Ewing Sarcoma of the Head and Neck.

OBJECTIVES: Ewing sarcoma is a rare tumor of the head and neck. Previous efforts to characterize Ewing sarcoma of the head and neck (ES-HN) have been limited to small retrospective series. The objective of this study was to analyze the demographic, clinicopathologic, treatment, and survival characteristics of ES-HN compared to Ewing sarcoma at other locations (ES-other). METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) database, we compared 183 patients with ES-HN to 3177 patients with ES-other. Patient characteristics were analyzed with chi-square or t test. Ten-year disease-specific survival (DSS) and overall survival (OS) were estimated via the Kaplan-Meier method and compared using the log-rank test. Multivariate Cox regression analysis was used to determine if HN location was an independent prognosticator. RESULTS: The ES-HN displayed a lower tumor size ( P < .001) and metastatic rate ( P < .001) compared to ES-other. The ES-HN had a better 10-year DSS and OS than ES-other ( P = .001, P = .015, respectively). The HN location did not achieve statistical significance on multivariate Cox regression analysis ( P = .88). CONCLUSION: ES-HN does not appear to be a separate clinical entity compared to ES-other; rather, its associated improved prognosis is likely secondary to its smaller size and lower metastatic rate compared to ES-other.

Head and neck chondrosarcomas: Analysis of the Surveillance, Epidemiology, and End Results database.

BACKGROUND: Chondrosarcoma is a rare tumor of the head and neck. Because of the limited clinical data, no studies have conducted a thorough comparison between head and neck chondrosarcomas and those arising at other body sites. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) database, we compared 682 patients with chondrosarcomas of the head and neck with 4757 patients with non-head and neck chondrosarcomas, evaluating disease-specific survival (DSS) and overall survival (OS) based on tumor location, histology, grade, stage, and treatment. RESULTS: Head and neck chondrosarcomas displayed a lower grade (p < .001) and stage (p < .0001) compared with other chondrosarcomas. Overall, chondrosarcomas of the head and neck had significantly higher DSS and OS than non-head and neck chondrosarcomas (p < .001). CONCLUSION: Chondrosarcomas of the head and neck display a lower grade and stage compared with other chondrosarcomas. DSS and OS were significantly greater for chondrosarcomas of the head and neck. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1359-1366, 2016.

Factors influencing survival in acinic cell carcinoma: a retrospective survival analysis of 2061 patients.

BACKGROUND: Acinic cell carcinoma is an uncommon salivary neoplasm with clinical and histologic features known to influence prognosis. The purpose of this study was to further describe variables influencing survival in a large cohort of patients with acinic cell carcinoma. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) registry, we obtained demographic, clinicopathologic, and treatment data pertaining to patients diagnosed with acinic cell carcinoma. Kaplan-Meier and Cox regression analyses were performed to compare survival with various clinical and pathological parameters. RESULTS: We identified 2061 patients with acinic cell carcinoma from 1973 to 2009. Sex, staging, grade, subsite, and treatment were significant predictors of disease-specific survival (DSS). Patients who received surgery alone had the highest 20-year DSS (92.4%), followed by those treated with surgery and radiation (71.9%) or radiation alone (62.3%). CONCLUSION: Our results suggest that histologic grade is a stronger predictor of survival than TNM classification, survival after surgical resection alone is excellent, and adjuvant radiation may be of limited benefit

Spindle cell carcinoma of the upper aerodigestive tract: an analysis of 341 cases with comparison to conventional squamous cell carcinoma.

