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Medical Oncology.BACKGROUND The Surveillance of Rare Cancers in Europe (RARECARE) project proposed a definition and a list of rare cancers. The Joint Action on Rare Cancers (JARC), launched by the European Union and involving 18 member states and 34 partners, promoted a wide consensus effort to review the list. PATIENTS AND METHODS A group of experts was set up, including scientific societies, member state representatives of JARC, representatives of the European Reference Networks dedicated to rare cancers and rare cancer patient advocates. The definition and the list of rare clinical entities, based on the incidence data provided by two European projects (RARECARE and RARECAREnet), were rediscussed through a consensus meeting of the expert panel. RESULTS By consensus, it was reiterated that the best criterion for a definition of rare cancers is incidence, rather than prevalence. By consensus, the experts slightly modified the composition of the tiers of rare cancers, according to the definition based on an incidence threshold less then 6/100 000/year, and grouped all rare cancers within 12 families of rare cancers. Even when defined conservatively this way, rare cancers are not rare collectively, since they correspond to 10%-20% of all cancer cases. CONCLUSIONS The list of rare cancers reviewed by JARC should be viewed as a tool in the fight against rare cancers and rare diseases. It may help to appreciate that rare cancers are cancers and rare diseases at the same time, combining issues and difficulties of both. We hope that refinements to the list and a wider understanding of its implications may contribute to increase awareness of problems posed by rare cancers and to improve quality of care in a large group of patients with cancer, who may be discriminated against just because of the low frequency of their diseases. © Author (s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. Published by BMJ on behalf of the European Society for Medical Oncology.Bone health impairment is a frequent detrimental consequence of the high bone tropism of prostate cancer (PCa) cells. It is further worsened by administration of androgen-deprivation therapy (ADT), the current standard of care in the management of advanced PCa, through a rapid and dramatic increase in bone turnover and body mass changes. As a result, patients may experience substantial pain and poor quality of life (QoL) and have an increased risk of death. Notwithstanding the importance of this issue, however, bone health preservation is not yet a widespread clinical goal in daily practice.To address this urgent unmet need, following a thorough discussion of available data and sharing of their clinical practice experience, a panel of Italian experts in the field of bone health and metabolism formulated a number of practical advices for optimising the monitoring and treatment of bone health in men undergoing ADT during all phases of the disease. The rationale behind the venture was to raise awareness on the importance of bone preservation in this complex setting, while providing an instrument to support physicians and facilitate the management of bone health.Current evidence regarding the effects on bone health of ADT, of novel hormone therapies (which improve progression delay, pain control and QoL while consistently carrying the risk of non-pathological fractures in both non-metastatic and metastatic PCa) and of bone turnover inhibitors (whose use is frequently suboptimal) is reviewed. Finally, the expert opinion to optimise bone health preservation is given. © Author (s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. Clofarabine RNA Synthesis inhibitor Published by BMJ on behalf of the European Society for Medical Oncology.OBJECTIVE Patients in the last year of life experience medical emergencies which may lead to an emergency attendance by ambulance clinicians and some patients having a transfer to hospital even when this is unwanted by patients, carers or professionals. Here we report the patient characteristics and outcomes of a 24-hour hospice nursing telephone advice service to support an ambulance service. METHOD An evaluation of the outcomes of ambulance calls to a nursing telephone advice service for people living in northwest London, UK, attended at home during a 6-month period by the London Ambulance Service, whose clinicians then sought advice from the hospice's 24 hours' telephone line. RESULTS Forty-five attendances of 44 acutely ill people with palliative care needs resulted in a telephone call. Thirteen patients (30%) were male and the median age was over 80 years. Thirty-two attendances (71%) were managed without a transfer to hospital, with telephone advice from the hospice and in some cases arrangements for another clinician to visit. Seven attendances (16%) resulted in a transfer to hospital, of which at least five led to an admission. Six attendances (13%) resulted in a notification of the patient's death. CONCLUSIONS This preliminary study shows the feasibility, outcomes and acceptability of telephone advice to support ambulance clinicians attending patients with palliative care needs. The service was associated with low rates of subsequent transfer to hospital. Further controlled research is needed to assess the clinical and cost-effectiveness of the service. © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.BACKGROUND Few studies have investigated the content of interventions provided in early specialised palliative care (SPC). OBJECTIVES To characterise the content of interventions delivered in early SPC in the Danish Palliative Care Trial (DanPaCT), a multicentre trial with six participating sites. METHODS A retrospective qualitative and quantitative study coding all new interventions initiated by the palliative teams and documented in