Previously Reported Ectopic Cervical Thymoma Cases

Supplementary material from DOI 10.1210/clinem/dgad635 Lists all ectopic cervical thymoma cases described in the literature, last updated August 2023

Analysis of Failure in Patients with Adenoid Cystic Carcinoma of the Head and Neck

Background: Adenoid cystic carcinoma (ACC) accounts for less than 1% of all head and neck malignancies. It is a locally aggressive tumor with a high prevalence of distant metastases. The current study aimed to identify independent predictors of outcome and to characterize the patterns of failure. Methods: This is an international retrospective clinicopathologic review of 495 ACC patients treated between 1985 and 2011 in 9 cancer centers worldwide. Results: Five-year overall survival (OS), disease-specific survival (DSS) and disease-free survival (DFS) were 76%, 80% and 68%, respectively. Independent predictors of OS and DSS were: age, site, N classification and presence of distant metastases. Adjuvant treatment had no significant influence on outcome (p = 0.6). Advanced N stage (p = 0.004) age ≥70 years (p = 0.01) and bone invasion (p = 0.001) were associated with shorter DFS on multivariate analysis. Metastasis sites were: lung (20%), bone (4%), liver (3%) and brain (1%). Median time to diagnosis of distant metastasis was 30 months (range 2–192). Age ≥70 years, tumor site, orbital invasion and N stage were independent significant predictors of distant metastasis. Metastasis site had a significant impact on OS (p = 0.04) and DSS (p = 0.03). Conclusion: The clinical course of ACC was slow but persistent. Paranasal sinus origin was associated with the lowest distant metastasis rate but with the poorest outcome. We found no impact of adjuvant radiation or chemoradiation on outcome. These prognostic estimates should be taken into consideration when tailoring treatment for patients with ACC

Adenoid Cystic Carcinoma of the Nasal Cavity and Paranasal Sinuses: A Meta-Analysis

Objectives To identify independent predictors of outcome in patients with adenoid cystic carcinoma (ACC) of the paranasal sinuses and skull base. Design Meta-analysis of the literature and data from the International ACC Study Group. Setting University-affiliated medical center. Participants The study group consisted of 520 patients, 99 of them from the international cohort. The median follow-up period was 60 months (range, 32 to 100 months). Main Outcome Measures Overall survival (OS) and disease-specific survival (DSS). Results The 5-year OS and DSS of the entire cohort were 62% and 67%, respectively. The local recurrence rate was 36.6%, and the regional recurrence rate was 7%. Distant metastasis, most commonly present in the lung, was recorded in 106 patients (29.1%). In the international cohort, positive margins and ACC of the sphenoid or ethmoidal sinuses were significant predictors of outcome (p < 0.001). Perineural invasion and adjuvant treatment (radiotherapy or chemoradiation) were not associated with prognosis. Conclusion Tumor margin status and tumor site are associated with prognosis in ACC of the paranasal sinuses, whereas perineural invasion is not. Adjuvant treatment apparently has no impact on outcome

Adenoid Cystic Carcinoma of the Nasal Cavity and Paranasal Sinuses: A Meta-Analysis

Objectives To identify independent predictors of outcome in patients with adenoid cystic carcinoma (ACC) of the paranasal sinuses and skull base. Design Meta-analysis of the literature and data from the International ACC Study Group. Setting University-affiliated medical center. Participants The study group consisted of 520 patients, 99 of them from the international cohort. The median follow-up period was 60 months (range, 32 to 100 months). Main Outcome Measures Overall survival (OS) and disease-specific survival (DSS). Results The 5-year OS and DSS of the entire cohort were 62% and 67%, respectively. The local recurrence rate was 36.6%, and the regional recurrence rate was 7%. Distant metastasis, most commonly present in the lung, was recorded in 106 patients (29.1%). In the international cohort, positive margins and ACC of the sphenoid or ethmoidal sinuses were significant predictors of outcome (p < 0.001). Perineural invasion and adjuvant treatment (radiotherapy or chemoradiation) were not associated with prognosis. Conclusion Tumor margin status and tumor site are associated with prognosis in ACC of the paranasal sinuses, whereas perineural invasion is not. Adjuvant treatment apparently has no impact on outcome

The Significance of Close Margins on Outcome of Patients with Adenoid Cystic Carcinoma of the Skull Base: An International Collaborative Study

Background: The mainstay of treatment in adenoid cystic carcinoma (ACC) of the head and neck is surgical resection with negative margins. Surgical resection of skull base ACC might be limited by proximity to vital structures. The clinical significance of close margins (not involved but less than 5 mm) in ACC remains undefined. This study aims to characterize the impact of close margin on the outcome of patients with ACC of the skull base. Methods: Univariate and multivariate models were used to evaluate the clinical and pathologic data in an international collaborative study. Results: A total of 507 patients with ACC of the head and neck were analyzed. Of those, 108 (22%) had ACC of the paranasal sinuses and skull base. Negative margins were achieved in 38 (35%) patients with paranasal sinuses ACC compared with 49% and 57% in patients with major salivary glands and oral cavity ACC, respectively (p = .001). Close margins, were found in 16 patients (15%), with skull base ACC. Univariate analysis revealed that margin status was significant for 5 years overall survival (OS, p = .009) disease specific survival (DSS, p = .003) and disease free survival (DFS, p = .015) for patients with ACC of the skull base. Multivariate analysis in patients with ACC of the skull base, revealed that close margin status was a significant predictors for 5 years OS (HR-2.11, 95% CI, 1.3–4.2, p = .01), DSS (HR-2.1, 95% CI, 1.2–3. 1, p = .03) and DFS (HR-3.1, 95%CI, 1.3–8.2, p = .03). Conclusions: In patients with skull base ACC, close margins should be considered as an adverse pathological feature similar to positive margins

Aqueous Humor Dynamics and Its Influence on Glaucoma

The chapter describes the anatomical and functional features of the aqueous humor (AH) dynamics with special focus on pathological changes in glaucoma. The main therapeutic approaches to medically and surgically regulate AH production and outflow are discussed