Liam Masterson

Management of tumors arising from the parapharyngeal space: A systematic review of 1,293 cases reported over 25 years

We present a systematic review of 1,252 lesions published in the past 25 years, the largest to date. We also include our own experience of 41 cases.Objectives We present a systematic review of 1,252 lesions published in the past 25 years, the largest to date. We also include our own experience of 41 cases. Data Sources Our data sources were MEDLINE and Embase databases. Review Methods A systematic review of the literature (1988–2014) was performed and data on histological diagnosis, presentation, surgical approach, and postoperative complications were reviewed. Results In total, 22 studies (including our own institution) revealed 82% of lesions were benign. The most common presentation was an intraoral mass (52%) followed by a cervical mass (48%), and the most common primary lesion was a pleomorphic adenoma (29%). Ninety-six percent of patients underwent surgery. The cervical approach was most frequently used (46%), and the most common complication was vagus nerve injury (13%). Within our institution, 5-year progression-free survival for benign and malignant disease was 93% and 61%, respectively (P = .196). Conclusions A parapharyngeal space mass is an uncommon tumor and requires careful preoperative assessment. Definitive treatment is usually surgery, and these patients should be counseled appropriately about the potential for permanent cranial nerve deficits. Level of Evidence NA Laryngoscope, 125:1372–1381, 2015

Squamous Cell Carcinoma of the Temporal Bone: Clinical Outcomes From Radical Surgery and Postoperative Radiotherapy

Objective To review the treatment of squamous carcinoma of the temporal bone at a regional skull base unit for the period 1982–2012. Study Design Retrospective case review. Setting Tertiary referral center. Patients Sixty patients with primary squamous carcinoma of the temporal bone. Interventions Multidisciplinary team approach including surgical resection, reconstruction, and postoperative radiotherapy. Main Outcome Measures Disease-specific survival, overall survival. Results The 5-year disease-specific survival for the whole cohort was 44% (CI, 37%–51%). Multivariable analysis revealed nodal status, poorly differentiated squamous cell histology, and carotid involvement to be poor prognostic indicators. Conclusion Although the survival figures in this series are comparable with the best outcomes from other units, our experience would suggest improvements can still be achieved by reconsidering the selection of patients for neck dissection and temperomandibular joint excision in early stage disease. We also conclude that postoperative radiotherapy should be delivered to all patients, including surgical salvage cases who may have received previous irradiation. Finally, the minority of patients with poor prognostic features should be offered a more palliative therapeutic approach.

Systematic Review of Management Strategies for Middle Ear Myoclonus

Objective. Middle ear myoclonus is a rare condition with distinct characteristics at presentation. Diagnosis is based primarily on history, clinical examination, and long-time-based tympanometry. No consensus exists regarding treatment at present. This review was designed to identify relevant studies on current investigation and management. Data Source. A systematic electronic literature search of MEDLINE, Embase, Cumulative Index to Nursing and Allied Health Literature (CINAHL), Cochrane Trials register, and Web of Science was conducted for articles describing middle ear myoclonus through to May 2011. English- and non-English-language articles that focused on investigation and treatment were considered for review. Review Method. Two authors independently reviewed the articles for study design, treatment, intervention, and outcome. Data from human and experimental studies were considered. Results. A total of 21 articles were identified for this review. Most studies were found to be case reports or small case series. In general, there was no evidence of a conclusive diagnostic test or treatment for middle ear myoclonus. Conclusion. There is a need for high-quality prospective controlled trials to determine the most effective management of middle ear myoclonus. The authors describe a treatment algorithm based on the data available and clinical knowledge.

