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Langerhans cell histiocytosis is a rare disorder in children that can involve the head and neck, may mimic common ENT conditions and delay diagnosis. While various case reports exist, ENT-focused reviews remain limited.
Methods
We conducted a literature review of 73 studies encompassing 244 paediatric patients with head and neck Langerhans cell histiocytosis, extracting data on presentation, treatment and outcomes. We also present a retrospective case series of 25 patients treated at a single tertiary institution.
Results
Otologic and craniofacial bone involvement were the most common presentations. Treatments ranged from surgical curettage and intra-lesional steroids to systemic chemotherapy. Recurrence was more common in multifocal and multisystem disease. Our institutional series supported many of these findings, with detailed follow-up data and treatment responses.
Conclusion
This is the most extensive ENT-focused review of paediatric Langerhans cell histiocytosis to date. It highlights key clinical patterns and management approaches to aid early recognition and tailored treatment.
Head and neck (HN) radiotherapy contour quality directly impacts local control (LC) and survival; however, few departments peer review (PR) contours prior to radiotherapy planning (RTP). This study reports outcomes of a single institution’s formal HN contour PR process.
Methods:
A formal HN contour PR process was implemented. Prior to RTP, HN radiation oncologists reviewed contours, provided feedback and assigned contour grades as follows: R0 (no change), R1 (minor revision, not high risk) or R2 (major revision; high risk). The PR task was completed and the contour grade was recorded. Cochran-Armitage trend test was performed.
Results:
Pilot PR process was performed over a 7-month period for 88 patients, followed by a maintenance phase. Contours were graded as follows: R0 (N = 51), R1 (N = 20) and R2 (N = 17). Over time, the number of R2 revisions decreased (p = 0.0001); month 1 (N = 7), month 2 (N = 3), month 3 (N = 5), month 4 (N = 2) and months 5–7 (N = 0). Conversely, the number of R0 revisions improved over time (p = 0.0203); month 1 (N = 5), months 2–3 (N = 9), month 4 (N = 5), month 5 (N = 8), month 6 (N = 12) and month 7 (N = 3). Each radiation oncologist demonstrated reduction in R2 revisions during the pilot. During maintenance, all 3 radiation oncologists demonstrated low rates of R2 revisions of less than 3 cases per year.
Conclusion:
Incorporation of HN contour PR into routine clinical workflow is feasible. The collective experience of multiple high-volume HN radiation oncologists led to improved contour quality in the pilot for each radiation oncologist and continued to ensure high quality in the maintenance phase.
Accurate delivery of radiotherapy to head, neck and brain cancer relies on the use of sophisticated immobilisation devices, usually using a restrictive thermoplastic mask. These masks can cause anxiety and can make treatment difficult for many patients. Open-face or maskless techniques are alternatives which can improve the patient experience. This systematic review aimed to compare the effectiveness of open-face (OF) masks and maskless surface guided radiotherapy (SGRT) with conventional masks.
Method:
Primary research papers from the last 10 years were gathered from Scopus, PUBMED, Web of Science and OVID databases. Quantitative data reporting interfractional set-up errors and intrafractional patient motion were extracted from included studies and subjected to descriptive statistical analysis. Additional qualitative data relating to patient tolerance were also extracted to inform discussion.
Results:
Ten studies were identified for inclusion. The data identified that OF masks can reproduce patient set-up with an accuracy of <2 mm and <1° and can restrict movement to <1 mm and 0·4°, while maskless SGRT can achieve accuracy to within 0·05 mm and 0·1°.
Conclusion:
This review indicates comparable reduction of intrafractional motion between conventional masks, Open-Face masks and maskless SGRT techniques. More research is needed into the impact of maskless SGRT techniques on translational and rotational motions compared to traditional masks.
Adaptive radiotherapy (ART) is commonly used to mitigate effects of anatomical change during head and neck (H&N) radiotherapy. The process of identifying patients for ART can be subjective and resource-intensive. This feasibility project aims to design and validate a pipeline to automate the process and use it to assess the current clinical pathway for H&N treatments.
Methods:
The pipeline analysed patients’ on-set cone-beam CT (CBCT) scans to identify inter-fractional anatomical changes. CBCTs were converted into synthetic CTs, contours were automatically generated, and the original plan was recomputed. Each synthetic CT was evaluated against a set of dosimetric goals, with failed goals causing an ART recommendation.
