EZH2 immunoexpression in pleomorphic adenoma and adenoid cystic carcinoma and clinicopathological features

Abstract The aim of this study was to evaluate the expression of the EZH2 protein and describe the clinical and microscopic characteristics of adenoid cystic carcinoma (ACC) and pleomorphic adenoma (PA). The study included 16 ACC cases and 12 PA. All ACC and PA cases were positive for EZH2 and the ACC samples showed significantly higher EZH2 expression. The clinical and microscopic covariates were described in relation to EZH2 staining in ACC samples. The highest mean values of EZH2 were observed in cases with local metastasis, recurrence, perineural invasion, and predominantly cribriform growth pattern without solid areas. EZH2 is a potential marker of malignancy.

Reliability of nasofibroscopy for the evaluation of adenoid hypertrophy and its correlation with clinical symptoms

Abstract Objective: To analyze the reliability of estimating the percentage of rhinopharyngeal obstruction by the adenoid using nasofibroscopy and its correlation with clinical symptoms. Methods: Cross-sectional observational study was conducted, involving 80 patients between 4 and 14 years old, recruited from Santa Casa of São Paulo general otorhinolaryngology outpatient service during the years of 2020 and 2021. All patients underwent nasal endoscopy examination, and the recorded videos were randomly assigned to four evaluators in two different sessions, with a minimum interval of 1 month. The evaluators estimated the percentage of rhinopharyngeal obstruction caused by the adenoid. Intra- and inter-evaluator correlations were established by comparing the reports from each evaluator. The data were compared to the Pro Image J Software report, that also estimates a percentage of obstruction by computer graphics. To correlate the grading of obstruction with clinical symptoms, all patients completed the OSA (Obstructive Sleep Apnea) 18 questionnaire, a validated tool for assessing sleep apnea in children. The questionnaire data were then compared to the average scores assigned by the evaluators. Results: Satisfactory intra- and inter-rater correlations were observed, and the results were consistent with the Pro Image J Software. However, no correlation was found between the percentage of obstruction and the severity of clinical symptoms. Conclusion: Nasofibroscopy demonstrates good reliability in assessing a percentage of rhinopharyngeal obstruction caused by adenoids. However, there is no correlation between the degree of obstruction of the rhinopharynx and the clinical symptoms. Level of evidence: 4.

Clinical and radiologic outcomes of pleomorphic adenoma and adenoid cystic carcinoma of the lacrimal gland / Desfechos clínicos e radiológicos do adenoma pleomórfico e do carcinoma adenoide cístico da glândula lacrimal

ABSTRACT Purpose: To compare the radiologic and clinical features of primary lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Methods: This study retrospectively reviewed imaging findings and medical records of lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Results: Eleven patients with pleomorphic adenoma and 16 patients with adenoid cystic carcinoma were evaluated. There were no statistically significant differences between groups regarding age or sex. Proptosis was the most common presenting symptom in both groups. Adenoid cystic carcinomas were more likely to present with a palpable mass, diplopia, pain, and sensory loss than pleomorphic adenomas, although the differences were not statistically significant between groups. Furthermore, there were no significant differences in terms of homogeneity and globe indentation between lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma on computed tomography (CT)(all p>0.05). The rates of bone invasion, tumor calcification, and wedge sign were significantly higher in adenoid cystic carcinomas, and bone remodeling was statistically significantly higher in pleomorphic adenomas, on CT(all p<0.05). Pleomorphic adenomas were significantly more likely to show well-defined margins, lobulated contours, heterogeneous contrast enhancement, and hyperintensity on T2-weighted magnetic resonance images (all p<0.05). Conclusion: When differentiating between lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma, evaluation of radiologic features along with clinical features is of great importance. Lobulated contours may be a significant distinguishing radiologic feature suggesting pleomorphic adenoma.

