A Rare Pathology: Low-Grade Fibromyxoid Sarcoma of the Maxilla

J Oral Maxillofac Surg. 2016 Jan;74(1):219.e1-10. doi: 10.1016/j.joms.2015.09.018. Epub 2015 Sep 25.


Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor with a benign histologic appearance and fully malignant behavior. To date, only 5 cases of LGFMS in the maxillofacial region have been reported. This report describes the case of a 16-year-old boy who was referred to the authors' hospital with an intraosseous myxofibroblastic tumor, probably of the LGFMS type, of the right maxilla. Radical resection with wide safe margins and secondary reconstruction with a free forearm flap were performed. Six-month follow-up showed no sign of recurrence or metastasis. The authors review the scientific literature and discuss different tumor locations and treatment strategies for those in the maxillofacial region. The present case is the sixth reported case of LGFMS in the maxillofacial region (intraosseous LGFMS of the maxilla), adding another facet to the literature regarding this rare soft tissue tumor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Bone Transplantation / methods
  • Follow-Up Studies
  • Free Tissue Flaps / transplantation
  • Humans
  • Male
  • Maxillary Neoplasms / diagnosis*
  • Maxillary Neoplasms / surgery
  • Maxillary Sinus / surgery
  • Osteotomy / methods
  • Plastic Surgery Procedures / methods
  • Sarcoma / diagnosis*
  • Sarcoma / surgery
  • Skin Transplantation / methods