24例泪腺黏液表皮样癌的临床特征分析

智绪爽; 吴畏; 杨新吉; 黑砚; 吕小辉; 邓爱军

doi:10.3969/j.issn.2095-5227.2023.04.005

24例泪腺黏液表皮样癌的临床特征分析

doi: 10.3969/j.issn.2095-5227.2023.04.005

智绪爽^{1, 2,},
吴畏²,
杨新吉²,
黑砚²,
吕小辉^{1, 2},
邓爱军^{1, 2, ,}

1.
潍坊医学院，潍坊医学院附属医院(临床医学院)，山东潍坊　261053
2.
解放军总医院第三医学中心眼科医学部，北京　100039

基金项目: 山东省自然科学基金面上项目(ZR2020MH173)

详细信息

作者简介:
智绪爽，女，在读硕士。研究方向：眼科学。Email: zxs9622@126.com

通讯作者:
邓爱军，男，博士，主任医师。Email: dengaijun@hotmail.com

中图分类号: R320.37
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- 被引次数: 0
出版历程
- 收稿日期: 2022-11-22
- 网络出版日期: 2023-04-18
- 刊出日期: 2023-04-28

Clinical features of mucoepidermoid carcinoma of lacrimal gland: An analysis of 24 cases

ZHI Xushuang^{1, 2
,},
WU Wei²,
YANG Xinji²,
HEI Yan²,
LYU Xiaohui^{1, 2},
DENG Aijun^{1, 2
, ,}

1.
Weifang Medical University, Affiliated Hospital of Weifang Medical University, School of Clinical Medical University, Weifang 261053, Shandong Province, China
2.
Senior Department of Ophthalmology, the Third Medical Center, Chinese PLA General Hospital, Beijing 100039, China

More Information

Corresponding author: DENG Aijun. Email: dengaijun@hotmail.com

摘要

摘要: 背景黏液表皮样癌(mucoepidermoid carcinoma，MEC)是常见的唾液腺肿瘤，极少数原发于泪腺，临床实践中认识有限。目的分析泪腺MEC的临床特征、影像表现、诊断治疗及预后。方法回顾性分析2010年1月 - 2021年12月解放军总医院第三医学中心连续收治的经组织病理学证实的24例泪腺MEC患者的临床、影像及病理资料。结果 24例患者平均患病年龄为56.35岁，男女比例7∶1。临床表现：21例(87.5%)眼球突出，16例(66.7%)眶外上方可扪及肿物，3例(12.5%)眶周疼痛。初发为泪腺MEC有19例，泪腺多形性腺瘤术后恶变为MEC有5例；6例发生远处转移。10例泪腺MEC的CT图像中，7例呈不均匀软组织密度影，9例形状不规则，7例肿瘤内有钙化，7例肿瘤破坏周围骨质；8例泪腺MEC的MRI图像中，8例不均匀强化，均可见液化腔；4例形状呈不规则，3例侵犯颅内、鼻旁窦、颞窝。24例泪腺MEC均进行了手术切除。病理证实24例均为高级别泪腺MEC，其中20例(83.3%)可见肿瘤侵犯神经，肿瘤细胞坏死，4个以上的核分裂/10个高倍视野；9例(37.5%)可见侵犯骨质。随访7例患者中1例复发(随访时间为12个月)，6例患者未复发(平均随访时间为24.3个月)。结论高级别泪腺MEC好发于中老年男性，常表现为无痛性眼球突出；影像大多表现为眶外上方中密度占位病变，形状不规则，常发生钙化，多伴有骨质破坏，可见液化腔，可侵犯邻近组织结构；治疗以手术切除为主，但需警惕术后复发和远处转移的发生。
- 泪腺 /
- 黏液表皮样癌 /
- 临床特征 /
- 病理 /
- 预后
Abstract: Background Mucoepidermoid carcinoma (MEC), which is a common malignant salivary gland neoplasm, is rarely seen in the lacrimal gland. More comprehensive information is therefore needed to be summarized to guide the diagnosis and treatment of the disease. Objective To explore the clinical features, image characteristics, diagnosis, treatment, and prognosis of lacrimal MEC. Methods The clinical image and pathological data of 24 patients with lacrimal gland MEC confirmed in the Third Medical Center of Chinese PLA General Hospital from January 2010 to December 2021 were retrospectively analyzed. Results The average age of the 24 patients was 56.35 years, and the male to female ratio was 7:1. Of the 24 cases, there were 21 cases (87.5%) of proptosis, 16 cases (66.7%) of supraorbital palpable mass, and 3 cases (12.5%) of periorbital pain. There were 19 cases of primary lacrimal gland MEC, 5 cases of postoperative malignant changing to MEC, and 6 cases of distant metastasis. Among the CT images of 10 cases of lacrimal MEC, 7 cases manifested as inhomogeneous with soft-tissue density, 9 were irregularly shaped, 7 showed signs of calcifications in tumors, and 7 showed bone destruction around. Of the 8 cases undergoing MRI, all showed soft-tissue mass with marked inhomogeneous enhancement and liquefaction necrosis, 4 cases were irregularly shaped, and 3 had intracranial, sinus, and temporal fossa invasion. All of these patients were treated with surgical excision. Pathology confirmed that all cases were high-grade MEC, of which 20 (83.3%) showed tumor invasion of nerves, tumor cell necrosis, and more than 4 mitoses/10 HPF. There were 9 cases (37.5%) with bony invasion. One of the 7 patients who were followed-up had recurrence (follow-up time was 12 months), the others did not (mean follow-up was 24.3 months). Conclusion High-grade MEC of the lacrimal gland is likely to occur in middle-aged and elderly men, often manifested as painless proptosis. The majority of images appear as extraorbital middle density occupying lesions with irregular shape, calcification and bone destruction, and a visible liquefied cavity that can invade adjacent tissue structure. Surgical resection is the main treatment, but postoperative recurrence and distant metastasis should be aware of.
- lacrimal gland /
- mucoepidermoid carcinomas /
- clinical characteristics /
- pathology /
- prognosis

