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.

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.

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

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.

Prognostic indicators in clinically node-negative malignant primary salivary tumours of the parotid: A multicentre experience.

OBJECTIVES: Nodal metastasis is an important prognosticator in primary parotid cancers. The management of the clinically node-negative neck is an area lacking consensus. This study investigates the occult nodal metastasis rate, and prognostic indicators in primary parotid cancers. MATERIALS AND METHODS: We performed a multicentre retrospective case note review of patients diagnosed and treated surgically with curative intent between 1997 and 2020. Demographic, clinic-pathological and follow-up data was recorded. RESULTS: After exclusions, 334 patients were included for analysis, with a median follow-up of 48 months. The overall rate of occult lymph node metastasis amongst patients undergoing elective neck dissection was 22.4%, with older age, high-grade and more advanced primary tumours being associated with higher rates. On multivariable analysis, age ≥ 60 years (HR = 2.69, p = 0.004), high-grade tumours (HR = 2.70, p = 0.005) and advanced primary tumours (pT3-4, HR = 2.06, p = 0.038) were associated with worse overall survival. Occult nodal metastasis on final pathology was associated with a close-to-significant reduction in regional recurrence free survival (HR = 3.18, p = 0.076). CONCLUSION: This large series confirms the significant occult lymph node metastasis rate in primary parotid cancer, and demonstrates the importance of primary histology, tumour grade and stage in predicting survival outcome. This data supports the use of elective neck dissection in patients with high-risk tumours.

Role of concurrent chemoradiation on locally advanced unresectable adenoid cystic carcinoma.

BACKGROUND/AIMS: Adenoid cystic carcinoma (ACC) is a rare salivary gland tumor characterized by indolence, with a high rate of local recurrence and distant metastasis. This study aimed to investigate the effect of concurrent chemoradiation (CCRT) on locally advanced unresectable ACC. METHODS: We retrospectively analyzed clinical data from 10 patients with pathologically confirmed ACC of the head and neck who received CCRT with cisplatin in Seoul National University Hospital between 2013 and 2018. RESULTS: Ten patients with unresectable disease at the time of diagnosis or with positive margins after surgical resection received CCRT with weekly cisplatin. Eight patients (80%) achieved complete remission, of which three later developed distant metastases without local relapse; one patient developed distant metastasis and local relapse. Two patient achieved partial remission without progression. Patients experienced several toxicities, including dry mouth, radiation dermatitis, nausea, and salivary gland inflammation of mostly grade 1 to 2. Only one patient showed grade 3 oral mucositis. Median relapse-free survival was 34.5 months (95% confidence interval, 22.8 months to not reached). CONCLUSION: CCRT with cisplatin is effective for local control of ACC with manageable toxicity and may be an effective treatment option for locally advanced unresectable ACC.

Analysis on the characteristics of multi-slice spiral CT images in 27 cases of lung cancer with cystic lesions / 中国基层医药

Objective:To investigate the characteristics of multi-slice spiral CT images in lung cancer with cystic lesions and improve the accuracy of diagnosis.Methods:The clinical data of 27 cases of cystic lung cancer confirmed by pathological results of surgery or puncture who received treatment during 2012-2019 in Tongde Hospital of Zhejiang Province, China were collected. The clinical stages, pathological results and the characteristics of multi-slice spiral CT images were analyzed.Results:All 27 cases were diagnosed with peripheral lung cancer. Type VI peripheral lung cancer occurred in 12 (44.44%) cases, and adenocarcinoma in 17 (62.96%) cases. Stage Ia lung cancer occurred in 18 (66.67%) patients. Above 70% of CT images showed irregular cystic structure [70.37% (19/27)], polycystic structure [74.07% (20/27)], and vessels passing through the cysts [70.37% (19/27). Over 90% of CT images displayed unsmooth inner wall of the cysts [92.59% (25/27)] and fuzzy margin of the cysts [96.30% (26/27)].Conclusion:It is very difficult to diagnose lung cancer with cystic lesions. This kind of atypical lung cancer should be considered when the imaging features of peripheral lung cancer appear including thickened cystic wall, fuzzy cystic margin, vascular bundle sign and pleural indentation sign.

