Objective: To present a rare case of Adenoid Cystic Carcinoma of the external ear canal in a 73-year-old man, including its diagnosis and surgical management. : Methods Design: Case Report Setting: Tertiary Government Training Hospital Patient: One Results: A 73-year-old man who consulted due to decreased hearing on the right ear had a 4-year history of gradually enlarging mass occupying the right external auditory canal. Wide en bloc resection of the external auditory canal mass and superficial parotidectomy with facial nerve preservation was performed with final histopathology results of adenoid cystic carcinoma. Follow-up at 2 years has shown no signs or symptoms or recurrence. Conclusion Adenoid cystic carcinoma of the external ear canal is a very rare disease that can present similarly to other otologic infections. Early identification of symptoms, performance of necessary imaging, and timely biopsy are key to reducing misdiagnosis and improving the survival rate. Complete resection of the tumor with adequate margins is the recommended treatment due to its high rates for recurrence.
Carcinoma, Adenoid Cystic ; Ear Neoplasms

Adenoid cystic carcinoma usually occurs in the salivary glands of the head and neck. It is a malignant tumor with a high degree of malignancy, resistance to radiotherapy and chemotherapy and poor prognosis. The clinical course of adenoid cystic carcinoma is slow and easy to be misdiagnosed. The main diagnosis and treatment means are individualized and precise treatment under the multi-disciplinary consultation mode, that is, surgical treatment and radiotherapy and chemotherapy. Adenoid cystic carcinoma is prone to relapse and hematologic metastasis, and the traditional radiotherapy and chemotherapy based therapies have not achieved satisfactory efficacy in the past three decades. How to detect, diagnose and treat early is an urgent task faced by clinicians.
Humans ; Carcinoma, Adenoid Cystic/pathology* ; Neoplasm Recurrence, Local ; Neck/pathology* ; Oropharynx/pathology* ; Diagnostic Errors

Objective: To evaluate the efficacy of endoscopic transnasal surgery for sinonasal and skull base adenoid cystic carcinoma (ACC), and to analyze the prognostic factors. Methods: Data of 82 patients (43 females and 39 males, at a median age of 49 years old) with sinonasal and skull base ACC who were admitted to XuanWu Hospital, Capital Medical University between June 2007 and June 2021 were analyzed retrospectively. The patients were staged according to American Joint Committee on Cancer (AJCC) 8th edition. The disease overall survival(OS) and disease-free survival(DFS) rates were calculated by Kaplan-Meier analysis. Cox regression model was used for multivariate prognostic analysis. Results: There were 4 patients with stage Ⅱ, 14 patients with stage Ⅲ, and 64 patients with stage Ⅳ. The treatment strategies included purely endoscopic surgery (n=42), endoscopic surgery plus radiotherapy (n=32) and endoscopic surgery plus radiochemotherapy (n=8). Followed up for 8 to 177 months, the 5-year OS and DFS rates was 63.0% and 51.6%, respectively. The 10-year OS and DFS rates was 51.2% and 31.8%, respectively. The multivariate Cox regression analysis showed that late T stage and internal carotid artery (ICA) involvement were the independent prognostic factors for survival in sinonasal and skull base ACC (all P<0.05). The OS of patients who received surgery or surgery plus radiotherapy was significantly higher than that of patients who received surgery plus radiochemotherapy (all P<0.05). Conclusions: Endoscopic transonasal surgery or combing with radiotherapy is an effective procedure for the treatment of sinonasal and skull base ACC. Late T stage and ICA involvement indicate poor prognosis.
Male ; Female ; Humans ; Middle Aged ; Carcinoma, Adenoid Cystic/surgery* ; Retrospective Studies ; Skull Base/pathology* ; Disease-Free Survival ; Prognosis

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.
Humans ; Carcinoma, Adenoid Cystic/pathology* ; Paranasal Sinus Neoplasms/pathology* ; Retrospective Studies ; Neoplasm Recurrence, Local/pathology* ; Prognosis