RESUMO Objetivo: Comparar as características radiológicas e clínicas do adenoma pleomórfico primário e do carcinoma adenoide cístico da glândula lacrimal. Métodos: Este estudo revisou retrospectivamente os achados de imagem e os prontuários médicos de casos de adenoma pleomórfico e carcinoma adenoide cístico da glândula lacrimal. Resultados: Foram avaliados 11 pacientes com adenoma pleomórfico e 16 pacientes com carcinoma adenoide cístico. Não houve diferenças estatisticamente significativas em relação à idade e sexo. Proptose foi o sintoma de apresentação mais comum em ambos os grupos. Os carcinomas adenoides císticos foram mais propensos que os adenomas pleomórficos a apresentarem massas palpáveis, diplopia, dor e perda sensorial, mas essa diferença entre os grupos não foi estatisticamente significativa. Não houve diferenças estatísticas em termos de homogeneidade e indentação do globo ocular entre os dois tipos de tumores em imagens de tomografia computadorizada (p>0,05). Também à tomografia computadorizada, a invasão óssea, a calcificação do tumor e o sinal em cunha foram mais frequentes nos carcinomas adenoides císticos, enquanto a remodelação óssea foi mais frequente nos adenomas pleomórficos, com significância estatística para todas essas manifestações (p<0,05). À ressonância magnética, os adenomas pleomórficos foram significativamente mais propensos a terem margens bem definidas, contornos lobulados, realce heterogêneo pelo contraste e hiperintensidade na ressonância magnética ponderada em T2 (p<0,05). Conclusão: Ao se diferenciar o adenoma pleomórfico e o carcinoma adenoide cístico da glândula lacrimal, é muito importante avaliar as características radiológicas juntamente com as características clínicas. Os contornos lobulados podem ser uma característica radiológica significativamente distinta em favor do adenoma pleomórfico.

A Comparative Study of Fluticasone Propionate, Mometasone Furoate, and Saline Nasal Spray in the Treatment of Children with Adenoid Hypertrophy

Introduction:Adenoidectomy is currently considered the treatment of choice for relief of the nasal airway obstruction due to adenoid hypertrophy. Evidence suggests that topical nasal steroid sprays can cause a reduction in adenoid size. We aim to compare the effectiveness of fluticasone propionate, mometasone furoate (MF) and saline nasal sprays in relieving the signs and symptoms of adenoid hypertrophy and in reducing the size of the adenoids. MaterialsandMethods: We conducted a randomized comparative study on 60 patients divided into three groups A, B, C (20 each). Group A patients treated with fluticasone propionate nasal spray (400 μg/day), Group B patients treated with MF nasal spray (100 μg/day), and Group C patients treated with saline spray (0.65% w/v in purified water which is made isotonic and buffered). Treatment was given up to 12 weeks with follow‑up at 4, 8, and 12 weeks and at each follow‑up visit assessment was done. Final data were analyzed using SPSS software version 21 and numerical variables associated with different groups were analyzed and analysis of variance test was used. Results: Diagnostic nasal endoscopy and X‑ray grades at day 1 among the study groups were not statistically significant, whereas, at 12 weeks results among fluticasone and mometasone groups were significantly better (P < 0.001) as compared to the saline group. There was a significant improvement in the symptoms under all the categories with the use of fluticasone and mometasone. Conclusion: In our study, both fluticasone propionate and MF were able to effectively reduce symptoms and signs of adenoid hypertrophy as well as help in reducing the size of the enlarged adenoid. Both these drugs were well tolerated by the patients

