泪腺腺样囊性癌病例报告

腺样囊性癌有着二个矛盾的临床现象:(1)如发生于泪腺,属于常见病,几占泪腺恶瘤的1/2,而在眼外部位如涎腺等,却为罕见病;(2)后者为低度恶性,而在泪腺,却属最高度恶性。Henderson(1986)对本病作过广泛的社会调查,结论是他从来未治愈过,也从未见别人治愈过。发病部位不同,恶性程度如此悬殊,为什么?现将近年所见二例,报告讨论于下。     

泪腺腺样囊性癌的组织病理学特征

目的 分析泪腺腺样囊性癌的组织病理学特征,明确肿瘤侵袭相关的组织病理学特征与分型的关系.设计回顾性病例系列.研究对象经病理诊断证实的泪腺腺样囊性癌标本49例.方法 观察组织学形态特征并以WHO标准分型,根据Szanto法分级.免疫组化检查选用的一抗有S-100和CD34.主要指标各种组织病理学分型的特征,与肿瘤侵袭相关的病理学特征在各分级绀的阳性率,包括侵犯神经、脂肪、肌肉、血管内瘤栓等.侵犯神经在有无疼痛症状患者间的阳性率.结果 泪腺腺样囊性癌组织学病理分型分为管状、筛状和实性型3种,Szanto Ⅰ级19例,Ⅱ级9例,Ⅲ级21例.S-100和CD34分别标记肿瘤内的神经纤维和血管内皮.肿瘤侵犯神经表现为两种显微镜下形态,共22例,占45%,其中Ⅰ级中1例,Ⅱ级中6例,Ⅲ级中15例.Ⅰ级和Ⅲ级间侵犯神经的例数有统计学差异(P=0.001);Ⅰ级和Ⅲ级间侵犯神经的例数有统计学差异(P=0.000).分别有12例和7例显微镜下可见到肿瘤侵犯脂肪和肌肉,但三级间均无统计学筹异.仅1例可见静脉血管内瘤栓.有疼痛者32例,无疼痛者17例.各有15例和7例显微镜下可见肿瘤侵犯神经,两者比较无统计学差异(P=0.703).结论 泪腺腺样囊件癌中Ⅲ级比例较高.侵犯神经是该肿瘤重要的侵袭方式,也是特征性病理表现,在高分级的肿瘤中发生率较高.侵犯脂肪、肌肉以及血管内瘤栓发生率较低.疼痛症状与镜下肿瘤侵犯神经无明显对应关系.     

泪腺腺样囊性癌的放射治疗

泪腺腺样囊性癌(adenoidcysticcarcinoma,ACC)是泪腺恶性上皮性肿瘤中最常见的,也是恶性度最高的肿瘤。其发病率占泪腺上皮性肿瘤的25%～30%[1],占泪腺恶性上皮性肿瘤的41.7%[1],复发率达77.14%[26],远处转移率达37%[26],本病病程短,可经周围神经浸润性生长、血行及淋巴系统转移,预后不佳。目前多采用包括放化疗在内的综合治疗的方案。本文着重从放疗的角度对ACC治疗的具体方法加以阐述。     

泪腺腺样囊性癌的研究现状

泪腺腺样囊性癌是原发性泪腺上皮性肿瘤中最常见且恶性程度最高的肿瘤.近年来国内外对该病的组织学分类及病理学改变、主要临床表现、治疗手段有了新的认识;在细胞与分子生物学方面,对与泪腺腺样囊性癌的发生、侵袭性生长及转移、嗜神经性生长等特性相关的生物学指标的研究也取得了一些进展.就泪腺腺样囊性癌的研究现状做一综述.     