To validate pipeline performance, a ‘gold standard’ was synthesised by recomputing patients’ original plans on a rescan-CT acquired during treatment and identifying failed clinical goals. The pipeline sensitivity and specificity compared to this ‘gold standard’ were calculated for 12 ART patients. The pipeline was then run on a cohort of 12 ART and 14 non-ART patients, and its sensitivity and specificity were instead calculated against the clinical decision made.
Results:
The pipeline showed good agreement with the synthesised ‘gold standard’ with an optimum sensitivity of 0·83 and specificity of 0·67. When run over a cohort containing both ART and non-ART patients and assessed against the subjective clinical decision made, the pipeline showed no predictive power (sensitivity: 0·58, specificity: 0·47).
Conclusions:
Good agreement with the ‘gold standard’ gives confidence in pipeline performance and disagreement with clinical decisions implies implementation could help standardise the current clinical pathway.
Spinal CSF leak can cause disabling headaches and neurological symptoms. Lack of awareness, diagnostic delay and treatment inconsistencies affect the quality of CSF leak care globally. This is the first study aiming to identify and assess these challenges in Canada.
Methods:
A cross-sectional online survey of Canadian patients with spinal CSF leak was designed in collaboration with Spinal CSF Leak Canada, including questions on demographics, headache condition, investigations, treatments, quality of life, financial consequences and out-of-country care.
Results:
The survey captured 103 respondents with confirmed spinal CSF leak diagnosis, of whom 56% were still suffering. The majority were female (80%), most being highly educated, with a mean age of 41.8 (SD: 10.37) years at the time of diagnosis. Inconsistencies in care resulted in variable durations for obtaining diagnosis and treatment. The majority of respondents (88%) had seen multiple physicians, and only 50% had seen a CSF leak specialist. Invasive imaging was not performed in 43%. CSF leak relapse after initial successful treatment occurred frequently (43%). The incidence of rebound intracranial hypertension was high (52.5%), and the treatment was difficult to access (77%). Out-of-country care was common (28%), and the impact on financial health was omnipresent (81.5%).
Conclusion:
The survey demonstrates significant gaps in spinal CSF leak care in Canada, similar to global observations. Lack of awareness and access, delayed care, and inconsistencies in investigations and management are common. Spinal CSF leak significantly impacts patients’ physical, mental and financial well-being. Increased awareness, referral pathways and standardized treatment algorithms are key factors in optimizing patient care in Canada.
Management of paediatric non-tuberculous mycobacteria cervicofacial lymphadenitis (NTMCL) is variable without standard practice in the U.K. We undertook a survey to evaluate current U.K. practice in tertiary paediatric units.
Methods
A survey of twenty-one tertiary paediatric centres to evaluate NTMCL management.
Results
We received over 90% response rate. Only 21% have local guidelines, with the majority believing that written national guidance would improve management. Surgery is the most common management choice (69%), with a more limited procedure if skin is involved and/or in proximity to important structures. Observation alone is favoured (32%) when there is no skin involvement but in close proximity to the facial nerve. When treated medically, rifampicin and clarithromycin for 3 months was the commonest choice. Over 25% of respondents state their management has changed following previous adverse outcomes within their own practice or department.
Conclusion
This survey consolidates the current management of NTMCL in U.K. tertiary paediatric units.
Head and neck (HN) radiotherapy (RT) is complex, involving multiple target and organ at risk (OAR) structures delineated by the radiation oncologist. Site-agnostic peer review after RT plan completion is often inadequate for thorough review of these structures. In-depth review of RT contours is critical to maintain high-quality RT and optimal patient outcomes.
Materials and Methods:
In August 2020, the HN RT Quality Assurance Conference, a weekly teleconference that included at least one radiation oncology HN specialist, was activated at our institution. Targets and OARs were reviewed in detail prior to RT plan creation. A parallel implementation study recorded patient factors and outcomes of these reviews. A major change was any modification to the high-dose planning target volume (PTV) or the prescription dose/fractionation; a minor change was modification to the intermediate-dose PTV, low-dose PTV, or any OAR. We analysed the results of consecutive RT contour review in the first 20 months since its initiation.
Results:
A total of 208 patients treated by 8 providers were reviewed: 86·5% from the primary tertiary care hospital and 13·5% from regional practices. A major change was recommended in 14·4% and implemented in 25 of 30 cases (83·3%). A minor change was recommended in 17·3% and implemented in 32 of 36 cases (88·9%). A survey of participants found that all (n = 11) strongly agreed or agreed that the conference was useful.