Salivary gland tumor: atypical presentation of breast cancer

Breast cancer is a heterogeneous disease with various histological and molecular subtypes. Among them, salivary gland tumors are rare and can be divided into three groups: pure myoepithelial differentiation, pure epithelial differentiation and myoepithelial with mixed epithelial differentiation. In the last group, adenoid cystic carcinoma stands out, a rare entity with low malignant potential. It represents less than 0.1­3% of breast cancer cases and has the most frequent clinical presentation as a palpable mass. The diagnosis is confirmed by histology and immunohistochemistry. Classically, they are low-aggressive triple-negative tumors, with overall survival and specific cancer survival at five and ten years greater than 95%. However, there are rare reports of aggressive variants with a risk of distant metastasis and death. Treatment is based on surgical resection with margins. Lymphatic dissemination is rare, and there is no consensus regarding the indication of an axillary approach. Adjuvant radiotherapy is indicated in cases of conservative surgery and should be discussed in other cases. The benefit of chemotherapy remains uncertain, as most tumors are indolent. We report a case that required individualized decisions based on its peculiarities of presentation, diagnosed in an asymptomatic elderly patient during screening, in which mammography showed heterogeneous gross calcifications clustered covering 1.6 cm. Stereotacticguided vacuum-assisted biopsy was performed, and the area was marked with a clip. The anatomopathological examination led to a diagnosis of salivary gland-type carcinoma, triple-negative. The patient underwent segmental resection of the right breast and sentinel lymph node biopsy. The final anatomopathological result was similar to that of the biopsy, with an immunohistochemicalprofile of the adenoid cystic type and two sentinel lymph nodes free of neoplasia. Considering age and histological subtype, adjuvant therapy was not indicated. Follow-up for three years showed no evidence of disease

Clinical Diagnosis and Treatment of Lacrimal Gland Adenoid Cystic Carcinoma / 肿瘤防治研究

Lacrimal gland adenoid cystic carcinoma (LGACC) is the most common primary malignant epithelial tumor of the lacrimal gland and has the characteristics of high recurrence rate, neurotropic growth, and risk of distant metastasis. The clinical manifestation is mainly a mass in the lacrimal gland area with a poor specificity. The combination of MRI and CT is important in the diagnosis of LGACC, and PET/CT is of great value in determining the nature and metastasis of LGACC. Histopathological examination is the gold standard for diagnosis, and the type of histopathology is significant for predicting the prognosis of patients. At present, the most commonly used treatment for LGACC is surgical resection combined with postoperative local radiotherapy. However, the overall prognosis is poor after active treatment because the pathogenesis of LGACC remains unclear. This article will combine relevant literature and the author's clinical experience to review the epidemiology, clinical manifestations, imaging examination, histopathological changes, treatment, and prognosis of LGACC. Results will contribute to improve the quality of diagnosis and treatment and the prognosis of patients.

Progress of molecular targeted therapy for adenoid cystic carcinoma of salivary gland / 肿瘤研究与临床

Adenoid cystic carcinoma (ACC) is a salivary gland malignant tumor which mainly occurs in the head, neck and the mammary gland, and is characterized with easy invasion of peripheral nerves, local easy recurrence after resection and painless distant metastasis. The treatment of ACC is usually based on surgical resection combined with postoperative radiotherapy. Patients with advanced disease usually cannot be cured, so far there is no ideal treatment regimen. Some studies have suggested that some targeted drugs show advantages in ACC patients who are insensitive or resistant to conventional therapy. This paper summarizes the research progress of molecular targeted therapy for ACC of salivary gland in order to provide new treatment options for ACC patients especially for those at advanced stage.

Effects of ICMT gene silencing on the invasion and migration of human salivary adenoid cystic carcinoma cells in vitro / 口腔疾病防治

