Chondrosarcoma of the hyoid bone: a case of mistaken identity in the submandibular region.

We describe a case of woman in her 60s who presented with a painless mass in her left submandibular region that biopsies and imaging suggested was a pleomorphic adenoma. Intraoperative findings showed a submandibular mass originating from the hyoid bone, and subsequent histopathological examination showed a grade 2 chondrosarcoma of the hyoid bone. Chondrosarcomas in the head and neck region are uncommon and presentations in the hyoid bone are very rare with only approximately 27 previously published cases. Presentations that pose as more benign pathology can cause diagnostic dilemmas for clinicians.

A prevertebral desmoplastic fibroblastoma presenting with sleep-related breathing dysfunction.

Desmoplastic fibromas exceedingly rarely present in the retro/parapharyngeal space but should be considered in differential diagnosis for benign lumps in these anatomical regions.

Sorafenib in metastatic thyroid cancer: a systematic review.

BACKGROUND: Sorafenib was recently approved by the U.S. Food and Drug Administration for radioiodine-resistant metastatic differentiated thyroid cancer (DTC). In addition, two drugs (vandetanib and cabozantinib) have received U.S. Food and Drug Administration approval for use in medullary thyroid cancer (MTC). Several published phase II trials have investigated the efficacy of sorafenib in thyroid cancers, but to date, results from those studies have not been compared. METHODS: A systematic review of the literature was performed to assess response rate, median progression-free survival, and adverse events associated with sorafenib therapy for metastatic thyroid cancers. RESULTS: This review included seven trials involving 219 patients: 159 with DTC (papillary, follicular, and poorly differentiated), 52 with MTC, and 8 with anaplastic thyroid cancer. No study reported complete responses to treatment. Overall partial response, stable disease, and progressive disease rates were 21%, 60%, and 20%, respectively. The median progression-free survival was 18 months for patients with all subtypes of thyroid cancer. Drug was discontinued in 16% of patients because of toxicities or intolerance, and the dose was reduced in a further 56%. Side effects with an incidence ≥ 50% were hand-foot syndrome (74%), diarrhea (70%), skin rash (67%), fatigue (61%), and weight loss (57%). Deaths not related to progressive disease occurred in nearly 4% of patients. CONCLUSION: Treatment with sorafenib in patients with progressive DTC and MTC is a promising strategy, but the adverse event rate is high, leading to a high rate of dose reduction or discontinuation. Consequently, sorafenib use in patients with metastatic thyroid cancer requires careful selection of patients and careful management of side effects.

Coordinated targeting of the EGFR signaling axis by microRNA-27a*.

Epidermal growth factor receptor (EGFR) has been characterized as a critical factor in the development and progression of multiple solid tumors, including head and neck squamous cell carcinoma (HNSCC). However, monotherapy with EGFR-specific agents has not been as dramatic as preclinical studies have suggested. Since complex regulation of the EGFR signaling axis might confound current attempts to inhibit EGFR directly, we searched for microRNAs (miRNAs) that may target the EGFR signaling axis. We identified miR-27a (miR-27a-3p) and its complementary or star (*) strand, miR-27a* (miR-27a-5p), as novel miRNAs targeting EGFR, which were significantly downregulated in multiple HNSCC cell lines. Analysis of human specimens demonstrated that miR-27a* is significantly underexpressed in HNSCC as compared to normal mucosa. Increased expression of miR-27a* in HNSCC produced a profound cytotoxic effect not seen with miR-27a. Analysis for potential targets of miR-27a* led to the identification of AKT1 (protein kinase B) and mTOR (mammalian target of rapamycin) within the EGFR signaling axis. Treatment with miR-27a* led to coordinated downregulation of EGFR, AKT1 and mTOR. Overexpression of EGFR signaling pathway components decreased the overall effect of miR-27a* on HNSCC cell viability. Constitutive and inducible expression of miR-27a* in a murine orthotopic xenograft model of oral cavity cancer led to decreased tumor growth. Direct intratumoral injection of miR-27a* inhibited tumor growth in vivo. These findings identify miR-27a* as a functional star sequence that exhibits novel coordinated regulation of the EGFR pathway in solid tumors and potentially represents a novel therapeutic option.

Molecular-targeted therapy hypoxia in head and neck squamous cell carcinoma patients.

Despite advances in surgical techniques, radiotherapy, and chemotherapy, 5-year survival in patients with late-stage head and neck squamous cell carcinoma (HNSCC) have not improved significantly over the past decades. HNSCC tumors are commonly associated with hypoxia, which is characterized by an acute and/or chronic decline in oxygen tension. Hypoxia is an important cancer-aggravating microenvironmental factor that contributes to malignant behaviors such as acquisition of antiapoptotic ability by cancer cells and tumor progression, invasion, metastasis, and resistance to chemotherapy and radiotherapy. Numerous studies have assessed tumor hypoxia and identified molecular markers that are promising therapeutic targets in HNSCC cases. Moreover, investigators have suggested a number of molecular strategies to target cell processes critical to hypoxia development in HNSCC patients via the direct or indirect regulation of hypoxia-inducible factor-1α expression in cancer cells. In this review, we described recent advances in the identification and development of molecular-targeted therapy targeting hypoxia in HNSCC patients.