Objective: To investigate the expression of TRPS1 in salivary gland-type breast carcinoma and its clinical application. Methods: A total of 30 cases of salivary gland-type breast carcinoma diagnosed from May 2015 to November 2022 at the Department of Pathology of the First Affiliated Hospital of Nanjing Medical University were collected. The expression of TRPS1 was detected by immunohistochemistry and compared with that of GATA3. TRPS1 and GATA3 expression in 24 cases of primary salivary gland carcinoma. Results: There were 10 cases of breast secretory carcinoma, aged 21-61 years (median 53.5 years), with the size ranging from 0.9-2.2 cm (median 1.6 cm), 2 of which were accompanied by axillary nodal macrometastasis. All patients were alive after 2-55 months of follow-up (median 29.5 months, mean 29.7 months). There were 20 cases of breast adenoid cystic carcinoma, aged 36-77 years (median 53.5 years), with the size ranging from 1.2-5.5 cm (median 2.5 cm), 3 of which were accompanied by axillary nodal macrometastasis. All patients were alive after 3-92 months of follow-up (median 22.5 months, mean 31.7 months), and 1 patient had lung metastasis 15 months after surgery. The medium/high expression ratio of TRPS1 in breast secretory carcinoma was 10/10, which was higher than that of GATA3 (7/10). TRPS1 was also positive in the 2 cases with lymph node metastases. The medium/high expression rate of TRPS1 in breast adenoid cystic carcinoma was 20/20, which was significantly higher than that of GATA3 (2/20). TRPS1 was highly expressed in both classic and solid subtypes, while GATA3 was only expressed in a few cases of the classic subtype. TRPS1 was also positive in 3 cases with lymph node metastases and 1 case of the pulmonary metastases. The expression level of TRPS1 was the same in 1 case before and after neoadjuvant chemotherapy. In addition, TRPS1 was positive in parotid secretory carcinoma and adenoid cystic carcinoma. The medium/high expression rate of TRPS1 in parotid secretory carcinoma (6/6) was higher than that of GATA3 (2/6), and the medium/high expression rate of TRPS1 in parotid adenoid cystic carcinoma (17/18) was higher than that of GATA3 (2/18). Conclusions: The expression of TRPS1 is highly sensitive to salivary gland-type breast carcinoma, especially in GATA3-negative solid subtype of adenoid cystic carcinoma, which plays an important role in clinical practice.
Humans ; Female ; Carcinoma, Adenoid Cystic/pathology* ; Lymphatic Metastasis ; Salivary Gland Neoplasms/pathology* ; Parotid Neoplasms ; Lung Neoplasms ; Breast Neoplasms ; Parotid Gland ; Repressor Proteins

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.
Carcinoma, Adenoid Cystic/pathology* ; Humans ; Paranasal Sinus Neoplasms/therapy* ; Prognosis ; Proportional Hazards Models ; Retrospective Studies

Carcinoma, Adenoid Cystic ; Humans ; Neoplastic Syndromes, Hereditary ; Skin Neoplasms

Carcinoma, Adenoid Cystic/pathology* ; Cervix Uteri/pathology* ; Female ; Humans ; Uterine Cervical Neoplasms/pathology*

