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Myxofibrosarcoma of the head and neck is rare and even rarer are myxofibrosarcomas occurring in deep neck spaces: only 2 cases have been previously reported, both of which were low grade. Here we described a new case of low-grade myxofibrosarcoma which occurred in the carotid space. The clinical, radiological, histopathological, and prognostic features of the neck localization of this malignancy have been discussed. Radical surgical resection with free margins is the treatment of choice for neck low-grade myxofibrosarcoma. Chemotherapy and radiotherapy are not indicated. This malignancy is characterized by local recurrence and metastatic spread. For these reasons, all patients with neck lowgrade myxofibrosarcoma should be recommended for long-term follow-up.
Cite this article as: Brescia G, Marciani S, Brunello A. Low-grade myxofibrosarcoma of the deep neck spaces. B-ENT 2022;18(3):208-211.