Low-Grade Fibromyxoid Sarcomas with the Maxillary Sinus Localization: A Case Report and Review of the Literature

Abstract

A low-grade fibromyxoid sarcoma (LGFMS) is an extremely rare tumor first described by Evans in 1987.LGFMS is a type of soft tissue sarcoma characterized by a deceptively benign histological appearance but completely malignant behavior. LGFMS is usually seen in the deep soft tissues of the extremities and trunk. We have examined many reviews, case reports and case series previously published in PubMed and Google Scholar. To date, only five cases have been reported in the maxilla. LGFMS generally affects young adults, but it can also be seen in children and older adults. A radical surgical approach is the most recommended treatment option. LGFMS has a very low mitotic activity; therefore, it is considered that neither chemotherapy nor radiotherapy has a significant effect on long-term LGFMS prognosis. However, to date, there has been no study suggesting any protocol for the follow-up of patients with LGFMS. In this report, we present a case with LGFMS located in the maxillary sinus, in which despite radiotherapy following extensive surgical excision, the tumor recurred in a short period of three months and reached its former size.

Low grade fibromyxoid sarcoma of the oral cavity in an infant The youngest patient in the world A case report

“…LGFMS is a rare tumour in the head and neck region. The most recent literature review takes into account 45 cases of localization in the head and neck; among them, the oral cavity represents only 5 cases (2 cheek, 1 hard palate, 1 tongue and 1 buccal mucosa) and a case series of 15 patients in which only one has an LGFMS of the oral cavity (1 lower lip) [9,10]. Apart from the patient with the localization in the hard palate, the interesting thing is that all other patients were younger than eighteen.…”

“…[9] can help in differential diagnosis, and this case report would also like to make an effort to the importance of pathologist to gain the correct diagnosis. Additionally, this paper increases the number of LGFMS in the head and neck region; nowadays, around 50 cases, less or more, were described with different locations and variable ages from 22 months to 84 years [10]. The treatment of choice is a wide surgical resection of the primary lesion within clear margins and eventually chemotherapy and radiotherapy if required [11,12].…”