Is there scope to do better? Clinical communication with adolescents and young adults with cancer-A scoping review.

INTRODUCTION: How to communicate effectively with adolescent and young adults with cancer (AYACs) is a research priority. In a UK-wide survey of young people with cancer's research priorities, communication was a striking cross-cutting theme. It is increasingly recognised that AYACs have experiences and communication needs that differ significantly from those of younger children and older adults. The purpose of this review is to explore the features of effective clinical communication with AYACs. METHODS: A literature search was undertaken to identify and map the available evidence using a broad scope to get an overview of the pertinent literature, identify knowledge gaps and clarify concepts. The searches yielded 5825 records, generating 4040 unique articles. These were screened and 71 full articles were read by four researchers with disagreements resolved by discussion leaving 29 included articles. Narrative synthesis was undertaken in relation to each of the research questions. RESULTS: Three key themes were identified: being an adolescent/young adult, supporters, and healthcare professionals (HCPs). AYACs need to feel that HCPs understand their unique perspective. They want to be involved, this changes over time and in different contexts. Supporters are a central tenet, are most often parents and undertake several roles which are not always universally supportive. HCPs enable involvement of AYACs, and this needs to be actively promoted. AYACs preference for their level of involvement requires continual assessment. The three themes are interlinked and exist within the wider scope of the triadic encounter and cancer experience. CONCLUSION: Supporters, most often parents were a key feature across the data and were seemingly paradoxical in nature. Triadic communication, the presence of a third person, is a central tenet of communication with AYACs and we propose a conceptual model to represent the nuances, components, and facets of this complex communication.

Adolescent and Young Adult Cancer Placements: Developing Complex Clinical Communication Skills of Medical Students.

Purpose: Following implementation of a student selected module in adolescent and young adult cancer care for medical students, we sought to explore their experiences of the specialty. Methods: We undertook a focus group of five medical students all in their fourth to sixth year of study. Transcripts were transcribed verbatim and analyzed thematically. Results: We identified six key themes repeatedly elicited during the focus group; these were specialized and holistic care, patient's perspective, connectedness and professional boundaries, triadic communication, emotional impacts, and professional development and support. Conclusion: Early exposure to this specialty is positive for students and the model could be replicated elsewhere.

"A Different Person Entirely": Adolescent and Young Adults' Experiences Returning to Education after Cancer Treatment.

Purpose: Adolescent and young adults (AYAs) establish their independent, adult identities as part of their psychosocial development, a process that is largely informed by educational experiences. Not only is a cancer diagnosis disruptive to this process but also AYA cancer survivors (AYACs) face barriers as they attempt to reintegrate into educational systems. This study explores the experiences of AYACs as they return to education, to identify these obstacles and the implications for care teams. Methods: In-depth semistructured interviews were conducted with AYACs (n = 8), 16-19 years of age at diagnosis and 18-27 years of age at time of interview. Interviews were transcribed verbatim and analyzed using the principles of Giorgi's phenomenological analysis. Results: Four major themes were identified: AYACs suffer from debilitating late effects (theme 1) post-treatment as they adjust to a loss of normality and other fundamental losses (theme 2) associated with a cancer diagnosis, such as irrecoverable future plans. The educational systems (theme 3) to which they return can be both accommodating, capable of making allowances, and uncompromising, unable to adapt to AYAC survivors' needs. Appropriate mechanisms to facilitate resilience (theme 4) among AYACs are vital for successful return to education. Conclusion: This study supports previous findings that late effects and systemic barriers can hinder return to education, but further research focused on this age group is required. We believe that treating clinicians and specialist services can facilitate the return of AYACs to education by providing warning and comprehensive information about late effects, as early as possible before treatment completion, as well as effective information sharing with educational institutions.

How Can Health Care Professionals Communicate Effectively with Adolescent and Young Adults Who Have Completed Cancer Treatment? A Systematic Review.

Communication with teenage and young adult or adolescent and young adult (AYA) patients with cancer is critically important and not consistently taught in health care education. We have developed a local training for medical students to experience and join AYA cancer consultations to improve clinicians' abilities in the future. We have undertaken a systematic review of the literature, to provide a comprehensive overview of studies evaluating communication in this specific patient group, to guide clinical practice and future research. We searched MEDLINE, EMBASE, PsycINFO, Web of Science, and CINAHL databases for literature containing data relating to communication in AYAs previously treated for malignant disease. To ensure as comprehensive a review as possible, the researchers defined the AYA age range using the extremes used globally: 13-39 years. Two thousand eight hundred and thirty-seven articles were identified, which were reduced to 1517 after duplicates were removed. After handsearching references and citation tracking, 1556 abstracts were screened of which 40 full-text articles were assessed for eligibility. Only 12 met all inclusion criteria and were included in the final analysis. Young people want to be heard and have trust in their health care professionals (HCPs). They want to be involved in decisions about their care, and HCPs must establish who else the AYA wants to be involved in the decision-making process. AYAs want information to be given in an amount they can control and at a time preferred by them. Further education of communication skills, especially triadic communication, is essential for professionals involved in AYA care.

