Sclerosing epithelioid fibrosarcoma of the jaw: a case report and literature review.

Oral Surg Oral Med Oral Pathol Oral Radiol

Associate Professor, Director of Oral Pathology, Temple University School of Medicine, Pathology Department/Oral Pathology, Philadelphia, PA, USA. Electronic address:

Published: September 2024

AI Article Synopsis

  • Sclerosing epithelioid fibrosarcoma (SEF) is a very rare type of cancer, primarily affecting the deep soft tissues of the lower body, with limited occurrences in the head and neck region, particularly the oral and maxillofacial area.
  • A case study of a 52-year-old man revealed a radiolucent lesion near his left lower molar, initially mistaken for a common dental issue, discovered to be SEF after he had a history of the tumor in his skull.
  • The study emphasizes that SEF should be considered in the differential diagnosis for high-grade tumors in the oral region, highlighting the significance of immunohistochemical stains like MUC-4 for

Article Abstract

Introduction: Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare form of bone and soft tissue sarcoma. It occurs mainly in the deep soft tissue of the lower extremities, with few cases reported in the head and neck region. Tumors involving the oral and maxillofacial region (OMFR) and intraosseous examples are rare.

Case Presentation: We present a 52-year-old male with a radiolucent lesion at the apex of the left mandibular second molar tooth with the clinical impression of a periapical granuloma. The patient has a history of SEF of the skull, which was treated by chemotherapy and radiation. Histopathologic examination revealed islands of highly cellular, basophilic bone and osteoid surrounded by loose fibrous stroma, which contains large lobules and islands of round to oval cells with distinct cell borders and faintly granular eosinophilic cytoplasm. Tumor cells were strongly positive for MUC4 and INI-1. Based on these findings, a diagnosis of high-grade malignancy consistent with SEF was made, and correlation with the primary lesion was recommended.

Conclusion: SEF is a rare tumor seen in OMFR, and therefore, it should be included in the differential diagnosis of any high-grade malignancy. Immunohistochemical stain for MUC-4 evaluation is important for the diagnosis of SEF.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.oooo.2024.02.019DOI Listing

Publication Analysis

Top Keywords

sclerosing epithelioid
8
epithelioid fibrosarcoma
8
soft tissue
8
diagnosis high-grade
8
high-grade malignancy
8
sef
5
fibrosarcoma jaw
4
jaw case
4
case report
4
report literature
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!