Long-term quality of life in young adults treated for oral cavity squamous cell cancer.

OBJECTIVES: We assessed the long-term quality of life (QOL) in patients who survived oral cavity squamous cell cancer when they were young and looked for any clinical factors that might adversely affect function and QOL. METHODS: We performed a retrospective case series and questionnaire survey in a tertiary care center. The subjects were consecutive patients treated for oral cancers during a 25-year period, when they were 40 years of age or less. The patients completed the University of Washington Quality of Life questionnaire and the M. D. Anderson Dysphagia Inventory (MDADI). We made an overall descriptive report of swallowing and QOL measures in the study population and looked for any clinical factors associated with functional outcomes. RESULTS: Among the 62 patients treated over the course of 25 years, 46 were alive and disease-free. Twenty-six participated. The median follow-up duration was 14.7 years (range, 3 to 27 years). Age at diagnosis and duration of follow-up did not correlate with overall QOL or health-related QOL. Seventy-seven percent rated their overall QOL as outstanding, very good, or good. The key domains affected by cancer were appearance, mood, saliva, and shoulder function. Radiotherapy significantly adversely affected the QOL. The median MDADI scores on all 4 subscales were at least 85%. Higher T-stage and radiotherapy were significantly associated with lower scores on all subscales. CONCLUSIONS: The long-term health-related QOL in this cohort was quite good. Radiotherapy and tumor stage correlated with swallowing outcomes, and only radiotherapy seemed to adversely affect the overall QOL.

Prognostic features, human papillomavirus status, and epidermal growth factor receptor expression in oral squamous cell carcinoma in young adults.

PURPOSE: There is still debate in literature about the survival outcomes of patients who have cancer of the oral cavity when young. Hence the aims were (1) to estimate disease-free survival, overall survival, and cause-specific survival in patients who developed oral cavity squamous cell carcinoma between 18 and 40 years of age and (2) to assess the clinicopathologic factors including detection of human papillomavirus and epidermal growth factor receptor (EGFR) overexpression in primary lesions affecting recurrence. METHODS: This is a retrospective case-note review and reevaluation of histopathologic slides of patients treated more than 25 years. Descriptive statistics, Cox proportional hazard models, and Kaplan-Meier survival curves were used for statistical analysis. RESULTS: A total of 62 patients were treated, with mean follow-up of 11.4 years. Forty-five were oral tongue tumors and 43 had stage I or II disease. The 5-year disease-free survival was 73.5%. The 10-year overall survival and cause-specific survival rates were 81.8% and 83.4%, respectively. Smoking and alcohol intake were not seen as risk factors in this population. Multivariate modeling identified only nodal involvement as significantly associated with overall survival and only extracapsular spread as significantly associated with locoregional recurrence. At 5 years after treatment, the cause-specific survival was 100% for patients with low EGFR expression and 81.1% for patients with high EGFR expression (hazard ratio for high vs low, 3.1; 95% confidence interval, 0.4-406.9; P = .46). Human papillomavirus was not detected in all but 2 tumor specimens. CONCLUSIONS: Survival outcomes are quite good in young patients with oral cancer.

Open conservation partial laryngectomy for laryngeal cancer: a systematic review of English language literature.

BACKGROUND: Different modalities of treatment in early laryngeal cancer lead to equivalent oncological outcomes. Hence this systematic review was undertaken to synthesise the key oncological outcomes following primary open partial laryngectomy for laryngeal cancer. METHODS: A systematic review of the English literature with statistical pooling of outcomes, the main outcome measure being local control at 24 months. RESULTS: A total of 53 articles satisfied inclusion criteria and were included in the review. The pooled local control rate at 24 months from 5061 patients was 89.8% (95% CI 88.3-91.2), pooled overall survival was 79.7% (n = 3967; 95% CI 76.5-782.8) and pooled mean disease free survival was 84.8% (n = 2344; 95% CI 80.6-88.7). The pooled mean operative mortality, laryngectomy for function, tracheostomy decannulation and permanent gastrostomy rates were 0.7%, 1.7%, 96.3%, and 2.0%, respectively. CONCLUSIONS: Open conservation laryngectomy is a good option in selected primary laryngeal cancers with excellent oncological outcomes.

Oncologic outcomes of open conservation laryngectomy for radiorecurrent laryngeal carcinoma: a systematic review and meta-analysis of English-language literature.