OBJECTIVE: To investigate the clinicopathologic features and prognostic factors in oral basaloid squamous cell carcinoma. METHODS: Retrospective analysis of oral basaloid squamous cell carcinomas patients who underwent tumor resection during the period from January 2002 to December 2020 in the authors' hospital, especially the clinicopathologic characteristics of 28 cases with confirmed diagnosis and follow-up data. Immunohistochemistry was performed to define the helpful markers for differentiation diagnosis. The factors influencing the prognosis were evaluated based on Kaplan-Meier method. RESULTS: The tongue and mouth floor (11 cases, 39.3%) were the most frequently involved sites, followed by gingiva (6 cases, 21.4%), buccal (5 cases, 17.9%), palate (4 cases, 14.3%), and oropharynx (2 cases, 7.1%). The majority of basaloid squamous cell carcinomas were in advanced stage, with 12 cases in stage Ⅱ and 16 cases in stages Ⅲ-Ⅳ. Twelve of 28 patients were identified to have cervical lymph node metastasis, which was confirmed by histopathological examination. The incidence rate of lymph node metastasis was 42.9% (12/28). Nine tumors recurred, with one metastasized to the lung. At the meantime, the 28 conventional squamous cell carcinomas were matched with the same stage, among which 13 cases were identified with cervical lymph node metastasis. The incidence rate of lymph node metastasis was 46.4% (13/28). Five cases recurred, with two cases that metastasized to the lung and one to the brain. The 5-year overall survival rates of the basaloid squamous cell carcinoma and conventional squamous cell carcinoma patients were 54.6% and 53.8%, respectively. Histopathologically, basaloid cells consisted of tumor islands without evident keratinization but frequently with comedo-like necrosis within the tumor islands. CK5/6 and P63 exhibited strongly positive in all the 28 cases, whereas neuroendocrine markers, CgA and Syn, were negative. Eight cases positively expressed P16; one case showed focal SOX10 positive but CK7 negative. CONCLUSION The majority of oral basaloid squamous cell carcinomas present in advanced stage with a high tendency to lymph node metastasis, but the overall survival rates are not significantly different from conventional squamous cell carcinomas matched with the same stage. The human papilloma virus (HPV), as HPV-positivity rate is high, correlates to good prognosis. In addition, CK7 & SOX10 immunohistochemistry could contribute to differential diagnosis for basaloid squamous cell carcinoma with solid adenoid cystic carcinoma.
Carcinoma, Adenoid Cystic ; Carcinoma, Squamous Cell ; Humans ; Immunohistochemistry ; Neoplasm Recurrence, Local/epidemiology* ; Retrospective Studies

Objective: To analyze the prognosis and risk factors of lung metastasis of patients with adenoid cystic carcinoma(ACC) of head and neck. Methods: A retrospective study was conducted. The data of 157 patients with ACC of head and neck treated in Beijing Tongren Hospital, Capital Medical University from January 2014 to October 2020 were collected, including 72 males and 85 females, with onset age between 14 and 72 years old. According to whether lung metastasis occurred, the patients were divided into lung metastasis group (88 cases) and non-pulmonary metastasis group (69 cases). Kaplan-Meier method was used to calculate the overall survival rate and progression-free survival rate using SPSS 26.0 software. Log-rank test was used to evaluate statistically relevant clinicopathological factors. Cox proportional risk model was used in multivariate analysis for the factors affecting the lung metastasis-free survival using R Studio 1.2.5042. Results: The 3-year and 5-year overall survival rates were 91.5% and 85.2%, respectively. The 3-year and 5-year progression-free survival rates were 57.7% and 34.3%, respectively. Univariate analysis showed that primary site, histological grade, high-grade transformation, Ki-67, T stage, and lymph node status were the risk factors for lung metastasis (χ²=11.78, 10.41, 4.06, 4.71, 5.37, 16.20, respectively, all P<0.05). Multivariate analysis showed independent risk factors for lung metastasis, including submandibular gland and sublingual gland (HR=3.53, 95%CI: 1.19-10.46, P<0.05), T3-4 stage (HR=3.09, 95%CI: 1.54-6.23, P<0.05), and Grade Ⅱ-Ⅲ grade (HR=2.47, 95%CI: 1.26-4.86,P<0.05). Conclusion: Distant metastasis, mainly pulmonary metastasis, affects the long-term prognosis of patients with ACC significantly. Primary site, T stage and histopathological grade can be used as the predictors for the risk of lung metastasis.
Adolescent ; Adult ; Aged ; Carcinoma, Adenoid Cystic ; Female ; Humans ; Lung/pathology* ; Lung Neoplasms/secondary* ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Risk Factors ; Young Adult