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 Metaanalysis 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 intraarterial 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.

阿帕替尼治疗腺样囊性癌肺转移1例

腺样囊性癌(adenoid cystic carcinoma,ACC)是一种较少见的来源于腺体的恶性肿瘤,约占头颈部恶性肿瘤的1%[1]。其病程进展缓慢但极易侵袭,有较高的复发率和远处转移率,最常见的转移部位是肺[2]。对于ACC肺转移患者,目前并无特效药物。MYB基因表达失调是ACC患者最常见的突变。

Expression of Innate Immunity Genes in Epithelial Cells of Hypertrophic Adenoids with and without Pediatric Chronic Rhinosinusitis： A Preliminary Report

Background： Adenoid hypertrophy （AH） is associated with pediatric chronic rhinosinusitis （pCRS）, but its role in the inflammatory process of pCRS is unclear. It is thought that innate immunity gene expression is disrupted in the epithelium of patients with chronic rhinosinusitis （CRS）, including antimicrobial peptides and pattern recognition receptors （PRRs）. The aim of this preliminary study was to detect the expression of innate immunity genes in epithelial cells of hypertrophic adenoids with and without pCRS to better understand their role in pCRS. Methods： Nine pCRS patients and nine simple AH patients undergoing adenoidectomy were recruited for the study. Adenoidal epithelium was isolated, and real-time quantitative polymerase chain reaction （RT-qPCR） was employed to measure relative expression levels of the following messenger RNAs in hypertrophic adenoid epithelial cells of pediatric patients with and without CRS： Human β-defensin （HBD） 2 and 3, surfactant protein （SP）-A and D, toll-like receptors 1-10, nucleotide-binding oligomerization domain （NOD）-like receptors NOD 1, NOD 2, and NACHT, LRR and PYD domains-containing protein 3, retinoic acid-induced gene 1, melanoma differentiation-associated gene 5, and nuclear factor-riB （NF-KB）. RT-qPCR data from two groups were analyzed by independent sample t-tests and Mann-Whitney U-tests. Results： The relative expression of SP-D in adenoidal epithelium ofpCRS group was significantly lower than that in AH group （pCRS 0.73 ± 0.10 vs. AH 1.21 ±0.15; P = 0.0173, t = 2.654）. The relative expression levels of all tested PRRs and NF-κB, as well as HBD-2, HBD-3, and SP-A, showed no statistically significant differences in isolated adenoidal epithelium between pCRS group and AH group. Conclusions： Down-regulated SP-D levels in adenoidal epithelium may contribute to the development of pCRS. PRRs, however, are unlikely to play a significant role in the inflammatory process ofpCRS.

Anatomy and physiology of the palatine tonsils, adenoids, and lingual tonsils

Objective::This review aims to discuss the basic anatomy and physiology of the palatine and pharyngeal tonsils,with reference to how this foundational understanding may affect patient management and surgical procedures in these regions of the upper airway.Methods::A literature search was performed using PubMed and Google Scholar using the MeSH terms tonsils,adenoids,anatomy,physiology,and adenotonsillectomy.Primary sources were excluded if they were abstracts only,non-English language,or non-human studies.Thirty-five sources were included in this review.Results and conclusions::The pharyngeal and palatine tonsils are compact yet physiologically complex mucosa-associated lymphoid tissues that make up a portion of Waldeyer’s ring.As part of the mucosal immune system,these structures function in exogenous antigen sampling and stimulation of immune responses.Aberrant immune activation and/or regulation can lead to a myriad of pathologies,with adenotonsillar hypertrophy,chronic tonsillitis/adenoiditis,and recurrent otitis media among the most commonly encountered conditions by otolaryngologists.While the pathophysiology of these conditions is still incompletely understood,current evidence and future investigations may reveal patterns amenable to targeted medical management.When medical management fails,tonsillectomy and/or adenoidectomy may be indicated for patient care.Though routine procedures,the execution of tonsil and/or adenoid removal requires a thorough understanding of the anatomy of these lymphoepithelial organs so as to minimize the risk for rare serious complications that can occur.

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.