Objective @#To investigate the effect of isoprene cysteine carboxymethyltransferase (ICMT) gene on the migration and invasion of salivary adenoid cystic cancer cells (SACC) and the related mechanism, to provide experimental evidence for molecular targeted therapy of SACC.@*Methods@# Adenoid cystic cancer cells SACC-LM and SACC-83 were cultured in vitro, and siRNA was transfected into human SACC-LM and SACC-83 cells (experimental group) by transient transfection of a liposome vector. A blank control group and negative control group were set up respectively (transfected NC-siRNA). qRT-PCR was peformed to measure the mRNA expression of ICMT and RhoA in each group after transfection and to determine the silencing efficiency. The expression of ICMT, membrane RhoA, total RhoA, matrix metalloproteinase-2 (MMP-2), matrix metalloproteinase-9 (MMP-9) and Rho associated with coiled helical binding protein kinase 1 (ROCK1) in each group was detected by Western blot. The proliferation abilityies of SACC cells was detected by CCK-8 assay. The migration and invasion ability of SACC cells were detected by comparing the relative healing area of cell scratch assay and the number of Transwell assay cells. @*Results@#After transfection of ICMT-siRNA into SACC-LM and SACC-83 cells, the expression of ICMT gene and protein in the experimental group was significantly decreased compared with the negative control group and blank control group (P<0.05), but there were no significant differences in the expression of RhoA gene and total protein among all groups (P>0.05). The expression of RhoA membrane proteins, ROCK1, MMP-2, MMP-9 in the experimental group was significantly decreased compared with that in the negative control group and blank control group (P<0.05). Cell proliferation ability was significantly decreased (P<0.05). The migration and invasion abilities were significantly decreased (P<0.05). @*Conclusion @#In vitro silencing of ICMT gene can effectively inhibit the migration and invasion of human SACC-LM and SACC-83 cells, and the mechanism may be related to RhoA-ROCK signaling pathway.

The outcome of transnasal endoscopic total maxillectomy in the treatment of sinonasal adenoid cystic carcinoma / 临床耳鼻咽喉头颈外科杂志

Objective:To assess the prognosis of sinonasal adenoid cystic carcinoma with hard palatine invasion treated by transnasal endoscopic total maxillectomy. Methods:Clinical data of twenty-six patients with sinonasal adenoid cystic carcinoma invading hard palatine treated by transnasal endoscopic total maxillectomy between May 2014 and December 2020 was analyzed retrospectively. Survival rate, local recurrence and distant metastasis were analyzed using Kaplan-Meier method. Cox regression was used to investigate the prognosis factors. Masticatory function after maxillectomy has also been assessed using the questionnaire of patients' satisfaction about masticatory function. Results:Margins in 8 patients（30%） were positive. The median time of follow-up was 38 months（6 to 85 months）. Twenty-five patients recurred. Four patients died of distant metastasis. The 5-year overall survival rate and relapse-free survival rate was 79.5% and 89.1%, respectively. Independent predictors of outcome on multivariate analysis were positive margin（P=0.018）, recurrence（P=0.006） and distant metastasis（P=0.04）. Conclusion:Transnasal endoscopic total maxillectomy could be performed for the treatment of the sinonasal adenoid cystic carcinoma with hard palatine invasion. Positive margin, local recurrence and distant metastasis were important predictors for patients' prognosis.

Prevalence of middle ear effusion among children with adenoid hypertrophy at a national referral hospital in Tanzania

Introduction: Middle ear effusion (MEE) is a common childhood disorder that causes hearing impairment due to the presence of fluid in the middle ear which reduces the middle ear's ability to conduct sound. Temporary or persistent hearing loss as a result of MEE causes speech, language and learning delays in children. There are few studies on MEE in Tanzania despite the huge burden of hearing loss among children with adenoid hypertrophy which is a known risk factor for MEE. Method: A cross-sectional study was conducted among 420 children aged nine years and below having adenoid hypertrophy with or without MEE. The diagnosis of adenoid hypertrophy was confirmed with a lateral view x-ray of the nasopharynx and tympanometry for cases with MEE. The primary objective of the study was to assess the prevalence of MEE among children with adenoid hypertrophy. Results: The prevalence of MEE among children with adenoid hypertrophy was 61.7%, with 218 (51.9%) males and 202 (48.1%) females. The most affected age group was 2-4 years with an incidence 193 (46%) and in this age group, males (53.9%) were more affected than females (46.1%). Generally, males, 134 (51.7%) were more affected by MEE than females, 125 (48.3%) of all 259 children with MEE. In terms of age group predominance by MEE, children aged 3-4 years, 107(41.3%) were more affected than all other age groups. Additionally, 4 (1.5%) children with MEE presented with hearing loss. Conclusion: There is a high prevalence of MEE among children with adenoid hypertrophy but no significant association with hearing loss.