[Comparison of the clinicopathological features between adenoid basal cell carcinoma and adenoid cystic carcinoma of the cervix].

Objective: To compare the clinical and histopathological characteristics of cervical adenoid basal cell carcinoma and adenoid cystic carcinoma for improving the diagnosis accuracy and differential diagnosis of these tumors. Methods: A retrospective study was conducted on 9 cases of cervical adenoid basal cell carcinoma and 3 cases of adenoid cystic carcinoma which were diagnosed and consulted at the First Medical Center of PLA General Hospital from March 2009 to April 2019. Detailed clinical data were reviewed. All pathological sections and immunohistochemical results were reviewed and the clinicopathological characteristics were analyzed. Follow-up information by telephone was collected and relevant literature was consulted. Results: Both tumors were more commonly found in postmenopausal women (the age of onset ranged 43-74 years). Adenoid basal cell carcinoma was often clinical asymptomatic. Most of them presented as abnormal smears of the cervix during physical examination, and there was no definite mass in colposcopy.Adenoid cystic carcinoma was mostly presented with abnormal vaginal bleeding. A mass was seen in colposcopy.Histologically, the two tumors were characterized by nest-like growth of the tumors, consisting of basal-like tumor cells, and often surrounded by palisade structures. The two lesions might coexist, or be mixed with squamous cell carcinoma or high-grade squamous intraepithelial lesions. The difference was that adenoid basal cell carcinoma was mostly located at the junction of cervical squamous epithelium and columnar epithelium and beneath the overlying epithelium, the tumor cells were arranged in nests, with squamous differentiation in the center of the nests, or in double-layer adenoid arrangement. The cell morphology was bland with occasional mitoses, and the stromal reaction was not obvious. And adenoid cystic carcinoma cells in the nest arranged like a sieve, the homogenous red-stained and blue-stained secretions were observed in the sieve holes, with obvious cell atypia, frequent mitoses, and obvious stromal reaction.In one case of adenoid cystic carcinoma, sarcomatoid area presented around the nests.Both of them were positive in clinical HPV test. Among the 9 cases of adenoid basal cell carcinoma, 3 were tested for HPV and 5 were tested for p16, and all showed positive expression. Among the 3 cases of adenoid cystic carcinoma, 2 were tested for HPV and 3 were tested for p16, both of which showed positive expression. Telephone follow-up was conducted by June 2019(follow-up time ranged 2-37 months). No recurrence or metastasis occurred in 7 of the 9 cases of adenoid basal cell carcinoma, while 1 case had a ground-glass nodule in lung and another had recurrence of vaginal stump 32 months after the surgery.One case of adenoid cystic carcinoma developed lung metastasis 8 months after surgery and died 2 years after surgery; another case was followed up for 6 months, which showed no recurrence or metastasis; the third case was lost to follow-up. Conclusions: Both adenoid cystic carcinoma and adenoid basal cell carcinoma of the cervix are the tumors originating from cervical reserve cells and are associated with high-risk HPV infection. Due to the differences in clinical treatment and prognosis, careful histological evaluation and immunohistochemical analysis should be carried out to make accurate pathological diagnosis.

[Covered airway stent loaded with (125)I seeds for tracheal adenoid cystic carcinoma: a clinical observation of 8 cases].