Conclusion:
Dedicated review of RT targets/OARs with a HN subspecialist is associated with substantial rates of suggested and implemented modifications to the contours.
The intent of the review was to identify different methodological approaches used to calculate the planning target volume (PTV) margin for head and neck patients treated with volumetric arc therapy (VMAT), and whether the necessary factors to calculate the margin size with the selected formula were used.
Materials and Methods:
A comprehensive, systematic search of related studies was done using the Hydi search engine and different databases: MEDLINE, PubMed, CINAHL, ProQuest (Nursing and Allied Health), Scopus, ScienceDirect and tipsRO. The literature search included studies published between January 2007 and December 2020. Eligibility screening was performed by two reviewers.
Results:
A total of seven studies were found. All the reviewed studies used the Van Herk formula to measure the PTV margin. None of the studies incorporated the systematic errors of target volume delineation in the PTV equation. Inter-fraction translational errors were assessed in all the studies, whilst intra-fraction errors were only included in the margin equation for two studies. The studies showed great heterogeneity in the key characteristics, aims and methods.
Findings:
Since systemic errors from target volume delineation were not considered and not all studies assess intra-fraction errors, PTV margins may be underestimated. The recommendations are that studies need to determine the effect of target volume variance on PTV margins. It is also recommended to compare PTV margin results using various formulas.
The aim was to evaluate the feasibility of an intraoral stent (10 and 20 mm thickness) in radiotherapy of tongue cancer, and to measure the reduction in acute mucositis in the palate.
Materials and method:
There were six patients in the intervention group, and seven patients in the control group. Target coverage was measured by the minimum dose covering 98% of the clinical target volume (CTV). Data were collected from the planning CT and daily cone-beam computer tomography (CBCT).
Results:
The 10 and 20 mm stent yielded a mean distance of 26 and 36 mm, respectively, between the tongue and the hard palate. We found comparable dose coverage of the CTV in the treatment plan, and on the CBCT. The stent reduced mean dose to the hard palate by 61.0% (p = 0.002). Dose to the soft palate was not reduced (p = 0.18). Average Common Terminology Criteria for Adverse Events (CTCAE) mucositis scores of the hard palate were 0 and 0.8 in the intervention and control group, respectively. The mucositis scores of the soft palate were 1.2 and 1.8.
Findings:
Use of an intraoral stent substantially reduced the dose to the hard palate. CTV coverage was maintained. We did not find any significant reduction in visually scored radiation-induced mucositis.
Incidence rates of Human Papillomavirus (HPV) positive head and neck cancer (HNC) are increasing. The aim of this study was to explore the availability of information and the knowledge and confidence of healthcare professionals (HCPs) involved in the cancer pathway to provide information on HPV to patients.
Materials and method:
An online questionnaire was designed using closed and open questions to investigate the availability of patient information for patients diagnosed with HPV-associated HNC; health professional’s knowledge of the information available; and their confidence in discussing the topic. The questionnaire was advertised to health professionals through the British Association of Head and Neck Oncologists website. Twenty-five health professionals from six professions across five UK Cancer Alliances completed the questionnaire between November and December 2018.
Results:
Nearly half of the participants did not know whether patients were routinely provided with information on HPV following an HPV-positive (HPV+) cancer diagnosis; 52% indicated that specific information was available within their hospital trust, however, there were inconsistencies with participants’ knowledge of the content of the information leaflets currently available. On a scale of 1–100, the mean confidence in providing HPV-related information was 58.
Findings:
Inconsistencies appear to exist with respect to information about HPV available to patients presenting with HNC. A need has been identified for the education of health professionals involved in the patient care to support information provision.
The aim of this study was to evaluate planning target volume (PTV) margins for two different locations using an electronic portal imaging device (EPID) to ensure that the correct radiation dose is delivered to the tumour when using intensity-modulated radiation therapy (IMRT).
Materials and methods:
Setup data were collected from 40 patients treated with IMRT for head and neck cancer (HN) (20 patients) and prostate cancer (20 patients). Setup errors from 720 registration images were analysed to evaluate systematic and random errors. Thereafter, optimal PTV margins were calculated based on International Commission on Radiation Units and Measurements 62 (ICRU), Stroom and Parker formulas compared with the Van Herk’s recipe.
Results:
To calculate the margins around the PTV, several different formulas have been used. Setup margins ranged between 2–4·3, 2·2–4·6 and 2·1–4·7 mm in X, Y and Z directions, respectively, for HN cases. Similarly, for the prostate site, setup margins ranged between 3·7–8·3, 3·2–6·8 and 3·3–8·2 mm in X, Y and Z directions.