Carcinoma adenoide quístico / Adenoid cystic carcinoma

Introducción: El carcinoma adenoide quístico es una neoplasia maligna poco común y a menudo agresiva, que puede surgir en una variedad de órganos portadores de glándulas. Objetivo: Caracterizar la presentación de una neoplasia maligna agresiva infrecuente, el carcinoma adenoide quístico. Caso clínico: Paciente de 56 años que acudió a cuerpo de guardia con lesión en cuero cabelludo correspondiente con cilindroma maligno. Se estudia posteriormente y se constataron además lesiones a nivel de mamas, colon y recidiva en el cuero cabelludo, que dieron como resultado carcinoma adenoide quístico. Conclusiones: Es infrecuente; realizar su diagnóstico conlleva un alto índice de sospecha y su diagnóstico es histopatológico. Su pronóstico depende de la localización primaria y tiende a ser muy agresivo cuando se localiza fuera de la mama(AU)

Introduction: Adenoid cystic carcinoma is an uncommon and often aggressive malignant neoplasm that can arise in a variety of gland-bearing organs. Objective: To characterize the presentation of an uncommon aggressive malignancy, adenoid cystic carcinoma. Clinical case: 56-year-old patient who presented to the on-call department with a lesion on the scalp corresponding to malignant cylindroma. She was subsequently studied and lesions were also found in the breast, colon and recurrence in the scalp, which resulted in adenoid cystic carcinoma. Conclusions: It is infrequent; making its diagnosis involves a high index of suspicion and its diagnosis is histopathological. Its prognosis depends on the primary location and it tends to be very aggressive when located outside the breast(AU)

Clinico - pathological profile of Adenoid Cystic Carcinoma of the Lacrimal gland - A Retrospective Study at Tertiary Cancer Care Hospital, Punjab

Background: A retrospective study conducted in the department of radiation oncology to review the management aspects of the adenoid cystic carcinoma of the lacrimal gland and identify the outcome and corelation of the recurrence pattern with pathological and clinical findings. Methods: Data of 10 patients of adenoid cystic carcinoma of the lacrimal gland treated over a period of six years were reviewed with respect to presentation, treatment received , end points and outcome. Results: All of the 10 patients underwent surgery followed by adjuvant radiation therapy. Five patients received adjuvant chemotherapy . Eight (80%) patients were alive at a median follow up of 2 years. Five ( 50%) patients withou t evidence of disease and 3 patients with the disease. One patient developed scalp metastasis during the course of radiation therapy and another one developed lung metastasis. Conclusion: Ad enoid cystic carcinoma of the lacrimal gland is a rare neoplasm wi th aggressive outcome. It is often treated with surgery and adjuvant radiotherapy and needs long follow up.

Primary cutaneous adenoid cystic carcinoma – An onerous task for clinicians and pathologists

Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm usually confined to the salivary glands, lungs, and breasts. Primary cutaneous adenoid cystic carcinoma (PCACC) is an extremely rare entity with solitary cases reported at sites away from the scalp and chest. Hence, one must follow the multidisciplinary approach to exclude any primary ACC elsewhere in the body. We report a rare case of PCACC arising from the skin of the left lower limb in a 55-year-old woman with a history of recurrent swelling, clinically diagnosed as a metastatic tumor.

Case report on adenoid cystic carcinoma of palate with review of literature

Adenoid cystic carcinoma (ACC) is a malignant tumor of minor salivary glands of the oral cavity, particularly the palate which accounts for about 5–10%. These tumors are slow growing with a high incidence of local recurrence, perineural invasion, and distant metastasis. Here, we report the case of a 69-year-old female who presented with swelling over the hard palate. Computerized tomography shows enhancing lesion along the undersurface of the left side of the hard palate, abutting the maxillary alveolus in the region of the second premolar and first two molars. There was no erosion of the inner cortex of the maxillary alveolus and no extension into the nasal cavity. Following the biopsy, she underwent a left hard palatectomy with upper alveolar resection. The final histopathological report confirmed ACC with no perineural invasion. Following she received adjuvant radiotherapy using an intensity-modulated radiotherapy technique. Now at 6 months of follow-up, there was no evidence of recurrence. Thus, our patient requires long-term follow-up to assess the benefit and survival outcome.

