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Malignant fibrous histiocytoma of the larynx in a patient with neurofibromatosis type 1. Problem: Soft tissue sarcomas are rare malignancies in the head and neck region. Malignant fibrous histiocytoma is one of the most common types of head and neck soft-tissue sarcomas. Neurofibromatosis type 1 (NF1), or von Recklinghausen disease, is an autosomal dominant condition characterized by the development of multiple neurofibromas that can transform into aggressive sarcomas.
Case report: We present an unusual case of malignant fibrous histiocytoma originating from the right vocal fold of the larynx associated with NF1 and discuss its histopathological and clinical features in light of the current literature.
Conclusion: Because of their increased risk for malignancy, patients with NF1 should be carefully monitored for the development of additional neoplasms. Our experience supports the use of conservative surgery for treating limited lesions of laryngeal malignant fibrous histiocytoma.