Optimizing surgical approaches for lacrimal gland adenoid cystic carcinoma to minimize cross-organ invasion

AIM:To evaluate the outcomes of eye-sparing surgery for lacrimal gland adenoid cystic carcinoma and the impact on tumor recurrence and orbital integrity.METHODS:The study enrolled four patients with recurrent lacrimal gland adenoid cystic carcinoma.The outcome focused on the relevance of the integrity of the lateral orbital wall to the occurrence of extraorbital metastasis in the local recurrence of lacrimal gland adenoid cystic carcinoma.RESULTS:Three patients underwent eye-sparing surgery via lateral orbitotomy without postoperative radiotherapy,and one patient who underwent eye-sparing surgery via sub-brow approach.These four patients all demonstrated a recurrence involving the invasion of extraorbital tissues as metastatic form through surgical bone seams.CONCLUSION:Preserving intact orbital bone tissue is crucial for mitigating direct cross-organ metastasis of lacrimal gland adenoid cystic carcinoma.The findings suggest avoiding the lateral orbitotomy approach with no or limited orbital bone wall invasion.

Rare esophageal carcinoma-primary adenoid cystic carcinoma of the esophagus: A case report

BACKGROUND Esophageal adenoid cystic carcinoma(EACC)is an exceedingly rare malignant tumor of the esophagus,posing significant challenges in the clinic.CASE SUMMARY This report detailed the case of a 72-year-old male whose diagnosis of EACC was confirmed through postoperative histopathological examination.The patient underwent thoracoscopy-assisted radical resection of the esophageal tumor,coupled with lymph node dissection.Pathological findings revealed an adenoid cystic carcinoma infiltrating the entire layer of the muscularis propria,locally extending into the outer membrane of the esophageal fiber,involving the cardia and exhibiting no lymph node metastasis.The patient’s condition was classified as primary EACC,T3N0M0,per the American Joint Committee on Cancer(2017;8th edition).One month after surgery,the patient received postoperative adjuvant radiation therapy.CONCLUSION In addressing the rarity and high potential for biopsy misdiagnosis of EACC,this study delved into its diagnostic methods and treatment.

Anal metastasis in esophageal cancer:A case report

BACKGROUND Esophageal cancer is the sixth leading cause of cancer-related death and eighth most common cancer,affecting>450000 people worldwide.Esophageal squamous cell carcinoma is the most common histological type,whereas esophageal adenoid cystic carcinoma(EACC)is rare.The liver is the most common distant metastatic site in esophageal cancer.Anal metastasis is rare and has not been reported in clinical practice before.Here,we report anal metastases in a patient with EACC after regular chemotherapy and surgical resection.CASE SUMMARY A 61-year-old esophageal cancer patient was found to have lung and brain metastases during standardized treatment.The patient’s treatment plan was continuously adjusted according to the latest treatment guidelines.However,the patient subsequently noticed rectal bleeding and itching,and after obtaining pathology results at the local hospital,anal metastasis of esophageal cancer was diagnosed.CONCLUSION Postoperative pathology and immunohistochemistry confirmed EACC with rare anal metastasis.More exploration of EACC diagnosis and treatment is needed.

Ethmoidal Polyposis, Adenoid Hypertrophy and Tympanic Membrane Perforation—A Case Report

Background: Ethmoidal polyposis coexisting with adenoid enlargement and tympanic membrane perforation is a rare condition in otolaryngological practice. Evidences have shown that antrochoanal polyp often times coexists with adenoid enlargement especially in the young which was in sharp contrast to ethmoidal polyposis occurring in the presence of adenoid enlargement. Moreover, the hidden location of nasopharynx housing the adenoids, coupled with the masking effect of bilateral sinonasal polyps, leads to inadvertent misdiagnosis of this complex pathology with subsequent difficulty in providing effective management. Case Presentation: A 24 year-old lady presented with recurrent bilateral nasal obstruction that became persistent associated with nasal discharge and anosmia. She also had recurrent right otorrhoea with associated hearing loss. Diagnostic rigid nasal endoscopy revealed mucoid discharge with bilateral polypoid masses filling both nasal cavities. Otoendoscopic finding revealed a small (about 5%) central tympanic membrane perforation. A clinical assessment of chronic rhinosinusitis with nasal polyposis complicated by chronic suppurative otitis media (CSOM) was made. A non-contrast CT scan of the paranasal sinuses showed isodense lesions in the nasal cavities, all paranasal sinuses and the entire nasopharynx. She had endoscopic sinus surgery and a nasopharyngeal clearance biopsy. The nasal, paranasal and nasopharyngeal masses had histologic confirmation of inflammatory nasal polyps and lymphoid (adenoid) hyperplasia respectively. Her condition improved remarkably with subsequent medical treatment. She was followed up for 8 months and no recurrence was observed. Conclusion: Sinonasal polyposis can coexist with adenoid hypertrophy and middle ear disease as a single pathological condition. Hence, a high index of suspicion and thorough evaluation become necessary for making timely diagnoses and instituting effective management.