Carcinoma adenoide cístico cutâneo primário no couro cabeludo com metástases pulmonares: um relato de caso / Primary cutaneous adenoid cystic carcinoma in the scalp with pulmonary metastases: a case report

O carcinoma adenoide cístico cutâneo primário (CACCP) é um câncer extremamente raro, com uma taxa estimada de ocorrência de 0,23 caso/1.000.000 de pessoas ao ano. Paciente do sexo masculino, 60 anos de idade, apresentou lesão no couro cabeludo em 2017. Inicialmente, não foi possível realizar o diagnóstico histopatológico. Em 2018, biópsia e imuno-histoquímica indicaram tratar-se de CACCP (tratamento realizado com cirurgia e radioterapia). Em 2020, ocorreu nova recidiva local, sendo realizada ressecção cirúrgica. Paciente apresentou recidiva sistêmica (metástases pulmonares). Foi realizado tratamento quimioterápico. Atualmente, as metástases pulmonares estão estáveis e o CACCP progrediu para paquimeninges.

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare tumor with an estimated incidence rate of 0.23 cases/1,000,000 people per year. A 60-year-old man presented a scalp lesion in 2017. Initially, it was not possible to perform a histopathologic diagnosis. In 2018, surgical biopsy and immunohistochemistry of the local skin recurrence indicated a diagnosis of PCACC (treatment was surgery and radiotherapy). In 2020, a new local tumor recurred and was surgically resected. The patient had systemic metastases (pulmonary metastases) and underwent chemotherapy. Currently, the pulmonary metastases are stable. The PCACC has progressed to pachymeninges

Avaliação da expressão da proteína EZH2 em carcinoma adenoide cístico e adenoma pleomórfico / EZH2 immunoexpression in adenoid cystic carcinoma and pleomorphic adenoma

As neoplasias de glândulas salivares apresentam comportamentos diferenciados, que não seguem os padrões clássicos das neoplasias benignas e malignas. A raridade de algumas destas lesões dificulta ainda mais o entendimento dos mecanismos envolvidos na etiopatogenia. Marcadores moleculares como a proteína EZH2 têm sido utilizados na investigação de alterações epigenéticas em diferentes neoplasias, auxiliando na definição do diagnóstico e prognóstico das lesões. O objetivo do presente trabalho é avaliar a expressão da proteína EZH2 e descrever as características clínicas e microscópicas de amostras de carcinoma adenoide cístico (CAC) e adenoma pleomórfico (AP) com ênfase na importância da definição da malignidade da lesão. A análise dos cortes microscópicos corados em Hematoxilina e Eosina dos casos de Adenoma pleomórfico mostraram células epiteliais e mioepiteliais glandulares dispostas em lençóis e estruturas ductiformes em meio a estroma variável. Os casos de Carcinoma adenoide cístico mostraram três padrões distintos de crescimento incluindo formações tubulares, cribriformes e sólidas. Todos os casos de AP e CAC foram positivos para reação imuno-histoquímica para EZH2. As amostras de CAC apresentaram expressão de EZH2 significativamente maior comparado ao AP. As covariáveis metástase em linfonodos, recorrência, padrão histológico, presença de áreas sólidas e invasão perineural foram descritas em relação à marcação de EZH2 em amostras de CAC. Dessa forma, os resultados do estudo melhoram o entendimento das características clínicas e histológicas do CAC, assim como sobre o comportamento das lesões. Além disso, a análise mostra que o EZH2 é um potencial marcador de malignidade e ressalta a importância da validação de marcadores moleculares de alterações epigenéticas.

