上皮样肉瘤：12 例头颈部病例的临床病理研究。

Epithelioid sarcoma: A clinicopathological study of 12 head and neck cases.

机构信息

Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

National Center for Stomatology, National Clinical Research Center for Oral Diseases, Research Unit of Oral and Maxillofacial Regenerative Medicine, Chinese Academy of Medical Sciences, Shanghai, China.

出版信息

Oral Dis. 2022 Sep;28(6):1519-1527. doi: 10.1111/odi.13835. Epub 2021 Apr 7.

DOI:10.1111/odi.13835

PMID:33751732

Abstract

OBJECTIVES

To determine the clinicopathological features of epithelioid sarcoma presenting in head and neck region (HNES) and elucidate diagnostic key points and treatment options for HNES.

MATERIALS AND METHODS

A total of 12 HNES cases were collected in our department from 2010 to 2020. Their clinical information and pathological features were documented, and relevant follow-up was performed. Immunohistochemistry was carried to analyze the protein markers of HNES.

RESULTS

Of the 12 HNES cases, 10 were primary tumors and 2 were metastasized from foot and shoulder, respectively. The patients with primary tumors were significantly younger than those with metastasized ones (22.7 vs 41.5, p = .0157), and male patients outnumbered female patients (3:1). Of all HNES cases, 9 were classic subtype, and 3 were proximal subtype. HNES patients had a poor prognosis, with 5-year overall survival of 41.5% and 5-year relapse-free survival of 22.5%. A loss of INI1 was identified as the hallmark of HNES with 83.3% (10/12) of HNES cases presenting as EZH2 positive.

CONCLUSIONS

HNES is more prevalent at younger ages and in males, has a poor prognosis, and exhibits a greater proportion of classic subtype than proximal subtype. EZH2 inhibitor has therapeutic potential in HNES.

摘要

目的

确定头颈部上皮样肉瘤（HNES）的临床病理特征，并阐明 HNES 的诊断要点和治疗选择。

材料和方法

我们科室从 2010 年到 2020 年共收集了 12 例 HNES 病例。记录了他们的临床资料和病理特征，并进行了相关随访。通过免疫组织化学分析 HNES 的蛋白标志物。

结果

12 例 HNES 病例中，10 例为原发性肿瘤，2 例分别从足部和肩部转移而来。原发性肿瘤患者明显比转移性肿瘤患者年轻（22.7 岁比 41.5 岁，p=0.0157），且男性多于女性（3:1）。所有 HNES 病例中，9 例为经典型，3 例为近端型。HNES 患者预后较差，5 年总生存率为 41.5%，5 年无复发生存率为 22.5%。INI1 的缺失被认为是 HNES 的标志，83.3%（10/12）的 HNES 病例呈 EZH2 阳性。