Oral Soft Tissue and Jawbone Sarcomas: A Retrospective Clinicopathologic Analysis of 128 Cases from Two Institutions and Comprehensive Literature Review

Purpose: Oral soft tissue and jawbone sarcomas (OSTJS) are rare neoplasms accounting for only 1% of all intraoral malignancies. As a result, robust epidemiologic data pertaining to OSTJS are limited. Here, we present a collaborative, retrospective analysis of the clinicopathologic characteristics of 128 cases of OSTJS, together with a comprehensive review of the literature. Methods: Archived OSTJS cases (2000–2022) were retrieved from the electronic laboratory databases of the oral pathology services at The Ohio State University and University of Minnesota. Patient age and sex, anatomic site and histopathologic diagnosis were recorded. Results: Among 128 OSTJS, 123 (96.1%) were primary and 5 (3.9%) metastatic (M: F = 1.5:1; mean age = 43.7 years, range = 4-102 years). Most OSTJS presented in adults (113, 88.3%; mean age = 47.8 years) with only 15 pediatric cases (11.7%; mean age = 13 years). Favored sites included the mandible (48, 37.5%), maxilla (39, 30.4%), gingiva (15, 11.7%), palate (13, 10.2%), and tongue (4, 3.1%). In adults, osteosarcoma represented the predominant OSTJS (58, 51.3%), followed by Kaposi sarcoma (18, 15.9%), leiomyosarcoma (7, 6.2%), chondrosarcoma (6, 5.3%), low-grade myofibroblastic sarcoma (5, 4.4%), and 4 each (3.5%) of angiosarcoma, rhabdomyosarcoma and undifferentiated pleomorphic sarcoma. Similarly, osteosarcoma comprised the most common OSTJS histotype in the pediatric population (8, 53.3%), followed by Ewing sarcoma (4, 26.7%) and 1 each (6.7%) of TFCP2::EWSR1-rearranged rhabdomyosarcoma, mesenchymal chondrosarcoma, and alveolar soft part sarcoma. Conclusion: OSTJS represent an uncommon, histopathologically diverse, subset of mesenchymal malignancies. In our series, most patients were adults in their 4th − 5th decade with a broad age range and a slight male predilection. Overall, jawbone osteosarcoma and Kaposi sarcoma accounted for two-thirds of OSTJS cases in this cohort. While the diagnosis of OSTJS relies heavily on routine light microscopic findings, ancillary immunohistochemistry and/or cytogenetic studies are frequently warranted.