Objective: To explore the efficacy and safety of the covered airway stent loaded with (125)I seeds for the treatment of tracheal adenoid cystic carcinoma (TACC). Methods: We retrospectively reviewed the clinical data from 8 patients with TACC who had received placement of the covered stent loaded with (125)I seeds between December 2014 and July 2017 in the endoscopic center of the Second Affiliated Hospital of Xiamen Medical College. We compared the difference in the dyspnea index, the diameter of the airway lumen, and the lesion surrounding the airway wall before and after treatment. The complications were also recorded during follow-up. Results: Eight patients underwent successful placement of a total of 11 radioactive stents (2 straight-type stents, 2 L-shape stents, and 7 Y-shape stents, all loaded a total of 243 radioactive particles). Displacement of stents took place within 2 weeks in 2 patients, who were managed with re-stenting and fixation. No further displacement occurred during follow-up. The median time to stent removal was 2.9(interquartile range: 2.3,3.0) months. After stent placement, the dyspnea index was significantly decreased compared with pre-treatment level (mean: 0.1 vs. 3.4, t=8.881, P<0.001). Bronchoscopic re-assessment showed that the residual tumor within the airway was detected in only one patient and that the tumor completely disappeared in the remaining 7 patients. Treatment with stents loaded with radioactive particles yielded smooth and pale airway mucosa with formation of partial scar formation. Chest computed tomography re-assessment demonstrated significantly larger luminal diameter than that before treatment (mean: 13.1 mm vs. 3.3 mm, t=-7.839, P<0.001). The airway wall thickness was notably reduced after treatment (mean: 4.3 mm vs. 14.4 mm, t=7.620, P<0.001). The lesions surrounding the airway wall completely disappeared in 7 patients and decreased for more than 50% in a single patient. The median duration of follow-up was 28.0(interquartile range: 24.8,31.5) months. Recurrence of tumor was documented in a single case within 2 years. Six patients did not experience recurrence within the 2-year follow-up period. No death or severe complications were recorded during follow-up. Conclusion: The (125)I radioactive stent is effective for dilating the stenotic airway and ameliorating the symptoms, and thus might be an effective and safe method for the treatment of TACC. Further studies that explore the efficacy of stents loaded with (125)I particles are needed.

[Clinicopathological features of lung salivary gland-type tumors].

Objective: To study the clinicopathological characteristics of lung salivary gland-type tumors (SGT), and to compare with the corresponding primary SGT in salivary glands. Methods: Twenty-three cases of lung SGT were retrieved from the files of Peking University First Hospital from January 2004 to September 2018. The morphology, immunophenotype, genotype and outcome of these cases were analyzed. Results: The 23 patients included 13 males and 10 females, with age range of 13-79 years (median 54 years). There were 11 cases of adenoid cystic carcinoma, 10 cases of mucoepidermoid carcinoma (MEC), one case each of clear cell carcinoma and myoepithelioma. The morphology and immunophenotype of lung SGT were very similar to their counterparts in salivary gland. MYB rearrangement was detected in one of 11 adenoid cystic carcinomas. MAML2 rearrangement was detected in all the MECs. EWSR1 rearrangement was detected in the one case of clear cell carcinoma. Of patients with adenoid cystic carcinoma, the survival time was more than 60 months (three cases), 52 months (one case), and 12-36 months (three cases). There was no recurrence and death in seven cases of MEC with follow-up results. One case of clear cell carcinoma recurred after 52 months of follow-up. Conclusions: Although the SGT of lung and their counterparts in salivary gland are very similar in their morphology, immunophenotype, genotype and prognosis, there are also some differences between each other. MYB rearrangement can be detected in most adenoid cystic carcinomas of salivary gland, but rarely in lung adenoid cystic carcinoma. The prognosis of patients with lung MEC is better than that of patients with salivary gland MEC, while the prognosis of patients with lung adenoid cystic carcinoma is worse than that of patients with salivary gland adenoid cystic carcinoma.

[Clinical features and pathological analysis of lacrimal gland occupying lesions in 91 cases].