Langerhans cell sarcoma: A systematic review

Langerhans cell sarcoma (LCS) is a rare malignant tumour of Langerhans cells with a poor outcome. Given its rarity, there is a lack of evidence regarding the most appropriate treatment for this condition. Therefore the aim of this work was to review, compile, analyse and present clinical details and to determine the optimal treatment regimen. A search of PubMed, CINAHL, EMBASE, Cochrane, CENTRAL, clinicaltrials.gov and Google Scholar was supplemented by hand searching. Data extracted included demographics, treatment, type of LCS and clinical outcome. Of 510 citations identified by a systematic literature search, 46 case series including 66 subjects with LCS met criteria for analysis. The most common treatment modality was chemotherapy, used alone or in combination in 47 cases (71%) followed by surgery in 31 cases (47%). Overall mean (S.E.) disease specific survival and disease free survival were 27.2 (3.9) and 18.3 (3.8) months respectively. There was a significant difference in both disease specific and disease free survival between the local, loco-regional and disseminated disease cohorts (DSS p=0.014; DFS p<0.001). More localised disease confers a survival advantage. Multi-modality therapy appears to be most effective, with the addition of radiotherapy to chemotherapy appearing beneficial. Complete surgical excision with clear margins being most effective for local disease control. Any adjuvant therapy should not be delayed. Bone marrow transplant appears to be the most reliable treatment in terms of outcome especially in disseminated disease however has well known patient selection and toxicity/tolerance issues. The role of cell surface markers for prognostication remains unclear.

Deregulation of SYCP2 predicts early stage human papillomavirus-positive oropharyngeal carcinoma: A prospective whole transcriptome analysis.

This study was designed to identify significant differences in gene expression profiles of human papillomavirus (HPV)‐positive and HPV‐negative oropharyngeal squamous cell carcinomas (OPSCC) and to better understand the functional and biological effects of HPV infection in the premalignant pathway. Twenty‐four consecutive patients with locally advanced primary OPSCC were included in a prospective clinical trial. Fresh tissue samples (tumor vs. matched normal epithelium) were subjected to whole transcriptome analysis and the results validated on the same cohort with RT–quantitative real‐time PCR. In a separate retrospective cohort of 27 OPSCC patients, laser capture microdissection of formalin‐fixed, paraffin‐embedded tissue allowed RNA extraction from adjacent regions of normal epithelium, carcinoma in situ (premalignant) and invasive SCC tissue. The majority of patients showed evidence of high‐risk HPV16 positivity (80.4%). Predictable fold changes of RNA expression in HPV‐associated disease included multiple transcripts within the p53 oncogenic pathway (e.g. CDKN2A/CCND1). Other candidate transcripts found to have altered levels of expression in this study have not previously been established (SFRP1, CRCT1, DLG2, SYCP2, and CRNN). Of these, SYCP2 showed the most consistent fold change from baseline in premalignant tissue; aberrant expression of this protein may contribute to genetic instability during HPV‐associated cancer development. If further corroborated, this data may contribute to the development of a non‐invasive screening tool. This study is registered with the UK Clinical Research Network (ref.: 11945).

Quality-of-life outcomes after sinus surgery in allergic fungal rhinosinusitis versus nonfungal chronic rhinosinusitis.

Background Given the differences in pathophysiology between allergic fungal rhinosinusitis (AFRS) and other chronic rhinosinusitis (CRS) subgroups, it remains unclear about whether these patients respond differently to a combination of surgical and medical treatments. Objective To evaluate differences in quality-of-life (QoL) outcomes for a cohort of patients who underwent endoscopic sinus surgery (ESS) for CRS. Methods This retrospective review included patients with CRS who underwent ESS between 2010 and 2013. QoL was measured by using the 22-item Sino-Nasal Outcome Test (SNOT-22). Variables collected included baseline demographics, SNOT-22 scores before ESS and at 1, 3, 6, 9, and 12 months after ESS. Groups tested were CRS with nasal polyposis, CRS without nasal polyposis (CRSsNP), and patients with AFRS. A linear mixed- effects regression model was used to calculate the adjusted mean QoL differences. Results Among the 250 patients included, 61.6% had CRS with nasal polyposis (n = 154), 28.8% had CRSsNP (n = 72), and 9.6% had AFRS (n = 24). Significant differences were seen in SNOT-22 scores between pre- and postoperative visits and between the etiologic subgroups (p < 0.001). Multivariate analysis revealed significantly greater improvement in QoL for patients with AFRS in comparison with those with CRSsNP at the 9-month follow-up (change in SNOT-22 score, 22.6 [95% confidence interval, 1.2–44.1]; p < 0.0) and the 12-month follow-up (change in SNOT-22 score, 20.2 [95% confidence interval, 0.5–39.9]; p < 0.04). Conclusions Patients with AFRS experienced a more-prolonged QoL benefit from surgical and targeted medical intervention compared with those with CRSsNP, which may reflect the severity of inflammation that they presented with compared with other CRS subtypes.

