Histol Histopathol

Original Article Open Access

Primary undifferentiated pleomorphic sarcoma in oral-maxillary area: retrospective study and molecular analysis

Yuanhe You1,2,3*, Rongxin Shi4*, Meilu Dai5, Zhong Du1,2,3, Zhuowei Tian1,2,3, Guisong Xu1,2,3, Lizhen Wang1,2,6, Yan'an Wang1,2,3 and Meng Xiao1,2,3

1National Clinical Research Center for Oral Disease, 2Department of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University, School of Medicine, 3Shanghai Key Laboratory of Stomatology and Shanghai Research Institute of Stomatology, Shanghai, 4Department of Stomatology, Tengzhou Central People's Hospital, Tengzhou, Shandong Province, 5Department of Orthodontics and Oral Biomedical Engineering Laboratory, Shanghai Stomatological Hospital, Fudan University, Shanghai and 6Department of Oral Pathology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China
*The first two authors contributed equally to this paper


Corresponding Author: Dr. Meng Xiao, Dr. Yan'an Wang, and Dr. Lizhen Wang Shanghai Ninth People's Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai 200011, China. e-mail: wangya1982@sh9hospital.org; lizhenwang535@hotmail.com; xiaomeng70001@126.com


Summary. Undifferentiated pleomorphic sarcoma (UPS) in oral-maxillary area is rarely reported. Herein, we aimed to investigate the clinical characteristics, treatment strategies, prognosis, and molecular features of the oral-maxillary UPS. In total, 10 cases with primary oral-maxillary UPS were included. The rapidly progressive UPS can easily develop to an advanced and life-threatening stage, especially concerning the complex anatomical structures and spaces in the oral-maxillary area. The final diagnosis for UPS greatly depended on histological findings and immunohistochemistry staining after the exclusion of all possible differential diagnoses. Retrospectively, the treatment strategies for the included cases still referred to those of oral squamous cell carcinoma (OSCC). Statistically, the median overall survival (OS) for all the included cases was 7.75 months (range: 5-17 months). Comparatively, 3 cases had improved OS (median survival: 17 months, range: 17-18 months) and experienced PR/SD with neoadjuvant chemotherapy (anlotinib). The molecular features were demonstrated by using whole exonic sequencing for 1 included case. Cancer driver gene detection revealed GBP4 as a candidate driver gene for the primary oral-maxillary UPS. Additionally, a missense mutation in gene PIK3CA (p.E545K) was also identified. Our findings could greatly expand the knowledge about primary oral-maxillary UPS, and provide molecular evidences to improve the therapeutic options for primary oral-maxillary UPS. Histol Histopathol 36, 889-898 (2021)

Key words: Undifferentiated pleomorphic sarcoma, Oral-maxillary area, Retrospective study, Whole exonic sequencing

DOI: 10.14670/HH-18-359

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©The Author(s) 2021. Open Access. This article is licensed under a Creative Commons CC-BY International License.