涎腺玻璃样变透明细胞癌8例临床病理学特征分析

张岩; 王正; 陈彤箴; 柏乾明; 李小秋

doi:10.19401/j.cnki.1007-3639.2023.02.010

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涎腺玻璃样变透明细胞癌8例临床病理学特征分析

论著 | 更新时间：2025-12-31

- 涎腺玻璃样变透明细胞癌8例临床病理学特征分析
- Hyalinizing clear cell carcinoma of salivary gland: a clinicopathological study of 8 cases
- 中国癌症杂志 2023年33卷第2期页码：168-173
- 作者机构：
  
  1. 复旦大学附属肿瘤医院病理科，复旦大学上海医学院肿瘤学系，复旦大学病理研究所，上海200032
  2. 扬州大学附属医院病理科，江苏扬州 225000
- 作者简介：
  
  [ "张岩（ORCID: 0009-0008-3230-9084），副主任医师。" ]
  李小秋（ORCID: 0000-0002-8758-2191），复旦大学附属肿瘤医院病理科副主任。
- 基金信息：
  
  上海市科学技术委员会科研计划项目(19MC1911000);上海市临床重点专科建设项目-病理科(shslczdzk01301)
- DOI：10.19401/j.cnki.1007-3639.2023.02.010
  中图分类号： R739.87
- 收稿：2022-02-22，
  
  修回：2022-10-31，
  
  纸质出版：2023-02-28
- 稿件说明：
移动端阅览
张岩, 王正, 陈彤箴, 等. 涎腺玻璃样变透明细胞癌8例临床病理学特征分析[J]. 中国癌症杂志, 2023,33(2):168-173.

Yan ZHANG, Zheng WANG, Tongzhen CHEN, et al. Hyalinizing clear cell carcinoma of salivary gland: a clinicopathological study of 8 cases[J]. China Oncology, 2023, 33(2): 168-173.
张岩, 王正, 陈彤箴, 等. 涎腺玻璃样变透明细胞癌8例临床病理学特征分析[J]. 中国癌症杂志, 2023,33(2):168-173. DOI： 10.19401/j.cnki.1007-3639.2023.02.010.

Yan ZHANG, Zheng WANG, Tongzhen CHEN, et al. Hyalinizing clear cell carcinoma of salivary gland: a clinicopathological study of 8 cases[J]. China Oncology, 2023, 33(2): 168-173. DOI： 10.19401/j.cnki.1007-3639.2023.02.010.

摘要

背景与目的：

涎腺玻璃样变透明细胞癌（hyalinizing clear cell carcinoma，HCCC）是好发于小涎腺的少见肿瘤，生物学低度恶性。由于罕见，该肿瘤不为病理科医师所熟知，误诊时有发生。本研究旨在探讨涎腺HCCC的组织学形态、免疫表型及分子病理学特点。

方法：

收集复旦大学附属肿瘤医院病理科2015年1月—2019年10月确诊的涎腺HCCC 8例，分析其临床、病理学特点，并采用荧光原位杂交的方法检测肿瘤组织中

EWSR

1基因相关易位情况，总结诊断及鉴别诊断要点，并结合文献复习进行分析。

结果：

患者均为女性，年龄26~68岁，肿瘤发生于口腔、鼻咽、腮腺及头颈部区域其他部位。显微镜下主要由透明细胞和嗜酸性细胞以不同比例混合而成，形成巢团状、片状、梁索状结构。1例肿瘤几乎完全由嗜酸性细胞构成，6例可见明显的细胞核内假包涵体，1例肿瘤组织与表面黏膜鳞状上皮相连，1例肿瘤部分区域可见腺管样结构，3例肿瘤癌巢周围形成淋巴细胞套，7例具有硬化性或玻璃样变性的间质，亦或出现促纤维结缔组织反应性间质，1例间质伴有黏液变性。所有肿瘤均未见明确角化、坏死，核分裂象罕见。免疫组织化学检测结果显示，8例肿瘤弥漫表达AE1/AE3，7例表达P63，7例肿瘤细胞灶性表达低分子角蛋白CK7或CAM5.2，但S-100、calponin、PAX-8、CD10均呈阴性，所有肿瘤增殖活性较低，Ki-67增殖指数均小于5%。荧光原位杂交检测显示，8例均有

EWSR

l基因相关易位。随访4~50个月，所有患者均无复发或转移。

结论：

涎腺HCCC是好发于小涎腺的低度恶性肿瘤，其形态学特征明显，但需与其他多种原发或继发性涎腺肿瘤鉴别。多数患者预后良好。

Abstract

Background and purpose:

Hyalinizing clear cell carcinoma (HCCC) of salivary gland is a rare tumor with low-grade malignancy

which usually occurs in minor salivary glands. Due to its rarity

the tumor type is not well known to pathologists and misdiagnosis frequently occurred. The aim of this study was to investigate the histologic

immunophenotypic and molecular features of HCCC.

Methods:

Eight cases of salivary gland HCCC diagnosed at the Department of Pathology

Fudan University Shanghai Cancer Center from January 2015 to October 2019 were collected. Clinicopathologic characteristics were analyzed

and fluorescence

in situ

hybridization (FISH) assay was used to detect

EWSR

1 translocation in tumor tissue. Diagnostic criteria and differential diagnosis were summarized

with a review of the literature.

Results:

All patients were female

aged 26 to 68 years

and the tumor locations included the oral cavity

nasopharynx

parotid gland and other sites of head and neck. Microscopically

the tumors consisted mainly of clear cells and eosinophilic cells with variable proportions

arranged in nests

sheets and trabeculae. In 1 case

the tumor was composed almost entirely of eosinophilic cells; in 6 cases

obvious nuclear pseudo-inclusion bodies were seen; in 1 case

neoplastic cells were connected to the surface squamous epithelium; in 1 case

the glandular structure was seen in some areas of the tumor; in 3 cases

a sheath of lymphocyte was formed around the tumor nests; in 7 cases

there was a sclerosing or hyalinizing stromal reaction

showing desmoplastic changes; and 1 case featured stoma with myxoid changes. There was no definite keratinization or necrosis in all tumors

and mitotic figures were generally rare. Immunohistochemical staining showed that tumor cells of 8 cases were diffusely positive for AE1/AE3 protein

7 cases expressed P63

and 7 cases focally expressed low molecular weight keratin CK7 or CAM5.2

however

S-100

calponin

PAX-8 and CD10 were all negative. All tumors showed low proliferative activity

with the Ki-67 proliferation index less than 5%. FISH test revealed

EWSR

1 translocation in all 8 cases. All patients demonstrated no recurrence or metastasis during the follow up ranging 4-50 months.

Conclusions:

HCCC of salivary gland is a low-grade malignancy occurring mainly in minor salivary glands

with characteristic histological and molecular features. This cancer type needs to be distinguished with many morphologic mimics of salivary gland. Most cases with salivary HCCC feature a good prognosis.

关键词

Keywords

references

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