Objective: To analyze the pathogenesis, histopathological classification and clinical features of lacrimal gland occupying lesions. Methods: This was a retrospective case series study. Clinical data of 91 patients (102 eyes) with lacrimal gland area occupying diseases who received ophthalmic surgery in the Second People's Hospital of Yunnan Province from January 2014 to November 2018 were retrospectively analyzed, including patients' age, reasons for treatment, gender, imageological examination data and pathological diagnosis results. All patients had more than one medical imaging examination results and histopathological diagnosis results. Results: Among 91 cases, 46 patients (50.5%) were male and 45 (49.5%) were female. The age distribution ranged from 1.1 years to 72 years old, with an average age of 43 years. All of benign tumors added up to 58 cases (63.7%). Pleomorphic adenoma (43 cases, 47.3%), dermoid cyst (6 cases, 6.6%), and inflammatory pseudotumor (6 cases, 6.6%) were the most common cases in the benign lacrimal gland occupying tumors. There were 33 cases (36.3%) of malignant tumors. Adenoid cystic carcinoma (15 cases, 16.5%), adenocarcinoma (6 cases, 6.6%) and lymphoma (5 cases, 5.5%) had the highest incidence among the malignant lacrimal gland occupying tumors. The most common reason for seeking medical treatment was exophthalmos (50 cases, 54.9%; 30 cases were pleomorphic adenoma). Brow arch mass (22 cases, 24.2%) and pain in and around the eye (9 cases, 9.9%; 5 cases were adenoid cystic carcinoma) were also major reasons. Conclusions: The most common benign lacrimal gland area occupying lesion in surgery patients of Yunnan is pleomorphic adenoma, which more occurred in patients with exophthalmos as the main symptoms. The most common malignant tumor in the lacrimal gland area is adenoid cystic carcinoma and the most common reason to seek medical advice was pain in and around the eye. (Chin J Ophthalmol, 2019, 55:842-846).

[Salivary gland malignancies-highlights of the 2019 ASCO Annual Meeting]. / Maligne Speicheldrüsentumoren ­ Highlights der ASCO-Jahrestagung 2019.

BACKGROUND: Due to their comparatively low incidence, salivary gland carcinomas have only been the subject of isolated clinical studies in recent years. In addition, surgery with/without adjuvant radiotherapy is considered standard treatment. Systemic therapies have received little attention and are only used for advanced and distantly metastasized salivary gland malignancies. OBJECTIVE: The contributions with the highest relevance for this year's meeting of the American Society of Clinical Oncology (ASCO) were to be reviewed. MATERIALS AND METHODS: A total of 12 contributions pertaining to clinical studies on salivary gland malignancies were identified, eight of which were classified as relevant for future changes to the therapeutic landscape. RESULTS: Three studies dealt with different combinations of a checkpoint blockade, and each showed a low response rate. In addition, studies on targeted therapies depending on the results of a mutation analysis and expression of HER2 or the androgen receptor were presented. CONCLUSION: A favorable response of HER2-positive salivary gland carcinomas to an antibody-drug conjugate could be shown. Furthermore, no convincing data regarding response to programmed cell death protein 1 (PD1)/programmed death ligand 1 (PD-L1) inhibitors in advanced salivary gland cancer were presented. Further studies and ideas for new treatment approaches will be needed to improve the therapeutic options for patients with salivary gland carcinoma.

Case of Unrecognised of Maxillary Adenoid Cystic Carcinoma.

Adenoid cystic carcinoma is a less commonly diagnosed cancer that may affect the major or minor salivary glands. We present a 70 year old male patient who was admitted to the Department of Oral Medicine, School of Dental Medicine in Zagreb, Croatia due to pain in the right maxilla. In this case we report a case of the patient with unilateral pain in the maxilla & eye which lead to the diagnosis of adenoid cystic cancer without any visible oral lesions.

[Clinicopathological features and prognostic factors of primary pulmonary adenoid cystic carcinoma: a study of 59 cases].