OBJECTIVES: We sought to better characterize spindle cell carcinoma (SpCC) of the upper aerodigestive tract, a rare and aggressive variant, through comparison of a large cohort of head and neck SpCCs against a cohort of conventional head and neck squamous cell carcinoma (SCC) patients. METHODS: We compared epidemiologic and clinicopathologic characteristics of 341 SpCCs with 67 882 SCCs of the head and neck, drawing data from the SEER national database. We also compared disease-specific survivals (DSS) for SpCC and SCC based on tumor site and mode of treatment. RESULTS: SpCCs were predominantly laryngeal (46.4%, P < .001) and were more likely to be high grade (P > .001). SpCCs were also more likely than SCCs to present at an early stage (P < .001 to P < .05). Rates of distant metastasis were similar between the tumor types. DSS was similar between SpCCs and SCCs, although site-specific survival rates were higher for SpCCs of the larynx (P = .017) and lower for those of the oral cavity (P = .008). CONCLUSION: SpCC of the head and neck is more likely than SCC to present at an early stage, with fewer nodal metastases. Survival rates appear to depend on anatomic site as well.

Case-control analysis of survival outcomes in sinonasal acinic cell carcinoma.

BACKGROUND: Acinic cell carcinoma (AciCC) is an uncommon malignant-epithelial salivary gland cancer. In very rare cases, this tumor may arise from sinonasal subsites, with only 19 cases described in the English-language literature. METHODS: We performed a retrospective analysis of 18 cases of AciCC, obtained from searching the Surveillance, Epidemiology, and End Results (SEER) database between 1973 and 2009. RESULTS: Patient demographics, including age, gender, and race, were similar to AciCC cases found in more common locations. However, all patients had low-grade tumors without regional or distant metastases. Comparing these patients to a stage, grade, and treatment-matched cohort of parotid AciCC, we found a lower 10-year overall survival (52.3%) with no significant difference in disease-specific survival (88.9%). Our meta-analysis of survival from cases in the literature estimated 10-year recurrence-free survival at 92.9%. CONCLUSION: To our knowledge, this is the largest cohort of patients with AciCC reported in the English-language literature. Our results suggest that patients with sinonasal AciCC have excellent disease-specific survival, comparable to matched patients with AciCC in more common salivary gland subsites.

Basaloid squamous cell carcinoma of the oral cavity: an analysis of 92 cases.

OBJECTIVES/HYPOTHESIS: Basaloid squamous cell carcinoma (BSCC) is considered a rare and possibly more aggressive squamous cell carcinoma (SCC) variant. Until now, a series of exclusively oral cavity BSCC patients has not been previously reported. We endeavored to compare BSCC and SCC of the oral cavity, focusing on epidemiologic factors and survival outcomes. STUDY DESIGN: Retrospective analysis of population-based data. METHODS: We compared epidemiologic factors, clinicopathologic data, and disease-specific survivals (DSS) between 92 patients with oral cavity BSCC and 15,181 patients with SCC. RESULTS: High-grade tumors and distant metastases were more common in the BSCC group (P≤0.001). On multivariable analysis controlling for disease stage, BSCC patients had similar DSS to those with typical SCC (P=0.231). Although there was a trend favoring definitive radiotherapy for BSCC, there were no significant differences in treatment approach between BSCC and SCC. The choice of treatment modality (radiation, surgery±radiation) did not reveal a difference in DSS between the two tumor subtypes. CONCLUSION: Analysis of the largest oral cavity BSCC series to date demonstrates that BSCC of the oral cavity carries a comparable prognosis to conventional-type oral SCC. LEVEL OF EVIDENCE: 2b.

Liposarcoma of the head and neck: analysis of 318 cases with comparison to non-head and neck sites.

BACKGROUND: Liposarcomas are rare in the head and neck. We analyzed a large series of head and neck liposarcomas to determine features unique to the head and neck. METHODS: Three hundred eighteen liposarcomas of the head and neck were contrasted with 9485 liposarcomas of other regions using the Surveillance, Epidemiology, and End Results (SEER) database. RESULTS: Head and neck liposarcomas were most commonly subcutaneous (81.%), low grade (70.1%; p < .001), and early stage (p < .001). They were more likely to be treated with surgery alone, whereas conventional liposarcomas were more likely to receive adjuvant radiation (p < .001). Treatment that included surgery had better survival than radiation therapy alone (p = .008). Overall, liposarcomas of the head and neck had significantly higher disease-specific survival (DSS) and overall survival (OS) than conventional liposarcomas (p < .001). CONCLUSION: Liposarcomas of the head and neck are usually early stage, low grade, and with fewer nodal metastases than conventional liposarcomas. DSS and OS were significantly greater for liposarcomas of the head and neck.

