唾液腺复发或转移性腺样囊性癌的治疗进展北大核心CSCD

腺样囊性癌是唾液腺恶性肿瘤中第二常见的病理亚型,初始治疗后极易发生复发和转移,且复发和转移患者的最佳治疗方案尚存在争议。近年来,针对复发和转移性腺样囊性癌的治疗进行了多种尝试,如放射治疗、靶向治疗、免疫治疗。本文对复发和转移腺样囊性癌的治疗进行梳理,总结各项治疗的作用及发展前景。     

唾液腺复发或转移性腺样囊性癌的治疗进展

腺样囊性癌是唾液腺恶性肿瘤中第二常见的病理亚型, 初始治疗后极易发生复发和转移, 且复发和转移患者的最佳治疗方案尚存在争议。近年来, 针对复发和转移性腺样囊性癌的治疗进行了多种尝试, 如放射治疗、靶向治疗、免疫治疗。本文对复发和转移腺样囊性癌的治疗进行梳理, 总结各项治疗的作用及发展前景。     

肺腺样囊性癌4例病例报告

背景与目的 肺腺样囊性癌是肺癌中的少见类型,通常对其诊断及治疗认识不足.为了提高对肺腺样囊性癌的认识,本文对该病例进行了收集和分析.方法 回顾分析我院2012年1月-2016年12月收治的4例肺腺样囊性癌,对其病理免疫组化、表皮生长因子受体(epidermal growth factor receptor,EGFR)及间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)突变分析、诊断和治疗特点进行总结.结果 肺腺样囊性癌是一种主要发生在气道、EGFR及ALK突变少见、转移较晚、手术治疗效果好的一种疾病.结论 肺腺样囊性癌诊断主要依赖于病理学,早期以手术治疗为主,晚期可行放化疗,但靶向治疗机会不多,其预后较小细胞肺癌及非小细胞肺癌好.     

Twist在涎腺腺样囊性癌中的表达及其临床意义

目的 探讨转录因子Twist在涎腺腺样囊性癌中的表达水平及其与各临床病理因素的关系.方法 采用免疫组化方法检测48例涎腺腺样囊性癌、18例多形性腺瘤和10正常腮腺组织中Twist蛋白的表达情况,分析Twist表达与腺样囊性癌临床病理因素的关系.结果 Twist在涎腺腺样囊性癌组织中的表达水平明显高于多形性腺瘤和正常腮腺组织(P<0.05).Twist表达与腺样囊性癌的病理分型、嗜神经侵袭、术后复发及远位转移有相关性(P<0.05).结论 Twist蛋白表达可能与涎腺腺样囊性癌的分化调节、嗜神经侵袭及远位转移有关.检测Twist的表达水平对判断腺样囊性癌患者的预后有一定的参考价值.     

对放疗敏感的腺样囊性癌1例

对放疗敏感的腺样囊性癌１例陈乐腺样囊性癌对放射线欠敏感，单纯放射治疗的疗效差，复发及转移的病例更难达根治，治疗多以手术为主，术后辅以放疗或化疗。但笔者治疗１例颌下腺腺样囊性癌术后１年多又复发并转移的患者，效果好，报道于此。患者张某，男，３８岁，以左颌...     

肾脏转移性腺样囊性癌1例临床病理分析

目的：阐明肾脏转移性腺样囊性癌临床病理特点。方法：回顾性分析1例肾脏转移性腺样囊性癌的临床表现、组织学和免疫组织化学及超微结构特点。结果：右侧腮腺腺样囊性癌根治术后13年发生肾脏转移,肿瘤位于左侧肾脏上极,切面灰白、细腻、质中,与周围组织分界清楚。组织学显示瘤细胞由腺上皮细胞和肌上皮细胞两种细胞构成,肿瘤组织呈条索状、腺囊状或筛网状排列,囊腔内充以嗜碱性黏液样物质,局部可见瘤栓及神经束侵犯。免疫组化结果显示两种肿瘤细胞vimentin阳性;腺上皮细胞CDI17阳性;肌上皮细胞actin、p63、34βE12阳性。电镜显示瘤细胞间具有腺腔,瘤细胞巢周围有完整或不完整的基板。结论：腺样囊性癌是一种生长缓慢的恶性上皮性肿瘤,易复发和远处转移,甚至可以转移至肾脏,对此类肿瘤患者应进行长年随访观察。     