Development patterns of adenoids in Chinese children without sleep-disordered breathing: a retrospective magnetic resonance imaging study with consecutive age groups

To the Editor:Sleep-disordered breathing(SDB)is a health problem affecting approximately 12%of children in China,[1]characterized by habitual loud snoring and increased respiratory effort.Increased upper airway resistance related to enlarged adenoids has been identified as the most common cause in children with obstructive SDB,and adenoidectomy is the standard treatment for the disorder.Similar to lymphoid tissues,adenoids show a unique natural growth pattern,which incorporates both overgrowth and involution.However,the development patterns of adenoids have not been clearly elucidated.Since the adenoids are located in the nasopharynx roof,direct evaluation of their size and proportion to the upper airway area is difficult.Thus,radiological methods have been employed widely for such purposes,including traditional plain cranial radiographs,computed tomography(CT),cone-beam CT,and magnetic resonance imaging(MRI).MRI can reveal the accurate size of adenoids by differentiating them from other soft tissue structures without any ionizing radiation;therefore,it can effectively study the adenoids’developmental pattern in normal children.Previous studies have evaluated age-associated changes in adenoid size by MRI.Arens et al[2]indicated that adenoids grow proportionally to the skeletal structures in children aged 1 to 11 years.Papaioannou et al[3]reported that head MRI examinations demonstrated increased adenoid size during the first 7–8 years of life and then decreased gradually in children without snoring signs.Variabilities of the above-reported adenoids’development pattern exist due to the limitations associated with the small sample size.Furthermore,none of the above-mentioned studies on normal children used a strict method to exclude SDB.Additionally,for adenoid hypertrophy,the adenoid/nasopharyngeal(A/N)ratio was found to be a useful,tolerable,and confident diagnostic method in pediatric patients,with emphasis on the correlation of the adenoid size and upper airway lumen to variable degrees.Therefore,the A/N ratio can be considered a useful objective tool in evaluating adenoid development in children.We aimed to investigate the growth and involution patterns of adenoids in consecutive age groups from birth to 16 years in Chinese children without SDB using MRI data,which may benefit the study of diseases associated with the development of adenoids.

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)primar y cells cultured by high-grade transformation tissue and non-high-grade transformation(non-HGT)primary cells cultured by non-highgrade transformation tissue in proliferation,metastasis,drug susceptibility,and genes.METHODS:LACC-HGT primary cells were established by tissue block culture,and the 4^(th)to 10^(th)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 LACCHGT 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 upregulated 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 LACCHGT 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.

Preliminary proteomic analysis of human tears in lacrimal adenoid cystic carcinoma and pleomorphic adenoma

·AIM: To detect proteomic differences in tears between adenoid cystic carcinoma(ACC) and pleomorphic adenoma(PA).·METHODS: Tear samples were collected from 4 patients with ACC, 5 with PA, and 4 control cases. Label-free analysis and parallel reaction monitoring(PRM) were used to screen and validate the tear proteome. Gene Ontology(GO) annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) were conducted for bioinformatics analysis.·RESULTS: In total, 1059 proteins in tear samples were identified by label-free analysis. Between ACC and PA, 415 differentially expressed proteins were detected. Based on the GO annotation, enzyme regulator activity and serinetype endopeptidase inhibitor activity in the molecular function category, blood microparticle and extracellular matrix in the cellular component category, and response to nutrient levels in the biological process category were most predominant. By KEGG pathway annotation, the different proteins between ACC and PA mainly par ticipated in complement and coagulation cascades, amoebiasis, African trypanosomiasis and cholesterol metabolism. Eight proteins with mostly significant differences were verified by PRM, and five proteins with more than 10-fold increases in ACC compared with PA, including integrin β, α-2-macroglobulin, epididymal secretory sperm binding protein Li 78p, RAB5C, and complement C5, were identified.·CONCLUSION: The combined tools of label-free analysis and PRM are very effective and efficient, especially for samples such as tears. Some proteomic dif ferences in tears between ACC and PA are identified and these protein candidates may be specific biomarkers for future exploration.