Salivary gland neoplasms present different behaviors, which do not follow the classic patterns of benign and malignant neoplasms. The rarity of some of these lesions makes it even more difficult to understand the mechanisms involved in the etiopathogenesis. Molecular markers such as the EZH2 protein have been used to investigate epigenetic changes in different neoplasms, helping to define the diagnosis and prognosis of the lesions. The aim of the study was to evaluate the expression of the EZH2 protein and to describe the clinical and microscopic characteristics of adenoid cystic carcinoma (ACC) and pleomorphic adenoma (PA) which emphasizes the importance of defining the malignancy of the neoplasm. Histopathological analysis of PA cases showed myoepithelial and glandular epithelial cells arranged as duct-like structures and sheets intermingled in the variable stroma and ACC cases showed the three growth patterns, tubular, cribriform and solid forms. All ACC and PA cases were positive for EZH2, with diffuse nuclear staining in neoplastic cells. The ACC samples showed significantly higher EZH2 expression compared to the PA. The covariables nodal metastasis, recurrence, growth pattern, presence of solid areas and perineural invasion have been described in relation to EZH2 staining in ACC samples. The results of the study improve the understanding of the clinical and histological characteristics of ACC, as well as on the behavior of lesions. In addition, the analysis showed that EZH2 is a potential marker of malignancy and highlights the importance of validating molecular markers of epigenetic alterations.

Treatment and prognosis analysis of perineural invasion on sinonasal adenoid cystic carcinoma / 中华肿瘤杂志

Objective: To analyze the efficacy of sinonasal adenoid cystic carcinoma (ACC) with perineural invasion (PNI), and explore the prognostic value of PNI on sinonasal adenoid cystic carcinoma. Methods: The clinical data of 105 patients with sinonasal ACC admitted to Cancer Hospital, Chinese Academy of Medical Sciences from January 2000 to December 2016 were retrospectively reviewed. All patients were restaged according to American Joint Committee on Cancer 8th edition. Follow-up visits were conducted to obtain information of treatment failure and survival outcome. The Log rank test was used for univariate analysis of prognostic factors, and Cox regression model was used for multivariate prognostic analysis. Results: The maxillary sinus (n=59) was the most common primary site, followed by the nasal cavity (n=38). There were 93 patients with stage Ⅲ-Ⅳ. The treatment modalities included surgery alone (n=14), radiotherapy alone (n=13), preoperative radiotherapy plus surgery (n=10), and surgery plus postoperative radiotherapy (n=68). The median follow-up time was 91.8 months, the 5-year local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) rates were 72.6%, 73.0%, 52.9% and 78.0%, respectively. There were 33 patients (31.4%) with PNI-positive. The 5-year DMFS, PFS, and OS rates of PNI-positive group were 53.7%, 29.4% and 56.5%, respectively, which were significantly inferior to those of PNI-negative group (80.8%, 63.0% and 86.8%, respectively, P<0.05), while there was no significant difference in the 5-year LC rate between both groups (64.5% vs 76.5%, P=0.273). The multivariate Cox regression analysis showed PNI was one of the poor prognostic factors of DMFS (HR=3.514, 95%CI: 1.557-7.932), PFS (HR=2.562, 95%CI: 1.349-4.866) and OS (HR=2.605, 95%CI: 1.169-5.806). Among patients with PNI-positive, the 5-year LC, PFS and OS rates of patients received surgery combined with radiotherapy were 84.9%, 41.3% and 72.7%, respectively, which were significantly higher than 23.3%, 10.0% and 26.7% of patients receiving surgery or radiotherapy alone (P<0.05). Conclusion: The presence of PNI increases the risk of distant metastasis in patients with sinonasal ACC. Compared with patients with PNI-negative, the prognosis of patients with PNI-positive is relatively poor, and surgery combined with radiotherapy for PNI-positive sinonasal ACC results in good clinical outcomes.