肾脏转移性腺样囊性癌1例临床病理分析

目的:阐明肾脏转移性腺样囊性癌临床病理特点.方法:回顾性分析1例肾脏转移性腺样囊性癌的临床表现、组织学和免疫组织化学及超微结构特点.结果:右侧腮腺腺样囊性癌根治术后13年发生肾脏转移,肿瘤位于左侧肾脏上极,切面灰白、细腻、质中,与周围组织分界清楚.组织学显示瘤细胞由腺上皮细胞和肌上皮细胞两种细胞构成,肿瘤组织呈条索状、腺囊状或筛网状排列,囊腔内充以嗜碱性黏液样物质,局部可见瘤栓及神经束侵犯.免疫组化结果显示两种肿瘤细胞vimentin阳性;腺上皮细胞CD117阳性;肌上皮细胞actin、p63、34BE12阳性.电镜显示瘤细胞间具有腺腔,瘤细胞巢周围有完整或不完整的基板.结论:腺样囊性癌是一种生长缓慢的恶性上皮性肿瘤,易复发和远处转移,甚至可以转移至肾脏,对此类肿瘤患者应进行长年随访观察.     

唾液腺腺样囊性癌颈淋巴结转移规律的临床研究*

目的:本研究旨在回顾性分析头颈部腺样囊性癌的颈部淋巴结的临床转移规律。方法:回顾性分析1995年1 月至2008年12月就诊于上海交通大学医学院附属第九人民医院口腔颌面- 头颈肿瘤科的616 例腺样囊性癌患者的临床资料,对其中62例腺样囊性癌合并颈部淋巴结转移的患者进行临床统计学分析。结果:头颈部腺样囊性癌的颈淋巴结转移的发生率约为10% ,原发于舌根、舌体及口底部的腺样囊性癌较其他部位更易发生颈部淋巴结转移,分别为19.2% 、17.6% 和15.3% ,转移部位多发生在Ⅰb区与Ⅱ区。淋巴结转移的方式以经典的管道性转移为主,直接浸润型转移仅常见于下颌下腺的腺样囊性癌累及颌下淋巴结。原发部位及瘤周淋巴血管浸润与颈部淋巴结转移的发生密切相关。出现颈部淋巴结转移的患者其临床预后明显较差（P < 0.01）。结论:舌- 口底复合体为头颈部腺样囊性癌发生淋巴结转移的常见原发部位,在临床治疗及随访上应得到更多关注。      

纳武单抗联合放化疗治疗头颈部腺样囊性癌伴多发肝转移1例

目的：提供免疫治疗在头颈部腺样囊性癌中的应用，为晚期腺样囊性癌的治疗提供参考。方法：回顾性分析１例本院收治的头颈部腺样囊性癌伴多发肝转移的治疗过程，并查阅相关文献资料进行总结。结果：经原发头颈部腺样囊性癌扩大切除术后，拟行辅助放化疗，但在化疗前患者出现多发肝转移，经多学科诊疗模式讨论后行常规放化疗联合免疫的综合个体化方案。但在第５周期治疗后患者出现１型糖尿病而停止治疗，遂后未接受任何抗肿瘤治疗，仅门诊定期复查。目前患者病情平稳，原发病灶控制良好，肝转移病灶几乎完全缓解。结论：鉴于晚期头颈部腺样囊性癌缺乏具体的治疗共识，且出现多发肝转移的临床罕见，在常规放化疗的基础上联合免疫可能为晚期腺样囊性癌患者提供新的选择。     

头颈部腺样囊性癌的治疗进展

腺样囊性癌是涎腺常见的恶性肿瘤之一。目前,对于腺样囊性癌的标准治疗是手术为主和术后辅助放射治疗,化疗和分子靶向治疗对于腺样囊性癌治疗效果尚未肯定,文章主要介绍有关头颈部腺样囊性癌治疗的进展。     