Assessment and management of facial nerve palsy

#### The bottom line #### How patients were involved in the creation of this article We sought feedback on the paper from patient and medical representatives of the charity Facial Palsy UK. We incorporated their comments into the paper and developed a patient consultation guide for management and prognosis of Bell’s palsy (see box below) The facial nerve is important for both communication and expression, and impairment of its function can severely affect quality of life.1 The main concern at first presentation of a facial nerve lesion is to exclude the possibility of a stroke or other serious cause.2 The figure⇓ outlines possible causes. Correct management within the first few days may prevent long term complications. Differential diagnosis of a unilateral facial palsy. Percentages are based on combined epidemiological data from 6024 patients with lower motor neurone facial palsy (rarer conditions including mumps, syphilis, HIV, Guillain-Barre syndrome, otitic barotrauma, myasthenia gravis, systemic lupus erythematosus, sarcoidosis, and multiple sclerosis have been excluded).3 4 *Endemic in forested regions; †misdiagnosed cerebrovascular disease evident in about 1.5% of all patients3 The facial nerve is responsible for motor supply to the muscles of facial expression (frontalis, orbicularis oculi, buccinators, and orbicularis oris) and stapedius, parasympathetic supply to the lacrimal and submandibular glands, …

Conservative or radical surgery for pediatric papillary thyroid carcinoma: A systematic review of the literature

BACKGROUND Pediatric papillary thyroid carcinoma (PTC) is characterized by an aggressive clinical course. Early diagnosis is a challenge and treatment consists principally of partial or total thyroidectomy±neck dissection and radioactive iodine therapy. Due to the rarity of PTC in children, there is no consensus on optimal surgical treatment. METHODS AND RESULTS A literature search was carried out using PubMed, Embase, Medline, Cochrane and Web of Science. Seven studies (489 patients) investigating the outcome of surgically managed pediatric PTC were identified. No clear advantage in survival or recurrence rate was found for total thyroidectomy compared to other surgical approaches. CONCLUSION Despite the aggressive behavior of PTC, prognosis is good, with low mortality. After removal of disease and prevention of recurrence, reduction of iatrogenic complications are a priority in this age group. Due to the paucity of available evidence, this review cannot recommend conservative or radical surgery for pediatric papillary thyroid carcinoma. To answer this question, we recommend the establishment of a randomized controlled trial with adequately matched baseline variables.

Voice symptoms in patients with autoimmune disease:a cross-sectional epidemiological study

Objective To assess the prevalence and severity of voice symptoms in individuals with a diagnosis of autoimmune disease. Study Design Cross-sectional survey. Setting Study participants were recruited from a rheumatology tertiary referral clinic at Norfolk and Norwich University Hospital. Subjects and Methods A cross-sectional questionnaire analyzing 109 patients with autoimmune disease (rheumatoid arthritis, seronegative spondyloarthritis, connective tissue disease) and a control group of 41 patients with non-autoimmune disease (osteoarthritis/osteoporosis). Main outcome measures were the Voice Handicap Index–10 (VHI-10), xerostomia scale, acid reflux inquiry, and anxiety/depression scale. Results Patients with autoimmune disease were more likely to experience voice symptoms as assessed by the VHI-10 questionnaire (P = .0035). Subgroup analysis showed autoimmune patients were more likely to report voice symptoms regardless of whether they were on a disease-modifying antirheumatic drug (DMARD; P = .0010) or non-DMARD (P = .017), suggesting autoimmune disease may be an independent risk factor from pharmacotherapy. Xerostomia was more common in an autoimmune population compared with the control group (P = .02). A positive correlation between xerostomia and VHI-10 scores was found for the DMARD group (Spearman rank coefficient = 0.49, P < .001). No significant difference in acid reflux inquiry (P = .44) or the anxiety/depression scale (P = .36) was found when comparing the autoimmune and control groups. Conclusion Patients with autoimmune disease have increased likelihood of voice symptoms when compared with a control population with non-autoimmune disease. Further prospective studies to elucidate the cause of voice disorder would be valuable.

Kaposiform hemangioendothelioma of paranasal sinus.

Kapossiform hemangioendothelioma (KHE) of the paranasal sins (PNS) is a rare cause of recurrent epistaxis. To date, only two cases of PNS KHE have been reported in the literature, both occurring in the pediatric population. The case presented here appears to be the first case of PNS KHE occurring in an adult.