泪腺腺样囊性癌临床治疗进展

泪腺腺样囊性癌(adenoid cystic carcinoma,ACC)是最常见的泪腺恶性上皮肿瘤,单纯手术切除疗效不满意,易复发,预后差。我们总结近年来国内外报道的相关泪腺ACC的临床综合治疗手段,包括改进手术入路、采取手术前后化学治疗、放射治疗等综合治疗方案,特别还报告了青年泪腺ACC患者的治疗策略,并分析了原发于泪腺以外ACC的诊断与治疗,为眼科临床医生提供更广阔的思路。     

泪腺腺样囊性癌CT表现的研究

目的 分析泪腺腺样囊性癌常见的CT图像特征,从而探讨CT(computed tomography)影像对于泪腺腺样囊性癌的诊断价值.方法 病历来源为天津医科大学眼科中心及天津医科大学第二医院,经病理确诊的眼眶腺样囊腺癌39例.其中原发肿瘤20例,复发肿瘤19例,均为单眼发病,所有患者均行眼眶CT检查,获取水平及冠状扫描图像.结果 泪腺腺样囊性癌中原发肿瘤形状多为厚扁平状,贴附于眶壁增长,在体层像上呈锥形者10例、不规则形5例;复发肿瘤形状为不规则型8例、厚扁平状,贴附于眶外上壁向后增长者8例.原发与复发泪腺腺样囊腺癌的好发位置位于眶外上方分别为18例与11例.结论 CT可清晰显示肿瘤的位置,形状,范围、眶骨壁改变,对于泪腺腺样囊性癌的诊断和制定治疗方案具有重要指导意义.

Abstract：

Objective To analyze imaging characteristics of adenoid cystic carcinoma(ACC)of lachrymal gland on CT for the diagnosis of ACC in clinic. Methods Thirty-nine patients with ACC consisting of 20 cases of primary tumor and 19 cases of recurred tumors confirmed by pathology were collected from The Eye Centre of Tianjin Medical University and The Second Hospital of Tianjin Medical University. All the patients were unilateral, and examined by CT. The imaging characteristics of ACC on CT were analyzed. Results The most common shape of primary ACC included 10 cases of conical-shape, 5 cases of irregular-shape. The recurred cases contained 8 cases of irregular-shape, 8 cases of conical-shape. The primary and recurred ACC usually existed on superior-external side, with which there were 18 cases and 11 cases respectively. Conclusions CT examination can exactly reveal the shape, size, position, and ossa orbitale destruction of ACC and played an important role in the diagnosis of ACC.     

泪腺腺样囊性癌CT表现的研究

目的 分析泪腺腺样囊性癌常见的CT图像特征,从而探讨CT(computed tomography)影像对于泪腺腺样囊性癌的诊断价值.方法 病历来源为天津医科大学眼科中心及天津医科大学第二医院,经病理确诊的眼眶腺样囊腺癌39例.其中原发肿瘤20例,复发肿瘤19例,均为单眼发病,所有患者均行眼眶CT检查,获取水平及冠状扫描图像.结果 泪腺腺样囊性癌中原发肿瘤形状多为厚扁平状,贴附于眶壁增长,在体层像上呈锥形者10例、不规则形5例;复发肿瘤形状为不规则型8例、厚扁平状,贴附于眶外上壁向后增长者8例.原发与复发泪腺腺样囊腺癌的好发位置位于眶外上方分别为18例与11例.结论 CT可清晰显示肿瘤的位置,形状,范围、眶骨壁改变,对于泪腺腺样囊性癌的诊断和制定治疗方案具有重要指导意义.     

泪腺腺样囊性癌的超微结构观察

目的观察泪腺腺样囊性癌(adenoid cystic carcinoma,ACC)与正常泪腺的超微结构,明确肿瘤基底膜(basement membrance,BM)改变与病理类型的关系。方法收集泪腺ACC和正常泪腺标本各6例,行透射电子显微镜检查,观察不同病理分型ACC超微结构特点和BM形态的改变。结果正常泪腺的腺泡由腺上皮细胞和肌上皮细胞组成,细胞的基底面与结缔组织间有均匀的BM相隔。病理分型为管状型和筛状型的ACC肿瘤细胞由腺上皮和肌上皮细胞组成,排列紊乱,细胞染色质分布不均,深染;BM明显增厚,呈多层,互相交织成网状或不规则折叠,肿瘤细胞可伸出伪足侵入BM,使其中断溶解。实性型ACC细胞间界限不清,细胞外堆积大量溶解的细胞外基质,完整BM结构少见。结论 ACC的BM增厚、结构紊乱、溶解和中断可能是肿瘤侵袭能力的体现。阻断肿瘤细胞对BM的降解或减少相关酶的活性,有可能成为治疗或延缓肿瘤侵袭的新靶点。     