Epidemiologic evidence of superior outcomes for extramedullary plasmacytoma of the head and neck.

OBJECTIVE: To compare clinicopathologic data, epidemiologic factors, and survival outcomes between extramedullary plasmacytomas of the head and neck vs other body sites. STUDY DESIGN: Analysis of a national database. SETTING: Extramedullary plasmacytoma (EMP) is a rare B-cell neoplasm with a tendency to occur in the head and neck. Due to limited clinical data, few studies have conducted a thorough comparison between head and neck EMPs and those arising at other body sites. SUBJECTS AND METHODS: We compared 690 EMPs of the head and neck with 495 non-head and neck EMPs, evaluating disease-specific survival (DSS) and overall survival (OS) based on tumor type, location, grade, stage, and treatment. RESULTS: Head and neck EMPs more often affected men (P > .001) and were typically located in the pharynx (21.5%), nasal cavity (19.3%), oral cavity (14.7%), and paranasal sinuses (13.0%). Non-head and neck EMPs were more often limited to only local disease at presentation (94.8% vs 82.1%-86.7%, P < .001). Patients with non-head and neck EMP more often received surgery alone, whereas those with head and neck EMP were more often treated with combination therapy or radiation alone (P < .001). Head and neck EMPs had a significantly higher 5-year DSS and OS than other plasmacytomas (P < .001), and they had a higher 10-year OS when treated with surgery or combination therapy than with radiation alone (P = .003). CONCLUSION: Extramedullary plasmacytoma of the head and neck represents a type of tumor unique from other plasmacytomas, with distinct epidemiologic characteristics and a superior prognosis. Evidence suggests that surgically based treatments may offer improved outcomes.

The role of TASK1 in aldosterone production and its expression in normal adrenal and aldosterone-producing adenomas.

OBJECTIVES: Aldosterone production in the adrenal glomerulosa is mainly regulated by angiotensin II and K+. Adrenal glomerulosa cells are uniquely sensitive to extracellular K+. Genetic deletion of subunits of K+-selective leak-channels (KCNK), TASK1 and/or TASK3, in mice generates animals with hyperaldosteronism and histological changes in the adrenal cortex. Herein, we studied the expression of TASK1 in human adrenocortical cells, as well as its role in aldosterone production in H295R cells. DESIGN: TASK1 expression was investigated by comparative microarray analysis of aldosterone-producing adenomas (APA) and normal adrenals (NAs). The effects of TASK1 knockdown by siRNA transfection were investigated in H295R cells. Fluo-4 fluorescent measurements of intracellular Ca2+ and pharmacological inhibition of Ca2+ -dependent calmodulin kinases (CaMK) were performed to better define the effects of TASK1 on Ca2+ signalling pathways. RESULTS: Microarray analysis of APA and NA showed similar expression of TASK1 between these two groups. However, in APA, NA and H295R cells the expression of TASK1 was predominant when compared with other KCNK family members. Knockdown of TASK1 (with siRNA) induced the expression of steroidogenic acute regulatory (StAR) protein and aldosterone synthase (CYP11B2), and also stimulated pregnenolone and aldosterone production. Cells transfected with siTASK1 had increased intracellular Ca2+, leading to activation of CaMK and increased expression of CYP11B2. CONCLUSIONS: Our study reveals the predominant expression of TASK1 over other KCNK family genes in the human adrenal cortex. Herein, we also described the role of TASK1 in the regulation of human aldosterone production through regulation of intracellular Ca2+ and CaMK signalling pathways.