BACKGROUND: The role of open conservation (partial) laryngeal surgery in radiorecurrent laryngeal cancers is unclear, and the procedure is not widely accepted or practiced. The objective of this review was to assess the oncologic and functional outcomes of partial laryngectomy in radiorecurrent tumors of the larynx reported in the literature. METHODS: The systematic review was performed using independently developed search strategies and included Medline, Embase, Zetoc, conference proceedings, and, when appropriate, a manual search. Inclusion criteria for the articles were set a priori. All included articles were subjected to quality assessment. Pooled estimates of local control at 24 months and of disease-free and overall survival rates were calculated using both a fixed-effects model (inverse square) and a random-effects model (DerSimonian-Laird). RESULTS: The search identified 401 publications, of which 26 studies satisfied all inclusion criteria. Ten studies had a quality score ≥6 (good), and 16 had a score of 4 or 5 (fair). The pooled estimates of oncologic outcomes using the random-effects model were as follows: The local control rate at 24 months for 560 patients was 86.9% (95% CI, 84%-89.5%), the disease-free survival rate for 352 patients was 91.2% (95% CI, 88.2%-93.9%), and the overall survival rate for 360 patients was 83.1% (95% CI, 79.1%-86.7%). Decannulation of tracheostomy occurred in 95.1% (95% CI, 92.6%-97.2%) of the patients who were analyzed (n = 315), whereas the pooled mean larynx preservation rate was 83.9% (95% CI, 80.7%-87%; n = 502) CONCLUSIONS: The current results indicated that open partial laryngectomies are oncologically sound procedures in the salvage setting and have a high larynx preservation rate.

Day case tonsillectomy for the treatment of obstructive sleep apnoea syndrome in children: Alder Hey experience.

BACKGROUND: The aim of this study was to assess the safety of paediatric adeno-tonsillectomy for obstructive sleep apnoea syndrome (OSAS) performed as a day case procedure. METHODOLOGY: Postoperative airway related complications were prospectively evaluated in children undergoing adeno-tonsillectomy for OSAS over a 12-month period in a tertiary care centre. All data was analysed and descriptive statistics performed. RESULTS: The overall incidence of postoperative airway related complications were 21 (10.4%) in 202 consecutively operated children. Eleven were in children with other medical co-morbidities (n=37) of which 8 occurred after 6h. Amongst those without any medical illnesses (n=165), two (1.2%) developed OSAS related complication after 6h of surgery only one of which needed medical/nursing intervention. The incidence of postoperative desaturations after 6h was 21.6% in those with co-morbidities. CONCLUSION: It is safe to perform day case adeno-tonsillectomy for OSAS in children without any medical co-morbidities while those with other medical illnesses should be planned as in-patient procedures.

Three-dimensional CT reconstruction and virtual endoscopic study of the ostial orientations of the frontal recess.

BACKGROUND: Safe and efficient endoscopic frontal sinus surgery requires a thorough knowledge of the location of the frontal sinus outflow tract. This study was designed to describe the prevalence of the different frontal recess cells and 3D analysis by virtual endoscopy of their ostial orientation in relation to the frontal ostium. METHODS: Fifty high-resolution CT scans (100 sides) of disease-free paranasal sinuses were reviewed using triplanar and virtual 3D analysis. The prevalence and ostial locations of the frontal recess cells and the relationship of the various ostia to the frontal sinus ostium were mapped and analyzed. RESULTS: The cell type, prevalence, and most common relationship of the cell's ostium to the frontal ostium were intersinus septal cells, 7% medial and anterior; Kuhn type 1 cells, 28%, lateral and anterior; Kuhn type 2 cells, 6%, anterior and lateral; Kuhn type 3 cells, 11%, medial or lateral; supraorbital ethmoid cells, 11%, posterior; suprabullar cells, 68%, posterior; and frontal bullar cells, 16%, posterior or lateral. In 35% of the sides, none of the aforementioned cell types were found. There were many blind recesses that were seen in all locations except anterolateral to the frontal ostium. Openings of adjacent pneumatized middle turbinates were often posteromedial to the frontal ostium. Agger nasi cells were present in at least 86%, opening lateral to frontal ostium. CONCLUSION: Frontal outflow tract mapping by virtual endoscopy and knowledge of the ostial configurations will help surgeons perform more efficient and accurate surgery particularly for minimally invasive approaches including balloon dilation.

Inflammatory pseudotumour of the maxillary sinus presenting as a sino-nasal malignancy.

Inflammatory pseudotumours are a rare group of benign neoplasms of unknown aetiology. They are uncommon in head and neck sites, particularly the paranasal sinuses. Surgical excision, radiotherapy and steroids have all been used as treatment modalities. A report is made of a 27-year-old female who presented with a rapid-onset proptosis that clinically and radiologically mimicked an aggressive sino-nasal malignancy. Histology eventually confirmed an inflammatory pseudotumour of the maxillary sinus. The patient was treated successfully with a combination of surgery and steroid therapy.