超声、CT、MRI对泪腺腺样囊性癌诊断价值比较

目的：探讨泪腺腺样囊性癌X线、超声、CT及MRI的影像学特征,以及各种检查方法对疾病诊断价值的比较。方法：回顾性分析1977－1999年经病理组织学确诊的泪腺腺样囊性癌46例,对X线（8例）、B超（39例）、CDI（7例）、CT（37例）、MRI（4例）的特征进行比较。结果：X线可以发现眼眶密度增高及眶骨破坏;B超可以发现眶内占位病变,且对病变内部的钙斑、液化腔等组织结构的显示良好;CDI可以提供肿瘤内部血液供应情况;CT可以揭示病变范围、生长方式、肿物与眶内正常结构的关系、眶骨及眶周结构的改变;MRI对于显示肿瘤颅内和颞凹蔓延,优于以上检查方法。结论：联合应用多种影像学检查方法,可以提高术前诊断率。     

关注泪腺腺样囊性癌诊断和治疗中需要注意的问题

泪腺腺样囊性癌是泪腺最为常见的恶性肿瘤之一,具有较高的复发率和死亡率.此病发病机制不明,预后较差.目前,泪腺腺样囊性癌尚无标准化的诊疗方案,在临床诊疗过程中也存在着一些问题.为了提高有关泪腺腺样囊性癌的诊疗质量,本文将从泪腺腺样囊性癌的流行病学、临床表现、影像学检查方法 的选择、诊断与鉴别诊断、病理组织学改变和免疫组织化学指标、治疗方案的选择及预后等方面加以述评,以期有助于临床医师加强对该病的认识,提高早期诊断率,减少误诊率.     

Comparison of biological behavior of lacrimal gland adenoid cystic carcinoma with high-grade transformation cells

AIM: To evaluate the differences between human lacrimal gland adenoid cystic carcinoma with high-grade transformation (LACC-HGT) primary cells cultured by high-grade transformation tissue and non-high-grade transformation (non-HGT) primary cells cultured by non-high-grade transformation tissue in proliferation, metastasis, drug susceptibility, and genes. METHODS: LACC-HGT primary cells were established by tissue block culture, and the 4th to 10th generation primary cells were selected as research objects. The cells were preliminarily identified by immunofluorescent staining. The differences between non-HGT and LACC-HGT primary cells in terms of proliferation, metastasis, and drug susceptibility were compared by cell counting kit-8 (CCK-8) assay, wound healing, and drug sensitivity experiments. Differentially expressed genes were screened using mRNA array. Gene expression was analyzed using real-time quantitative polymerase chain reaction (RT-qPCR). RESULTS: LACC-HGT primary cells were successfully cultured by tissue block culture. Immunofluorescence staining results showed that cytokeratin (CK) and CK7 expression levels were positive in LACC-HGT primary cells. CCK-8 results showed that the proliferation ability of LACC-HGT cells was significantly higher than that of non-HGT cells. Wound healing experiment showed that the migration ability of LACC-HGT cells was significantly higher than that of non-HGT cells. LACC-HGT cells were also less sensitive to cisplatin and paclitaxel than non-HGT cells. Compared with non-HGT cells, 9566 differentially expressed genes were found in LACC-HGT primary cells, of which 5162 were up-regulated and 4404 were down-regulated. The expression of N-acetylneuraminate pyruvate lyase (NPL), MARVEL domain containing 3 (MARVELD3), syntabulin (SYBU), and allograft inflammatory factor 1 (AIF1) was higher in LACC-HGT cells than in non-HGT cells, whereas that of periostin (POSTN) was lower. CONCLUSION: LACC-HGT primary cells have faster proliferation, stronger migration ability, and poorer sensitivity to chemotherapy drugs than non-HGT primary cells. The expression of mRNAs in non-HGT and LACC-HGT primary cells are significantly different. These features are speculated to be the reasons why high-grade transformation tissues exhibit higher malignant degree and poorer prognosis than their counterparts.     