Diagnostic methods and criteria of obstructive sleep apnea hypopnea syndrome in children / 口腔疾病防治

@#Obstructive sleep apnea hypopnea syndrome (OSAHS), which is a common childhood disease, is a trending topic in clinical multidisciplinary research due to its detriment to the growth and development of children. Due to the wide variety and specificity of pathogenesis and clinical manifestations, the clinical diagnosis of OSAHS is sophisticated and difficult and remains controversial in the field. This review summarizes the common diagnostic methods in OSAHS for children, including polysomnography,which is known as the current “gold standard”, pulse oximetry, fiberoptic nasopharyngoscopy, nasopharyngeal lateral X-ray, CT, and magnetic resonance imaging (MRI). Furthermore, it emphasizes the new diagnostic critical value from Chinese guidelines for the diagnosis and treatment of obstructive sleep apnea in children (2020) for children with OSAHS released by China in 2020: the obstructive apnea hypopnea index (OAHI) is ≥ 1 time/h; it also emphasizes the importance of history and physical examination to contribute to clinical diagnosis and treatment for children with OSAHS.

Clinicopathological features of adenoid cystic carcinoma of the breast / 肿瘤研究与临床

Objective:To investigate the clinicopathological characteristics, gene mutation and prognosis of adenoid cystic carcinoma of the breast (AdCC).Methods:A total of 12 cases of AdCC samples archived at Linyi Cancer Hospital from January 2008 to December 2021 were collected, including 8 cases of classic adenoid cystic carcinoma (C-AdCC) and 4 cases of solid-basaloid adenoid cystic carcinoma (SB-AdCC). Histomorphology, immunohistochemicstry and molecular genetics were performed to analyze the clinicopathological characteristics and the prognosis of AdCC.Results:C-AdCC patients grew in the shape of beam-tubuler and cribriform architecture, with characteristic structures of true and pseudoadenoid lumen; SB-AdCC patients grew in nests and solid, with moderate to severe atypia, necrosis and high mitotic count. Immunohistochemistry showed that CK7, CK5/6, p63 and S-100 were expressed in 8 cases of C-AdCC, and CD117 and CD10 were diffusely expressed in 4 cases of SB-AdCC. Fluorescence in situ hybridization (FISH) showed that 3 C-AdCC patients had MYB gene break. Next-generation sequencing (NGS) revealed NOTCH1 gene mutation was detected in 2 SB-AdCC patients. No lymph node metastasis was observed in 8 patients with C-AdCC, but 2 had postoperative lymph node metastasis in 4 patients with SB-AdCC.Conclusions:C-AdCC mainly grows in beam-tubular and sieve shape, with low-grade atypia and good prognosis. Some cases have MYB-NFIB fusion gene. SB-AdCC mainly shows nest-mass, solid growth, moderate to severe atypia, necrosis and mitosis (>5/10 high power field) as well as poor prognosis; some patients have NOTCH1 gene mutation.

Clinical Features and Prognostic Factors of Adenoid Cystic Carcinoma of Head and Neck / 肿瘤防治研究

Objective To retrospectively analyze the clinical and pathological data of 48 patients with adenoid cystic carcinoma of the head and neck and screen out related factors that affect the patient's prognosis. Methods The overall survival rate of patients is used as the main indicator for observing prognosis. We selected 11 clinical and pathological factors as observation indicators that may have an impact on the survival and prognosis of patients. The relation between observation factors and the overall survival rate of patients was analyzed by Cox multivariate regression. Results The median follow-up time was 33.5(1-98) months. The 1-, 2- and 5-years overall survival rates were 95%, 91% and 87%. Local recurrence occurred in 14(29.2%) patients. Distant metastasis occurred in 22(45.8%) patients. Cox regression analysis showed that recurrence, distant metastasis and primary site were correlated with the overall survival rate of patients with head and neck adenoid cystic carcinoma (P < 0.05). Conclusion Adenoid cystic carcinoma is a relatively rare tumor of the head and neck with a low degree of malignancy. However, recurrence, distant metastasis and primary site have relatively great impact on the prognosis of patients.