Objective: To investigate the clinicopathological features and prognostic indicators of primary pulmonary adenoid cystic carcinoma. Methods: Fifty-nine cases of primary pulmonary adenoid cystic carcinoma were collected from August 2011 to December 2017 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin-eosin staining and immunohistochemistry. The clinicopathological features were reviewed and patient survival analysis was performed using Kaplan-Meier method and Cox regression model. Status of epidermal growth factor receptor (EGFR), KRAS, BRAF genes was analyzed in 15 of the 59 study cases. Results: Among 59 cases, there were 25 males and 34 females with male to female ratio of 1.0 to 1.4. The patient age ranged from 29 to 81 years with a mean age of 55 years. The tumor max diameters ranged from 1.0 to 9.6 cm with an average diameter of 2.8 cm. Fifteen (25.4%) patients were smokers while 44 patients (74.6%) were non-smokers. Tumors predominantly occurred in the trachea (28/59,47.5%), the left main bronchus (7/59,11.9%) and the right bronchus (5/59,8.5%). Grossly, the tumors were well circumscribed, greyish-white nodules. Microscopically the tumor cells were small and uniform, and arranged in tubular, cribriform, and solid patterns. Immunohistochemistry showed that the tumor cells were positive for CK7, S-100 protein, Sox-10, CD117 and p63. TTF1 was only positive in 2 cases and Ki-67 index ranged from 3% to 40%. Eighteen cases (30.5%) were gradeⅠ, 26 cases (40.1%) grade Ⅱ, and 15 cases (25.4%) grade Ⅲ. Overall, 39 cases (66.1%), 7 cases (11.9%), 10 cases (16.9%), and 3 cases (5.1%) were at stages Ⅰ, Ⅱ, Ⅲ, and Ⅳ, respectively. Twenty-three patients (39.0%) received surgical therapy, 3 patients (5.1%) surgery combined with radiotherapy, 9 patients (15.2%) surgery combined with chemotherapy, and 24 cases (40.7%) chemotherapy only. No mutation of EGFR, KRAS and BRAF was detected in all 15 tested cases. The overall survival rate at the first, third and fifth years was 94.9%, 86.4% and 84.7%, respectively. Prognostic analysis showed that patient's age and tumor size were statistically associated with the survival (P<0.05). Conclusions: Majority of the patients with primary pulmonary adenoid cystic carcinoma are at an early clinical stage with a favorable prognosis. The size of the tumor and the age of the patients are independent prognostic indicators.

ADENOID CYSTIC CARCINOMA OF DISTAL TRACHEA: A CASE REPORT.

Primary malignant tumors of the trachea are very rare with the incidence of less than two per million people per year, and only ten percent of them are adenoid cystic carcinomas. Eighty percent of all tracheal tumors are malignant. Diagnosis is usually late because the symptoms mimic other conditions such as asthma. Clinical picture may sometimes be dramatic when airway is almost closed and emergency recanalization is necessary. Diagnosis is made by chest computed tomography scan or magnetic resonance imaging. Definitive treatment is surgical resection alone or followed by radiation therapy or radiation therapy alone. Radical resection is only accomplished in about half of all cases because of the submucosal tumor growth and limited length of tracheal resection. The role of adjuvant radiation therapy in negative resection margin cases is not clear but all patients with positive resection margin benefit from radiation therapy. We present a case of a 43-year-old patient with primary adenoid cystic carcinoma of distal trachea treated by emergency bronchoscopic recanalization and resection of the tracheal tumor with end-to-end anastomosis.

Clinicopathological features of lung salivary gland-type tumors / 中华病理学杂志

Objective@#To study the clinicopathological characteristics of lung salivary gland-type tumors (SGT), and to compare with the corresponding primary SGT in salivary glands.@*Methods@#Twenty-three cases of lung SGT were retrieved from the files of Peking University First Hospital from January 2004 to September 2018. The morphology, immunophenotype, genotype and outcome of these cases were analyzed.@*Results@#The 23 patients included 13 males and 10 females, with age range of 13-79 years (median 54 years). There were 11 cases of adenoid cystic carcinoma, 10 cases of mucoepidermoid carcinoma (MEC), one case each of clear cell carcinoma and myoepithelioma. The morphology and immunophenotype of lung SGT were very similar to their counterparts in salivary gland. MYB rearrangement was detected in one of 11 adenoid cystic carcinomas. MAML2 rearrangement was detected in all the MECs. EWSR1 rearrangement was detected in the one case of clear cell carcinoma. Of patients with adenoid cystic carcinoma, the survival time was more than 60 months (three cases), 52 months (one case), and 12-36 months (three cases). There was no recurrence and death in seven cases of MEC with follow-up results. One case of clear cell carcinoma recurred after 52 months of follow-up.@*Conclusions@#Although the SGT of lung and their counterparts in salivary gland are very similar in their morphology, immunophenotype, genotype and prognosis, there are also some differences between each other. MYB rearrangement can be detected in most adenoid cystic carcinomas of salivary gland, but rarely in lung adenoid cystic carcinoma. The prognosis of patients with lung MEC is better than that of patients with salivary gland MEC, while the prognosis of patients with lung adenoid cystic carcinoma is worse than that of patients with salivary gland adenoid cystic carcinoma.