TACC diagnosed by transoesophageal endoscopic ultrasonography:A case report

BACKGROUND Primary adenoid cystic carcinoma in the trachea(TACC)is a rare tumour.Tracheal bronchoscopy is always chosen as a routine approach to obtain a pathological diagnosis,but it can be associated with an increased risk of asphyxia.CASE SUMMARY We describe a case of TACC in a patient evaluated by chest computed tomography(CT)with three-dimensional reconstruction imaging and diagnosed by transoesophageal endoscopic ultrasonography.The pathological diagnosis confirmed tracheal adenoid cystic carcinoma.CONCLUSION We highlight the importance of CT and provide a successful exploration of transoesophageal biopsy as a safe alternative approach.

腺样体肥大儿童伴变应性鼻炎与分泌性中耳炎的相关性

分泌性中耳炎影响患儿听力,甚至影响语言、交流能力发育[1],若不能进行合理治疗,可能会造成儿童听力障碍以及言语与治疗发育不良。近几年临床研究显示,该疾病与儿童腺样体肥大以及变应性鼻炎存在一定关系。本文选择我院曾接收的存在腺样体肥大病史的患儿500例,对其变应性鼻炎、咽鼓管功能不良以及分泌性中耳炎相关性进行分析。1.1资料来源。2012年1月~2012年12月我院住院的腺样体肥大患儿500例,腺样体肥大程度为3~4度阻塞。

上颌窦气化程度与腺样体肥大相关性分析

上颌窦(maxillary sinus)是颌面部的重要解剖结构。很多研究表明成人上颌窦形态与睡眠呼吸暂停综合征密切相关[1]。一般认为其气化程度受遗传因素影响,也有学者研究同卵双生的双胞胎其上颌窦容积亦有差别,说明后天因素在气化过程中亦有重要作用。上颌窦形态异常造成的睡眠呼吸暂停综合征很难通过手术或其他方式来改善,因此发现影响上颌窦气化的因素并早期加以干预,具有重要临床意义。在临床工作中,

腺样体肥大患儿机体免疫功能变化研究

<正>腺样体也称咽扁桃体或增殖体,属于淋巴组织,表面呈桔瓣样。正常生理情况下,儿童6~7岁时腺样体发育至最大,青春期后逐渐萎缩,成人后基本消失~[1]。腺样体位于鼻咽顶后壁处,病理性肥大易引起鼻堵、张口呼吸、耳闷、听力下降等症状,最终造成慢性鼻-鼻窦炎、阻塞性睡眠呼吸暂停低通气综合征及分泌性中耳炎等疾病,给儿童的颌面骨发育、智力发育造成严重影响。

阻塞性腺样体和扁桃体肥大与胰岛素样生长因子系统

阻塞性腺样体和扁桃体肥大可能引起儿童生长发育异常,其病理生理机制尚不明确。本文就血清胰岛素样生长因子-1(insulin-likegrowthfactorI,IGF-1)/胰岛素样生长因子结合蛋白-3(insulin-likegrowthfactor-bindingprotein3,IGFBP-3)概况及其在阻塞性腺样体和扁桃体肥大患儿生长激素分泌异常诊断和疗效判断方面的意义进行综述。

头颈部腺样囊性癌48例

腺样囊性癌(adenoid cystic carcinoma简称ACC)是涎腺常见的恶性肿瘤之一,多见于涎腺,也可发生于头颈其他部位。为了提高对本病的认识,根据我院治疗的病例,结合近年文献,对其临床特征、诊断和治疗等方面进行讨论。

抑癌基因与腺样囊性癌关系的研究进展

腺样囊性癌（adenoid cystic carcinoma,ACC）可发生于涎腺、泪腺、汗腺、乳腺以及上颌窦内、气管支气管等部位内壁的腺体等全身各部外分泌腺组织,因好发于大小涎腺中较小腺体,近年对涎腺来源的腺样囊性癌研究较为深入。腺样囊性癌生长速度缓慢,但由于其嗜神经血管生长及易远处转移的特点,使得腺样囊性癌易复发、早期转移率高,需要随访的周期较长。