Treatment of lacrimal gland adenoid cystic carcinoma: a systematic review and Meta-analysis

AIM: To evaluate lacrimal gland adenoid cystic carcinoma (LGACC) of prognosis in patients who underwent different treatment regimens. METHODS: We searched PubMed, EMBASE, and the Cochrane Library for studies done on the treatment of LGACC, between January 1987 and April 2022. A Meta-analysis was conducted to pool the 5-year overall survival rate (OR), and the 5-year recurrence rate (RR) and 5-year metastasis rate (MR) were assessed. RESULTS: The 30 studies involved 585 patients were included in the Meta-analysis. The pooled 5-year OR with surgery alone was 50%, the 5-year RR was 63%, and the 5-year MR was 34%. The pooled 5-year OR with surgery and adjuvant radiotherapy combined was 67% (95%CI 61%,73%), the 5-year RR was 41%, and the 5-year MR was 35%. The pooled 5-year OR with surgery and adjuvant chemoradiotherapy combined was 72% (95%CI 59%, 84%), the 5-year RR was 48%, and the 5-year MR was 36%. The pooled 5-year OR with surgery, intra-arterial cytoreductive chemotherapy, and adjuvant chemoradiotherapy combined was 78% (95%CI 68%, 89%), the 5-year RR was 15%, and the 5-year MR was 27%. CONCLUSION: Comprehensive treatment is more effective than surgery alone. Surgery combined with intra-arterial chemotherapy and adjuvant chemoradiotherapy seems to add value to the therapeutic effect of comprehensive treatment of LGACC but further high-quality research is required to validate this.     

Prognostic factors for lacrimal gland adenoid cystic carcinoma: a retrospective study in Chinese patients

AIM: To explore the prognostic factors for lacrimal gland adenoid cystic carcinoma (LGACC) in Chinese patients. METHODS: Clinical and histopathological data were reviewed in patients with pathologically confirmed LGACC. Local recurrence, metastasis, and disease-specific death were the main outcome measures. Univariate and multivariate analyses were performed by the Kaplan-Meier method and a Cox proportional hazard model. RESULTS: This retrospective cohort study included 45 patients with pathologically confirmed LGACC between January 2008 and June 2022. Tumor (T) classification (P=0.005), nodal metastasis (N) classification (P=0.018) and positive margin (P=0.008) were independent risk factors of recurrence; T (P=0.013) and N (P=0.003) classification and the basaloid tumor type (P=0.032) were independent risk factors for metastasis; T classification (P<0.001) was an independent factor of death of disease. In the further analysis, the durations from first surgery to radiotherapy is correlated with metastatic risk in LGACC patients with basaloid component (P=0.022). CONCLUSION: Histological subtype should be emphasized when evaluating prognosis and guiding treatment. Timely radiotherapy may reduce the risk of metastasis in patients with basaloid component.     

泪腺腺样囊腺癌的MRI特征分析

目的 探讨泪腺腺样囊腺癌（adenoid cystic carcinoma,ACC）的MRI特征,为临床早期诊断提供依据。设计 回顾性病例系列。研究对象 北京同仁医院经手术证实ACC患者26例,术前均行MRI扫描。方法 所有ACC患者常规MRI扫描;同时对动态增强磁共振成像（DCE-MRI）及磁共振扩散加权成像（DWI）图像进行后处理获得相应量化指标。主要指标 ACC动态增强的峰值强化指数（CIpeak）、峰值强化时间（Tpeak）、最大强化指数（CImax）、强化指数曲线（I型为持续上升型,II型为平台型,III型为流出型）、肿瘤平均表观扩散系数（ADC）值。结果  26例均起自泪腺眶部,20例形态不规则,14例边界不清,12例边界清晰,6例累及颅内,9例出现骨质破坏。T1WI呈等或略低信号,其中3例内部出现高信号区;T2WI呈等或稍高信号为主,增强后仅1例强化均匀,25例呈不均匀强化,其中16例伴有内部囊变、坏死区。泪腺ACC峰值强化指数（CIpeak）为0.9±0.41,峰值强化时间（Tpeak）为（109.3±58.5）s, 最大强化指数（CImax）为0.95±0.45。22例时间强化指数曲线中2例为I型,14例为II型,6例为III型。平均ADC值为（1.25±0.30×10-3）mm2·s－1。结论 MRI可以清晰显示泪腺ACC的部位、形态、范围、边界及骨质侵犯,DCE-MRI及DWI为肿瘤的诊断及鉴别诊断提供辅助量化特征。（眼科,2013, 22： 314-319）