Clinicopathological features and prognostic factors of primary pulmonary adenoid cystic carcinoma: a study of 59 cases / 中华病理学杂志

Objective@#To investigate the clinicopathological features and prognostic indicators of primary pulmonary adenoid cystic carcinoma.@*Methods@#Fifty-nine cases of primary pulmonary adenoid cystic carcinoma were collected from August 2011 to December 2017 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin-eosin staining and immunohistochemistry. The clinicopathological features were reviewed and patient survival analysis was performed using Kaplan-Meier method and Cox regression model. Status of epidermal growth factor receptor (EGFR), KRAS, BRAF genes was analyzed in 15 of the 59 study cases.@*Results@#Among 59 cases, there were 25 males and 34 females with male to female ratio of 1.0 to 1.4. The patient age ranged from 29 to 81 years with a mean age of 55 years. The tumor max diameters ranged from 1.0 to 9.6 cm with an average diameter of 2.8 cm. Fifteen (25.4%) patients were smokers while 44 patients (74.6%) were non-smokers. Tumors predominantly occurred in the trachea (28/59,47.5%), the left main bronchus (7/59,11.9%) and the right bronchus (5/59,8.5%). Grossly, the tumors were well circumscribed, greyish-white nodules. Microscopically the tumor cells were small and uniform, and arranged in tubular, cribriform, and solid patterns. Immunohistochemistry showed that the tumor cells were positive for CK7, S-100 protein, Sox-10, CD117 and p63. TTF1 was only positive in 2 cases and Ki-67 index ranged from 3% to 40%. Eighteen cases (30.5%) were gradeⅠ, 26 cases (40.1%) grade Ⅱ, and 15 cases (25.4%) grade Ⅲ. Overall, 39 cases (66.1%), 7 cases (11.9%), 10 cases (16.9%), and 3 cases (5.1%) were at stages Ⅰ, Ⅱ, Ⅲ, and Ⅳ, respectively. Twenty-three patients (39.0%) received surgical therapy, 3 patients (5.1%) surgery combined with radiotherapy, 9 patients (15.2%) surgery combined with chemotherapy, and 24 cases (40.7%) chemotherapy only. No mutation of EGFR, KRAS and BRAF was detected in all 15 tested cases. The overall survival rate at the first, third and fifth years was 94.9%, 86.4% and 84.7%, respectively. Prognostic analysis showed that patient′s age and tumor size were statistically associated with the survival (P<0.05).@*Conclusions@#Majority of the patients with primary pulmonary adenoid cystic carcinoma are at an early clinical stage with a favorable prognosis. The size of the tumor and the age of the patients are independent prognostic indicators.

[The clinicopathological features and curative effect of the bronchoscopic interventional therapy combined with radiotherapy for the treatment of locally recurrent lung adenoid cystic carcinoma].

