assess the association between clinical and radiological features as well as of isocitrate dehydrogenase 1 and 2 ( IDH 1, 2) mutations with outcome in head and neck chondrosarcomas. Study Design: Retrospective study. Setting: Tertiary referral center. Methods: Clinical, histological, and molecular data of patients with head and neck chondrosarcomas treated by surgery were collected. Results: 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 IDH mutation status. Conclusions: An adequate margin positively affects survival. IDH mutation status does not affect patient outcome.