J Korean Assoc Oral Maxillofac Surg.  2020 Aug;46(4):282-287. 10.5125/jkaoms.2020.46.4.282.

Undifferentiated pleomorphic sarcoma of the mandible

  • 1Department of Oral Medicine, Hospital Sírio Libanês, São Paulo, Brazil
  • 2Department of Oral Surgery and Pathology, School of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, São Paulo, Brazil
  • 3Department of Pathology, Hospital Sírio Libanês, São Paulo, Brazil


Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm that is usually located in the extremities and retroperitoneum. In the past, UPS was considered the most common soft tissue sarcoma in adults; due to improvements in diagnostic techniques, most cases have been reclassified as other lineage-specific tumors. Gnathic bones are rarely affected, and the clinicopathological characteristics of this neoplasm when diagnosed in the jaw remain to be better described. In this report, we present a rare case of mandibular UPS affecting an 88-year-old female who demonstrated a painful swelling on the right side of the mandible that was accompanied by a pathological fracture. Microscopic examination revealed a pleomorphic spindlecell neoplasm with mitotic figures and necrosis. The patient underwent surgery and adjuvant radiotherapy but experienced metastasis after 12 months of follow-up and died. Diagnosis of UPS is challenging, and oral pathologists must be aware of this entity when dealing with aggressive undifferentiated neoplasms.


Soft tissue sarcoma; Pleomorphic sarcoma; Malignant fibrous histiocytoma; Oral cavity; Mandible
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