Langerhans cell sarcoma of the head and neck.

Head and neck Langerhans cell sarcoma (HNLCS) is a rare malignant tumor carrying a poor prognosis. The aim of this work was to perform a systematic review of HNLCS cases, examine outcomes, and develop an evidence-based management algorithm. We performed a systematic literature search yielding 16 studies with 17 cases of HNLCS; 33 studies with 55 Non-HNLCS were used as a comparison. Mean disease-specific survival was 20.5 months (SE ± 5.1) for HNLCS versus 26.2 months (SE±4.2) for non-HNLCS. There was no significant difference in disease-specific (p = 0.768) or disease-free survival (p = 0.880) between the two cohorts. There was a significant difference in both disease-specific (p = 0.044) and disease-free survival (p = 0.001) between local, locoregional and disseminated disease favoring more limited disease. HNLCS appears to present later, with more disseminated disease. Surgery remains the mainstay of treatment of local disease, however clear margins do not guarantee clearance.

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.

De-escalation treatment protocols for human papillomavirus-associated oropharyngeal squamous cell carcinoma: a systematic review and meta-analysis of current clinical trials.

Iatrogenic complications associated with current treatment protocols for oropharyngeal squamous cell carcinoma are noted to cause high rates of acute and chronic morbidity. The aims of this study are to provide an overview of the current de-escalation trials for human papillomavirus positive (HPV+) oropharyngeal carcinoma and to evaluate the evidence supporting improved response to treatment of patients within this viral cohort. This study reviewed all completed or in progress randomised controlled trials (RCTs) assessing clinical interventions for human papillomavirus-associated locally advanced oropharyngeal squamous cell carcinoma. We utilised a validated 'risk of bias' tool to assess study quality. We identified nine RCTs that met the full inclusion criteria for this review (all of which are currently on-going and will report from 2015 onwards). Five RCTs performed a post hoc analysis by HPV status, which allowed meta-analysis of 1130 patients. The data reveal a significant difference in overall survival (hazard ratio (HR) 0.49 [95% confidence interval (CI) 0.35-0.69]), loco-regional failure (HR 0.43 [95% CI 0.17-1.11]) and disease specific survival (0.41 [95% 0.3-0.56]) in favour of the HPV+ category. In considering de-escalation treatment protocols, nine studies are currently ongoing. Our meta-analysis provides strong evidence for an improved prognosis in the viral associated cohort when treated by platinum based chemotherapy in combination with radiotherapy or primary radiotherapy. So far, one trial (with moderate to high risk of bias) suggests a reduced survival outcome for the HPV+ population when using the epidermal growth factor receptor (EGFR) inhibitor cetuximab.