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Otorinolaringologia 2019 September;69(3):188-91
DOI: 10.23736/S0392-6621.18.02205-1
Copyright © 2018 EDIZIONI MINERVA MEDICA
language: English
Low-grade fibromyxoid sarcoma of the tongue: a rare nosological entity
Raul PELLINI 1, Armando DE VIRGILIO 2 ✉, Gerardo PETRUZZI 3, Barbara PICHI 1, Giuseppe MERCANTE 1, Luca MALVEZZI 2, Antonello VIDIRI 4, Giuseppe SPRIANO 2
1 Unit of Otorhinolaryngology, Regina Elena National Cancer Center, Rome, Italy; 2 Department of Otorhinolaryngology, Humanitas University, Milan, Italy; 3 Unit of Otorhinolaryngology, Department on Neuroscience, Federico II University Hospital, Naples, Italy; 4 Unit of Radiology, Regina Elena National Cancer Center, Rome, Italy
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.
KEY WORDS: Fibrosarcoma; Tongue neoplasms; Soft tissue neoplasms