the medical records during the 8-week study period of DanPaCT. Interventions were categorised according to (a) symptom/problem prompting the intervention, (b) type of intervention and (c) professional(s) providing the intervention. RESULTS In total, 145 patients were randomised to the SPC teams. According to the medical records, patients received a median of 3.5 (range 0-22) new interventions in the 8-week intervention-period from the palliative teams. For 24 (18%) of the patients there was no documented interventions in the medical records. The most frequent symptom/problems treated were pain, (100 interventions; 20% of interventions given) and impaired physical function (62; 13% of interventions given). The most frequent type of intervention was pharmacological (232; 42% of interventions given). CONCLUSIONS This is one of the first studies to meticulously investigate the content of interventions documented in the medical records for patients receiving early SPC. Diverse symptoms were treated with many different interventions. However, a relatively low number of interventions were documented. This may explain the lack of effect in DanPaCT but also questions whether all interventions were adequately documented TRIAL REGISTRATION NUMBER NCT01348048. © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.OBJECTIVES Percutaneous cervical cordotomy (PCC) is an interventional ablative procedure in the armamentarium for cancer pain treatment, but there is limited evidence to support its use. This study aimed to assess the effectiveness and safety of PCC. METHODS Analysis was undertaken of the first national (UK) prospective data repository of adult patients with cancer undergoing PCC for pain treatment. The relationship between pain and other outcomes before and after PCC was examined using appropriate statistical methods. RESULTS Data on 159 patients' PCCs (performed from 1 January 2012 to 6 June 2017 in three centres) were assessed median (IQR) age was 66 (58-71) years, 47 (30%) were female. Mesothelioma was the most common primary malignancy (57%). The median (IQR) time from cancer diagnosis to PCC assessment was 13.3 (6.2-23.2) months; PCC to follow-up was 9 (8-25) days; and survival after PCC was 1.3 (0.6-2.8) months. The mean (SD) for 'average pain' using a numerical rating scale was 6 (2) before PCC and 2 (2) at follow-up, and for 'worst pain' 9 (1) and 3 (3), respectively. The median (IQR) reduction in strong opioid dose at follow-up was 50% (34-50). With the exception of 'activity', all health-related quality of life scores (5-level version of EuroQol-5 Dimension) either improved or were stable after PCC. Six patients (4%) had PCC-related adverse events. CONCLUSIONS PCC is an effective treatment for cancer pain; however, findings in this study suggest PCC referrals tended to be late in patients' disease trajectories. Further study into earlier treatment and seeking international consensus on PCC outcomes will further enhance opportunities to improve patient care. © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ.AIM Hernia sacs with pathological evaluation over a 19-year period were analysed with regards to pathological diagnoses, full costing and the impact on patient management. MATERIALS AND METHODS The database of the Department of Pathology were searched over the study period (2001 to 2019 inclusive) for hernia sacs. The total cost of complete pathology examination was calculated on average numbers and rates of pay that existed over the study period. RESULTS A total of 3619 hernia sacs from the abdominal, hiatus/diaphragmatic, inguinal and femoral hernias were retrieved. Of these 3592 cases (99.25%) had sections taken for histological evaluation. A total of 3437 cases representing 95.7% of all hernia sacs did not show any pathological abnormality. If non-neoplastic clinically insignificant lesions seen in hernia sacs is included, then 3552 of 3592 (98.9%) hernia sacs underwent full pathological evaluation for no patient benefit.On average two blocks or tissue sections per case were processed incurring a technical cost of $53 175.00. The total pathologist cost in reporting the 3592 cases was approximately $39 870.00 and rose to $40 410.00 when interpretation of ancillary tests was factored in. $95 328.90 (average $26.90 per specimen with a yearly average total cost of $5 017.31) was spent over the 19-year period in full pathological examination of 3592 hernia sacs. CONCLUSION Given the low return on investment and the difficult to quantify time savings and reallocation, we do not advocate the routine sampling of hernia sacs. Gross examination will suffice in 99% of the cases. Selective cases may be sampled if clinically indicated. © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.AIMS The aim of this study is to investigate the expression profiles of cell cycle related proteins in nasal extranodal NK/T cell lymphoma, nasal type (ENKTCL). METHODS The expression profiles of cell cycle related proteins were assessed with a cell cycle antibody array and validated by immunohistochemistry. Correlations between the expression levels of proteins and clinical outcomes of patients with nasal ENKTCL were evaluated. RESULTS The expression of full length ataxia telangiectasia mutated (ATM) in nasal ENKTCL significantly decreased compared with that in nasal benign lymphoid proliferative disease (NBLPD), but the expression levels of p-ATM, CHK2 and RAD51 significantly increased in nasal ENKTCL compared with that in NBLPD. Kaplan-Meier analysis showed that the expression levels of p-ATM and CHK2 in nasal ENKTCL were inversely related to overall survival (p=0.011 and p=0.025, respectively). CONCLUSION Abnormalities in the ATM pathway may play a crucial role in the oncogenesis and chemoradiotherapy resistance of nasal ENKTCL.
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