Conclusion:
To ensure better coverage of target volume, we adopted a PTV margin of 5 mm for HN PTVs and 10 mm for prostate PTVs in our department.
In Scotland, patients with suspected head and neck cancer are referred on the basis of the Scottish Referral Guidelines for Suspected Cancer, rather than the National Institute for Health and Care Excellence guidelines. A chest X-ray should be requested by the general practitioner at the same time as referral for persistent hoarseness. The evidence for this is level 4.
Methods:
This audit identified adherence to this recommendation and X-ray results. All ‘urgent suspicion of cancer’ referrals to the ENT department in the National Health Service Greater Glasgow and Clyde for 2015–2016 were audited.
Results:
Persistent hoarseness for more than 3 weeks instigated referral in 318 patients (15.7 per cent). Chest X-ray was performed in 120 patients (38 per cent), which showed: no abnormality in 116 (96.7 per cent), features of infection in 2 (1.7 per cent) and something else in 2 patients (1.7 per cent). No chest X-ray altered the management of a patient.
Conclusion:
Performance of chest X-ray does not alter management and its removal from the Scottish Referral Guidelines for Suspected Cancer is recommended.
Ultrasound-guided wire localisation is a technique that is well established in breast surgery, but is not well described in head and neck practice. It can be used to locate impalpable lesions for surgical removal, without the need for more extensive surgery.
Method:
This paper describes a case where the technique was used to assist in the removal of an impalpable but radiologically abnormal lymph node.
Conclusion:
The technique offers a safe and effective method for removing lesions within the head and neck.
This study aimed to compare the diagnostic effectiveness of narrow band imaging and autofluorescence imaging for malignant laryngopharyngeal tumours.
Methods:
Between May 2010 and October 2010, 50 consecutive patients with suspected laryngopharyngeal tumour underwent endoscopic laryngopharynx examination. The morphological characteristics of laryngopharyngeal lesions were analysed using high performance endoscopic systems equipped with narrow band imaging and autofluorescence imaging modes. The diagnostic effectiveness of white light image, narrow band imaging and autofluorescence imaging endoscopy for benign and malignant laryngopharyngeal lesions was evaluated.
Results:
Under narrow band imaging endoscopy, the superficial microvessels of squamous cell carcinomas appeared as dark brown spots or twisted cords. Under autofluorescence imaging endoscopy, malignant lesions appeared as bright purple. The sensitivity of malignant lesion diagnosis was not significantly different between narrow band imaging and autofluorescence imaging modes, but was better than for white light image endoscopy (χ2 = 12.676, p = 0.002). The diagnostic specificity was significantly better in narrow band imaging mode than in both autofluorescence imaging and white light imaging mode (χ2 = 8.333, p = 0.016).
Conclusion:
Narrow band imaging endoscopy is the best option for the diagnosis and differential diagnosis of laryngopharyngeal tumours.
To develop a regime of care for patients with head and neck cancers undergoing intensity-modulated radiotherapy (IMRT), with the support of a health advisor (HA) and temporary access to the mouth care product Caphosol™.
Materials and methods
A HA was temporarily employed to assess, monitor and refer patients as appropriate and ensure patients received and utilised supplies of Caphosol™. A retrospective audit was undertaken to provide a gap analysis of current service. The data were used to develop a pro forma for documenting assessments and monitoring lifestyle factors for IMRT patients. Assessments referrals and compliance, plus hospital admissions owing to treatment-related issues, were documented during the baseline audit and the temporary HA service and provision of Caphosol™.
Results
The presence of a HA facilitated 100% compliance with appropriate assessments, referrals and adherence to treatment. The data suggests that the additional provision of Caphosol™ may have reduced levels of mucositis and associated pain.
Conclusion
It is recommended that a HA role be established within radiotherapy departments to facilitate lifestyle assessments, referrals and compliance with positive behaviour changes (e.g., stopping smoking). The use of Caphosol™ as a routine part of mouth care regime for IMRT patients also warrants further investigation.
This study evaluated the impact of a daily and weekly image-guided radiotherapy protocols in reducing setup errors and setting of appropriate margins in head and neck cancer patients.
Materials and methods
Interfraction and systematic shifts for the hypothetical day 1–3 plus weekly imaging were extrapolated from daily imaging data from 31 patients (964 cone beam computed tomography (CBCT) scans). In addition, residual setup errors were calculated by taking the average shifts in each direction for each patient based on the first three shifts and were presumed to represent systematic setup error. The clinical target volume (CTV) to planning target volume (PTV) margins were calculated using van Herk formula and analysed for each protocol.
