Myofibroblastic sarcoma of the larynx : a case report and review

Myofibroblastic sarcomas or myofibrosarcoma, are extremely rare malignant neoplasms of myofibroblasts. They are characterized by the pattern of cells and special immunohistochemical markers such as vimentin, desmin and alpha-smooth-muscle actin. The case of a patient with a history of frequently relapsing papillomas of the larynx is reported. Chronic laryngitis with focal low-grade dysplasia of the squamous epithelium was diagnosed approximately 1 year after the first treatment of the papillomas. After approximately 2 years the pathologist diagnosed the rare myofibroblastic sarcoma of the larynx. The patient underwent laryngectomy due to the spread of the tumor with a bilateral selective neck dissection. The patient is at present still free of recurrence and metastases. There is a great danger of misjudging a myofibroblastic sarcoma as an inflammatory myofibroblastic tumor and consequently to delay the urgently needed treatment. Therefore, an overview of the present state of knowledge about diagnosis and treatment of myofibroblastic sarcomas will be given based on this case report.