Objective: To explore the clinical value of endoscopic interventional therapy for locally recurrent primary lung adenoid cystic carcinoma (ACC). Methods: The clinical data of 42 patients with locally recurrent ACC were retrospectively analyzed, and the differences of tracheal and bronchial diameter, airway scoring grade and airway obstruction degree before and after treatment were compared among three treatment methods: bronchoscopic interventional therapy + palliative radiotherapy, interventional therapy alone, and non-interventional therapy. Log rank test and Cox proportional risk model multi-factor analysis were used to determine the prognostic factors of ACC patients with local recurrence, and the long-term effect of bronchoscopic interventional therapy on ACC with local recurrence was determined. Results: The median overall survival of 42 patients was 59 months and 5-year survival rate was 54.2%.Univariate analysis showed that vascularized cancer, pleural invasion, pulmonary atelectasis, incisal margin, microscopic classification, tumor diameter, initial TNM stage, ki-67 index, and treatment after local recurrence were associated with long-term survival of ACC patients with local recurrence (all P<0.05). Cox multivariate regression analysis showed that margin status (RR=0.272, P=0.011), tumor diameter (RR=2.586, P=0.005), initial TNM staging (RR=0.369, P=0.035), ki-67 index (RR=3.569, P<0.001), and treatment methods after local recurrence (RR=0.126, P<0.001) were independent factors influencing the prognosis of ACC patients with local recurrence. After three months of treatment, the tracheal bronchus diameters, rating of shortness of breath, and degree of airway obstruction were all improved significantly (all P<0.05), both in the interventional therapy + palliative radiotherapy group [(14.5±2.8 mm, 0.86±0.45, (14.50±10.67)%, respectively], and the interventional therapy alone group [(13.7±2.3) mm, 0.97±0.25, (15.38±12.02)%, respectively]. Meanwhile, the difference before and after non-interventional therapy was not statistically significant (all P>0.05). 5-year overall survival rates were 55.8%, 46.6% and 42.6% for patients undergoing interventional therapy+ palliative radiotherapy, interventional therapy alone, and non-interventional therapy after recurrence, with statistically significant differences (P=0.015). Patients underwent bronchial endoscopic interventional therapy and palliative radiotherapy had the best efficacy of treatment. Conclusion: Endoscopic interventional therapy plus palliative radiotherapy is an effective local palliative treatment for locally recurrent ACC patients, which can rapidly relieve airway stenosis, improve the quality of life of patients and prolong the survival time of patients.

[Clinicopathologic features and prognosis of adenoid cystic carcinoma of external auditory meatus].

Objective: To investigate clinicopathologic features and prognosis of adenoid cystic carcinoma (ACC) involving external auditory meatus. Methods: The clinical presentation and follow-up data of 63 patients with ACC of external auditory canal were collected from January 2006 to February 2017 at PLA General Hospital and Hainan Branch of PLA General Hospital. The clinicopathologic features and prognostic factors of external auditory canal ACC were analyzed. Results: (1) There were 28 males and 35 females and the average age of the first diagnosis was 48.9 years (22-81 years). The tumors showed cribriform pattern in 35 cases (15 cases of late stage), tubular pattern in 14 cases (8 cases of late stage), and solid pattern in 14 cases (9 cases of late stage). Cases with solid pattern was relatively more frequent than that of cribriform pattern and tubular pattern, but the difference was not statistically significant (P>0.05). (2) The average follow-up time was 62.4 months (2-228 months) in the 57 available cases. Among the 33 cases with recurrence, 18 cases had local recurrence and 15 cases had distant metastasis. The mean recurrence time was 40.6 months (2-204 months). Nine patients died of ACC: 2 cases in early stage (died at 48 and 102 months after the first treatment), 7 cases in late stage and 57 with (died at 9, 30, 32, 60, 72, 94 and 228 months). (3) Among the 37 patients with perineural invasion, there were 21 cases of cribriform pattern, 4 cases of tubular pattern and 12 cases of solid pattern; the number of cases in early stage and late stage were 15 and 22, respectively; and the differences were statistically significant (P<0.05). In addition, 31 cases had otalgia among the 37 patients with perineural invasion, where differences were not significant (P>0.05). (4) Thirty of 45 cases with tumor resection or partial resection of temporal bone had recurrence, whereas 3 of 12 cases of tumor combined with superficial lobectomy of parotid gland had recurrence. The difference was statistically significant (P<0.05). Postoperative adjuvant radiotherapy was given in 19 cases, including 7 cases of early stage (2 cases of recurrence), and 12 cases of late stage (8 cases of recurrence), among which there was no significant difference (P>0.05). Conclusions: ACC occurring in external auditory canal frequently recurs. Superficial parotid lobectomy at the first operation is necessary to prevent tumor recurrence. Postoperative adjuvant radiotherapy has certain curative effect on patients with early stage tumor, but it does not affect the recurrence rate. Patients at late stage are more prone to perineural invasion than those in early stage. In addition, cribriform and solid patterns are more common that tubular pattern, and there is no significant correlation between perineural invasion and otalgia.