Results
The mean interfraction shifts for daily imaging were 0·8, 0·3 and 0·5 mm in the S-I (superior-inferior), L-R (left-right) and A-P (anterior-posterior) direction, respectively. On the other hand the mean shifts for day 1–3 plus weekly imaging were 0·9, 1·8 and 0·5 mm in the S-I, L-R and A-P direction, respectively. The mean day 1–3 residual shifts were 1·5, 2·1 and 0·7 mm in the S-I, L-R and A-P direction, respectively. No significant difference was found in the mean setup error for the daily and hypothetical day 1–3 plus weekly protocol. However, the calculated CTV to PTV margins for the daily interfraction imaging data were 1·6, 3·8 and 1·4 mm in the S-I, L-R and A-P directions, respectively. Hypothetical day 1–3 plus weekly resulted in CTV–PTV margins of 5, 4·2 and 5 mm in the S-I, L-R and A-P direction.
Conclusions
The results of this study show that a daily CBCT protocol reduces setup errors and allows setup margin reduction in head and neck radiotherapy compared to a weekly imaging protocol.
The management of extrapulmonary small cell carcinoma has not been standardised to date. This study reviewed the clinical course, management and survival outcomes of patients with extrapulmonary small cell carcinoma in the head and neck region.
Methods:
Nine patients with extrapulmonary small cell carcinoma in the head and neck were included in this study.
Results:
Five patients received radical surgery followed by adjuvant chemotherapy or radiotherapy or both. Two other patients were treated with chemotherapy consisting of CPT11 plus cisplatin or CPT11 plus cisplatin plus VP-16 three times. Two other patients received chemoradiotherapy consisting of S-1 or CPT11 plus cisplatin. The median overall survival was 14.5 months, with a three-year survival rate of 23.7 per cent.
Conclusions:
The prognosis of extrapulmonary small cell carcinoma is generally poor. Further prospective multicentre studies are required for better understanding of disease entities and response to treatment modalities.
Treatment for metastatic cutaneous head and neck squamous cell carcinoma is usually multimodal and associated with morbidity. This study aimed to evaluate the impact of treatment on patients' quality of life.
Method:
Cross-sectional survey of 42 patients (35 men, 7 women) at least 6 months after metastatic cutaneous head and neck squamous cell carcinoma treatment, using two standardised quality of life questionnaires: the Functional Assessment of Cancer Therapy – Head and Neck questionnaire and the Facial Disability Index, with statistical analysis to identify potential predictors of outcome.
Results:
Female gender correlated with significantly lower Facial Disability Index physical function scores (p = 0.017). Alcohol consumption correlated with significantly better scores for Functional Assessment social well-being (p = 0.016), general total score (p = 0.041) and overall total score (p = 0.033), and for Facial Disability Index physical function (p = 0.034). Marital status, education, employment, chemotherapy, time from last treatment, parotidectomy and facial nerve sacrifice did not affect quality of life. The commonest patient complaints were dry mouth (76 per cent), altered voice quality and strength (55 per cent), and physical appearance (45 per cent).
Conclusion:
Female gender predicts worse quality of life, while alcohol consumption (versus none) predicted for better quality of life.
This study aimed to compare recurrence and survival in patients undergoing either selective neck dissection or modified radical neck dissection to treat metastatic cutaneous head and neck squamous cell carcinoma to the cervical lymph nodes (levels I–V) only.
Methods:
Twenty-eight year, retrospective analysis of a prospectively maintained database from a tertiary referral hospital, with a minimum follow up of two years.
Results:
There were 122 eligible patients: 96 males (79 per cent) and 26 (21 per cent) females (median age, 66 years). Sixty-six patients (54 per cent) underwent selective neck dissection and 56 (46 per cent) modified radical neck dissection. The former patients had a lower rate of regional recurrence compared with the latter (17 vs 23 per cent, respectively). There was no significant difference in five-year overall survival (61 vs 57 per cent, respectively) or five-year disease-free survival (74 vs 60 per cent, respectively), comparing the two groups. Overall survival and disease-free survival were significantly improved by the addition of adjuvant radiotherapy.
Conclusion:
We found no difference in outcome in patients undergoing selective versus modified radical neck dissection. Adjuvant radiotherapy significantly improved outcome.