Low-grade fibromyxoid sarcoma of the tongue: A rare nosological entity

Low-grade fibromyxoid sarcoma (LGFMS) is a rare distinctive type of soft-tissue sarcoma characterized by benign clinical and histological appearance with very indolent but malignant behavior. LGFMS commonly arise in the proximal extremities, chest and trunk, rarely involving head and neck region. Management of LGFMS is challenging since it mimics a solitary benign lesion. It presents as a solitary mass with clinical and radiological benign features and without concomitant regional metastasis. Despite the benign histological appearance, LGFMS is considered an aggressive sarcoma, with 60% of local recurrence and more than 40% of distant metastasis rate. We describe a case of LGFMS of the oral cavity treated in our center, which to our knowledge is the second reported case in the English-language literature.