Adenoid cystic carcinoma of the esophagus. Complete response to combination chemotherapy

A 55-year-old man had adenoid cystic carcinoma of the esophagus metastatic to the lungs and right supraclavicular fossa. He was treated with local radiation therapy to the esophagus and supraclavicular fossa, followed by combination chemotherapy with doxorubicin, mitomycin C, and 5-fluorouracil (5-FU). After a modest initial response, disease progression was noted in the pulmonary nodules. He was then treated with cisplatin, cyclophosphamide, vincristine, and doxorubicin. After two cycles of this regimen, there was complete regression of his pulmonary nodules, which was sustained for 5 months. A review of 44 literature cases of esophageal adenoid cystic carcinoma contrasted with adenoid cystic carcinoma of salivary gland origin indicated that the esophageal adenoid cystic carcinomas have a high tendency to metastasize (76% of cases) and a much poorer prognosis, with only 23% 1-year survival rate. It was concluded that esophageal adenoid carcinoma is clinopathologically distinct from the salivary gland variant, and that combination chemotherapy may be an effective treatment modality for this cancer.     

A phase II study of Imatinib for advanced adenoid cystic carcinoma of head and neck salivary glands

Adenoid cystic carcinoma of the salivary glands is characterized by a poor response to chemotherapy. Most cases of adenoid cystic carcinoma express the c-kit protein. Imatinib mesylate (Gleevec) inhibits several protein-tyrosine kinases, including c-kit. We therefore hypothesized that Imatinib may be an effective drug in patients with locally advanced or metastatic adenoid cystic carcinoma and conducted a phase 2 trial in order to study this. Patients with locally advanced or metastatic adenoid cystic carcinoma and c-kit positive tumours were eligible. Fourteen patients were screened and 10 patients (71%) with c-kit positive tumours entered the study. Treatment was begun at a dose of Imatinib of 400mg/day. Dose escalation was allowed in the absence of toxicity. The dose was increased to 600mg/d in three patients and 800mg/d in one patient. Three patients required dose reduction to 300mg/d, due to grade 3 toxicity. No grade 4 toxicity was seen. No objective responses were seen. Two patients (20%) exhibited stable disease for 11 and 14 months, respectively. All other patients stopped treatment after 2-14 (median 6) months due to progressive disease. Imatinib has no major effect on advanced adenoid cystic carcinoma of the head and neck.     

Adenoid cystic carcinoma of the salivary gland. Sustained complete response to chemotherapy

A patient developed widely metastatic adenoid cystic carcinoma of the parotid gland after several local recurrences. A complete regression of pleural, pulmonary parenchymal, cutaneous, and bone metastases was seen following therapy with 5-fluorouracil, Adriamycin, and mitomycin C. This is the second complete response to chemotherapy reported for a patient with metastatic adenoid cystic carcinoma of salivary gland origin. Both complete responses and several partial responses have followed treatment with Adriamycin, suggesting that this drug is active in the disease.     

晚期泪腺癌的临床治疗分析

目的:探讨临床少见、目前尚无标准治疗方案的晚期泪腺癌的临床表现及治疗方法,以提高对晚期泪腺癌诊治的认识.方法:回顾性分析中国医学科学院肿瘤医院2007年1月至2016年3月收治的6例晚期泪腺癌患者临床资料.结果:6例患者均为男性(33～66岁),中低分化腺癌4例、腺样囊性癌2例.泪腺肿瘤平均最长径为(3.3±0.5) cm,4例侵犯眶壁骨质.在泪腺癌病灶完全切除及放疗后,6例患者均出现远处转移,其中5例骨转移、4例肺转移、2例远处淋巴结转移、皮肤和肝转移各1例.6例患者均接受化疗,5例患者一线采用紫杉醇联合顺铂方案,1例患者一线采用顺铂联合替吉奥方案;治疗后仅1例获得缓解,2例稳定,3例进展.其中1例三线化疗失败的患者应用阿帕替尼治疗取得部分缓解,无进展生存期达6.1个月,不良反应包括高血压、手足综合征、腹泻和咯血,对症治疗后均好转.结论:分化较差和局部侵犯范围广的泪腺癌患者易发生远处转移,骨和肺为常见转移部位.紫杉醇联合顺铂方案化疗疗效欠佳,阿帕替尼用于晚期泪腺癌的治疗值得进一步研究.     

