Survival differences of low-grade versus high-grade head and neck pleomorphic dermal sarcomas and a review of a scalp case.

Objective: To examine survival by tumor grade of pleomorphic dermal sarcomas (PDS) of the head and neck (H&N) and review a scalp PDS case. Methods: Patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1980 to 2016 based on a diagnosis of H&N PDS. Survival estimates were performed using Kaplan-Meier analysis. Additionally, a case of a grade III H&N PDS is presented. Results: Two hundred-seventy cases of PDS were identified. The mean age at diagnosis was 75.1 years (SD: 13.5). Two hundred-thirty-four (86.7%) patients were male. Eighty-seven percent of patients received surgery as a part of their care. The 5-year overall survival rates for grades I, II, III, and IV PDSs were 69%, 60%, 50%, and 42%, respectively (P = 0.03). Conclusions: H&N PDS occurs most commonly in older-age males. Surgical management is frequently a part of H&N PDS care. Survival rates significantly decline based on tumor grade.

Surgical Management Patterns of Sinonasal Malignancy: A Population-Based Study.

Objectives Determining surgical trends and outcomes for sinonasal tumors is challenging given their low incidence and heterogeneous pathology. This study utilized the National Cancer Database (NCDB) to identify trends and outcomes associated with surgical management of sinonasal tumors. Design Retrospective database analysis. Setting National Cancer Database. Participants Patients with sinonasal malignancies identified from the NCDB between 2010 and 2015. Main Outcome Measures The primary outcome was the choice of surgical therapy used for sinonasal tumor resection: endoscopic versus open approach. Each was cohort analyzed with respect to various demographic and clinicopathologic factors. A treatment effect model was used to identify potential differences between surgical approaches. Survival was evaluated using Kaplan-Meier analysis. Results A total of 10,193 patients with sinonasal malignancies were identified in the NCDB database; of these, 2,292 had a documented subsite, histology, and definitive surgical treatment with documented surgical approach and were included in the analysis. About 71.9% of patients had an open approach and 28.1% a purely endoscopic procedures. Tumor histology, treatment facility type, margin status, and length of stay were all variables that were associated with significant differences between the open and endoscopic cohort. Five-year survival rates for the open and endoscopic cohorts were not significantly different (59.6 and 60.8%, respectively). Conclusions Assessment of the NCDB revealed that 28% patients with sinonasal malignancy were selected for endoscopic surgery. These patients had comparable oncologic outcomes to open resection.

Timing of surgery and adjuvant radiation therapy for sinonasal malignancies: Effect of surgical approach.

BACKGROUND: Timely postoperative radiation therapy (RT) within 50 days of surgery for head and neck cancers provides a survival advantage. METHODS: Using the National Cancer Database, we performed a propensity score-matched analysis comparing patients undergoing open or endoscopic surgery for squamous cell carcinoma (SCC) of the nasal cavity and paranasal sinuses from 2010 to 2015. RESULTS: Among 168 pairs, patients undergoing endoscopic surgery had shorter time to surgery (24.2 vs 36.7 days, P < .001) and shorter postoperative time to RT (PTTR, 51.2 vs 58.4 days, P = .02). On multivariable linear regression, endoscopic surgery predicted shorter PTTR (ß = -7.6, P = .01). Using the Kaplan-Meier method, patients in the longest PTTR quartile had decreased overall survival (OS; Q1 vs Q4, 3-year OS 76.5% vs 53.3%, P = .007), a durable finding when adjusted for covariates (Q1 vs Q4, HR 0.50, P = .008). CONCLUSIONS: Patients undergoing endoscopic surgery for sinonasal SCC experience shorter PTTR. Shorter PTTR is associated with extended OS.

Comparing Kadish, TNM, and the modified Dulguerov staging systems for esthesioneuroblastoma.

BACKGROUND: Esthesioneuroblastoma (ENB) is a rare neuroendocrine tumor. The purpose of this study was to compare the Kadish, tumor-node-metastasis (TNM), and Dulguerov's modified TNM staging in order to determine the impact of the stage on primary surgical treatment selection, margin status, and survival. METHODS: The National Cancer Database (NCDB) was used to identify patients diagnosed with ENB between 2004 to 2015. Patients were excluded based on the ability to properly stage their disease as well as the availability of treatment data. RESULTS: Eight-hundred eighty-three patients had sufficient data for analysis. On multivariate analysis, age and government insurance were associated with primary surgical treatment, whereas tumor stage, gender, race, hospital type and volume, and comorbidity score were not. Age, charlson-deyo comorbidity (CDCC) score, hospital volume, and nodal status were found to be predictors of survival. Multivariate-analysis controlling for stage failed to demonstrate clear survival differences between staging in both TNM and Kadish systems. T-stage and the presence of regional nodal metastasis were associated with an increased risk of positive margins on multivariate analysis. CONCLUSION: Although primary surgical management and positive margins can be predicted by certain patient and tumor factors, clinical staging systems for ENB poorly predict prognosis over a 10-year horizon.

Iatrogenic Cushing's syndrome and adrenal insufficiency in infants on intranasal dexamethasone drops for nasal obstruction - Case series and literature review.

The use of intranasal steroid drops for nasal obstruction in infants is common practice and can prevent more invasive surgical procedures; however, it is not without complication. We describe 2 cases of iatrogenic Cushing's secondary to nasal steroids in infants with nasal obstruction, discuss the etiology of this unusual complication, and review previous literature reports. While reporting in the literature is sparse, these cases highlight the risk of development of adrenal insufficiency with usage of nasal steroid drops in infants as well as the need for close monitoring of administration and tapering of the drops. Additionally, we suggest an approach to the infant with symptomatic nasal obstruction that addresses the usage of intranasal steroid drops and emphasizes the need for quick tapering and possible endocrine consultation when appropriate.

Multiple endocrine neoplasia type 2 kindred with novel tandem RET mutations: Case report with an applied in silico mutational tolerance analysis.

BACKGROUND: The American Thyroid Association (ATA) has established guidelines for prophylactic thyroidectomy in multiple endocrine neoplasia type 2A (MEN2A) based on rearranged during transfection (RET) mutations. In silico analysis, which uses computer modeling to predict alterations in protein structure, is a new method for studying these mutations. METHODS: We describe a kindred with MEN2A, all sharing a well-documented RET mutation, p.C634Y, as well as a mutation of undetermined significance, p.I852M, which we analyzed via in silico analysis. RESULTS: The p.C634Y mutation resulted in severe predicted RET alterations, whereas the p.I852M resulted in only modest changes. Both mutations together resulted in only a small additional disruptive effect in protein structure beyond that which occurred with p.C634Y alone. CONCLUSION: Although in silico analysis may be helpful in quantitating changes in protein structure that occur in patients who have novel RET mutations (single or multiple), additional factors must account for the highly variable aggressiveness of the disease (C-cell hyperplasia/medullary thyroid carcinoma [MTC]) noted in our kindred. © 2016 Wiley Periodicals, Inc. Head Neck 38: E1881-E1885, 2016.