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图 1 泪腺黏液表皮样癌的CT表现

A：眼眶水平位CT显示，右侧泪腺区占位局限于眶外壁和外侧直肌的三角间隙中，可见“楔形征”；B：眼眶水平位骨窗显示，左眶外壁骨质破坏，呈虫蚀样；C：眼眶水平位CT显示，左侧泪腺区软组织密度影，形状不规则，可见钙化斑，破坏周围骨质

Figure 1. CT images of MEC of the lacrimal gland

A: CT images in horizontal axial showed the “wedge sign” in the right lacrimal gland, which refers to a sign that the tumor was confined to the triangular space between the outer wall of orbital and the lateral rectus muscle; B: Bone window at orbital horizontal axial showed a worm-like bone destruction of the left outer wall of orbit; C: CT images showed an irregularly shaped soft tissue density shadow in the left lacrimal gland area, with visible calcified spots, which destroyed the surrounding bone

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图 2 泪腺黏液表皮样癌的MRI表现

A：眼眶水平位MRI (T1WI)增强结合脂肪抑制扫描显示，右侧泪腺区肿块不均匀强化，侵犯右眶外直肌，内部可见液化区；B：眼眶冠状位MRI (T1WI)增强联合脂肪抑制扫描显示，病灶侵犯左眶外上壁，左侧颞肌，左侧额部脑膜，呈不均匀强化

Figure 2. Manifestations of MEC of the lacrimal gland on MRI

A: Orbital axial MRI (T1WI) enhanced combined fat-suppressed scan showed that the mass in the region of right lacrimal gland was inhomogeneous enhanced, invading the right lateral rectus muscle, and liquify area was visible inside; B: Orbital coronal MRI (T1WI) enhanced combined fat suppression scan showed that the lesion invaded the left superolateral-orbital wall, left temporalis and left forehead meninges. Heterogeneous enhancement was visible in the lesion

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图 3 泪腺黏液表皮样癌的HE形态(200×)。肿瘤细胞主要由表皮样细胞和中间型细胞为主

Figure 3. HE of MEC of lacrimal gland (200×). HE staining showed that the tumor cells were mainly composed of epidermoid cells and intermediate cells

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表 1 24例泪腺黏液表皮样癌患者的临床和人口学特征(例，%)

Table 1. Clinical and demographic characteristics of the patients (n, %)

特征	泪腺黏液表皮样癌(n=24)
性别
女	3(12.5)
男	21(87.5)
年龄
＜20岁	0
20 ~ ＜40岁	3(12.5)
40 ~ 60岁	10(41.7)
＞60岁	11(45.8)
临床表现
眼球突出	21(87.5)
斜视	18(75.0)
眼球运动异常	18(75.0)
眶周疼痛	3(12.5)
眼睑水肿	15(62.5)
眼睑下垂	7(29.0)
视力下降	5(21.0)
眶周扪及肿物	16(66.7)
有压痛	4(16.7)

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表 2 7例随访患者的预后情况

Table 2. Prognosis of the 7 patients

患者	年龄/性别	既往手术史	治疗方式	随访时间/月	复发
1	32岁/男	无	完整手术切除 + 放疗	68	否
2	69岁/男	无	完整手术切除	12	是
3	65岁/男	泪腺多形性腺瘤手术史	完整手术切除 + 放疗	45	否
4	39岁/男	泪腺多形性腺瘤手术史	完整手术切除 + 放疗	8	否
5	62岁/男	无	完整手术切除	13	否
6	73岁/男	无	完整手术切除 + 放疗	9	否
7	49岁/男	泪腺多形性腺瘤手术史	完整手术切除 + 放疗	3	否

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