Adenoid cystic carcinoma of the nasopharynx: a case report]

Nasopharyngeal adenoid cystic carcinoma is a rare tumor, few cases have been reported in the literature. The aim of this report is to present a new case of an adenoid cystic carcinoma of the nasopharynx. We report the case of a 50 years-old woman presenting with an adenoid cystic carcinoma of the nasopharynx with diffuse pulmonary metastasis. The treatment combined chemotherapy, based on cisplatin and adriamycin drugs, and palliative radiation therapy at the dose of 30 Gy. The patient died 6 months after diagnosis establishment. The best treatment of adenoid cystic carcinoma is complete surgical resection but extended resection can be difficult in some locations as nasopharynx. The adjuvant radiation therapy seems to improve local control.     

Carcinome adénoïde kystique du nasopharynx,une entité clinique rare en quête d’innovations thérapeutiques : cas clinique et revue de la littérature

Nasopharyngeal adenoid cystic carcinoma is a rare tumour. Compared with others nasopharyngeal tumours, it is characterised by slow evolution but it is locally aggressive and has a high tendency to recurrences. Due to the rarity of cases, no consensus exists about treatment approaches. We report the case of 45-year-old-man with a locally advanced adenoid cystic carcinoma. The patient received concurrent chemoradiation and had a good objective response. After one year, he developed a paucisymptomatic lung metastasis. The follow-up showed local recurrence after 3 years. One cycle of chemotherapy was given but poorly supported. Carbon ion radiotherapy was proposed. The aim of this work is to review the literature concerning this rare malignancy and discusses treatment approaches in initial situations and during recurrences.     

Adenoid cystic carcinoma of the lung: Response to tamoxifen after chemoradiation

Primary adenoid cystic carcinoma of the lung is a rare subtype of lung cancer with a prolonged natural history but is difficult to completely resect surgically due to its proximal location. Hence radiotherapy plays a role for local control of these cancers. The role of systemic therapies in the treatment of adenoid cystic carcinoma of the lung remains unclear. Isolated case reports have previously described the treatment of primary adenoid cystic carcinoma of the lung with tamoxifen, resulting in partial control. We report the first case of primary adenoid cystic carcinoma of the lung treated with primary chemoradiotherapy due to unresectable location of the tumor, and disease stabilization with symptomatic response to tamoxifen following tumor recurrence. There is a need for further studies to investigate the role of anti‐hormonal therapies in the treatment of adenoid cystic carcinoma of the lung.     

5-Fluorouracil, cyclophosphamide, and vincristine for adenoid cystic carcinoma of the head and neck

Twenty-one patients with adenoid cystic carcinoma of the head and neck were treated with intravenous boluses of cyclophosphamide and vincristine and 5-day continuous intravenous infusions of 5-fluorouracil (CVF) every 4 weeks. Eight patients received CVF as palliation for recurrent or metastatic disease. A sustained complete response (107+ months) was observed in one patient; one partial response and one mixed response each also were observed. In four patients disease stabilized, and in one disease progressed. Thirteen patients received six courses of CVF in the adjuvant setting after surgery and radiation for either primary or locoregional recurrent disease. Recurrences developed in two of seven patients with primary disease and three of six patients with recurrent disease with a median follow-up 45 months (range, 20-108+). Recurrence rate and time interval to recurrence were comparable to those of well-matched historical controls. Distant metastases have not developed in patients treated with CVF in the adjuvant setting, whereas distant metastases had developed in historical controls within comparable periods of follow-up. Serious toxicities were not encountered in any patient. The authors conclude that CVF is a well-tolerated combination chemotherapy program with activity in adenoid cystic carcinoma of the head and neck. This regimen, however, has not had a major impact in the adjuvant setting in preventing recurrent disease.