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

Affiliations
  • 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

Abstract

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.

Keyword

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

Figure

  • Fig. 1 Initial radiographic presentation of our patient prior to diagnosis of a central giant cell granuloma. A well-defined, irregular, multilocular radiolucent image is visible on the right side of the posterior region of the mandible.

  • Fig. 2 Radiographic appearance of the neoplasm when the second image evaluation was requested. The radiolucent image is larger, with an irregular and ill-defined posterior border. A pathological fracture can also be seen.

  • Fig. 3 A tridimensional reconstruction of computed tomography reveals fracture of the mandible and the lesion occupying three-quarters of the ascending ramus.

  • Fig. 4 Microscopic findings after the second biopsy. A. Under low-power view, the neoplasm showed hypercellularity and a fibrous connective stroma (H&E staining, ×50). B. The tumor was composed of spindle cells with areas resembling a storiform growth pattern (H&E staining, ×100). C. Pleomorphic cells and atypical mitotic figures also were observed (H&E staining, ×200).

  • Fig. 5 Immunohistochemistry (DAB stainings, ×200). A. Strong positivity for vimentin. B. Histiocytes demonstrated CD68 reactivity. Focal positive reaction for desmin (C) and α-SMA (D).

  • Fig. 6 Radiographic image of the patient at six months of follow-up (segmental mandibulectomy with condyle disarticulation).


Reference

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