Cardiotoxicity with trabectedin in the treatment of advanced soft tissue sarcoma.

Trabectedin is used routinely in the palliative management of patients with advanced soft tissue sarcoma. It is not generally considered to be cardiotoxic, and there is no specific caution for its use in patients with a history of cardiac disease or risk factors. Here, we report six cases from a single academic centre where life-threatening cardiotoxicity occurred acutely during treatment with trabectedin. These patients had a median age of 72.5 years (range: 68-81) at presentation with cardiotoxicity, significantly higher than the median ages of patients treated with trabectedin in clinical trials. Cardiotoxicity occurred between cycle 1, day 10, and cycle 5, day 5 of treatment (with three events occurring during cycle 2, and one during cycle 3). Two patients had a previous cardiac history and three patients had other relevant cardiac risk factors. Five patients had been treated previously with anthracyclines. Two patients developed acute pulmonary oedema, two developed fast atrial fibrillation, one developed an ST-elevation myocardial infarction and one suffered a fatal cardiac arrest. Two had unequivocal evidence of myocardial ischaemia, and two events were acutely fatal. Trabectedin was immediately discontinued in all cases and, for the four nonfatal events, there were no recurrences of the cardiac events. As no other definitive precipitants were identified, we consider these events to be related to trabectedin toxicity. We therefore urge caution with the use of trabectedin in elderly patients, and those with a previous cardiac history, abnormal cardiac function or significant cardiac risk factors including pericardiac lesions (present in two cases reported here).

Presentation and management of pulmonary artery sarcoma.

BACKGROUND: Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy that leads to heart failure and death without treatment. Here we reviewed the presentation and management of patients treated at a national centre for pulmonary endarterectomy (PEA) and its associated hospital in Cambridge, UK. METHODS: Details of PAS patients treated at Papworth and Addenbrooke's Hospitals between 2000 and 2014 were reviewed. RESULTS: Twenty patients were diagnosed with PAS (11 males, 9 females), with a median age of presentation of 57 years (range 27-77). Presenting symptoms include dyspnoea (20), chest pain/tightness (7), oedema (5), constitutional symptoms (5), cough (3) and haemoptysis (3). Twelve patients were in group III/IV of the NYHA functional classification of symptoms. Initial CT scans were suggestive of thromboembolism in seven patients. Histological findings were of intimal sarcoma (13) and high grade sarcoma NOS (6). Median overall survival (OS) was 17 months. Fourteen patients underwent PEA to relieve vascular obstruction, while six had inoperable and/or metastatic disease. There were three peri-operative deaths. Although there was no difference in median OS between patients who had PEA and those who did not (20 vs 17 months, P = 0.2488), surgery provided significant symptomatic improvement and some with long-term survival. Five patients received post-surgical chemotherapy (anthracycline +/- ifosfamide), and after completion four also had radiotherapy. Patients who received post-operative chemo- and radio-therapy showed a trend towards better survival compared to those who had surgery alone (24 vs 8 months, P = 0.3417). For palliative chemotherapy, partial responses were observed with the VID regimen and pegylated liposomal doxorubicin. Stable disease was achieved in a patient with intimal sarcoma with rhabdomyosarcomatous differentiation on third-line cisplatin and topotecan. The longest surviving patient (102 months) has had PEA, adjuvant epirubicin and radiotherapy. She developed lung metastases 7 years later, which were treated with radiofrequency ablation. CONCLUSIONS: PAS often presents with symptoms mimicking pulmonary hypertension, heart failure or thromboembolic disease. PEA provides good symptomatic relief and in some cases, offers a chance of long-term survival. Although outcome appears to be better when PEA is combined with post-operative chemo- and radio-therapy, further studies are warranted.

Trabectedin for advanced soft tissue sarcomas: a single institution experience.

BACKGROUND: We retrospectively analyzed data from patients who had been treated with trabectedin at our institution between April 2009 and August 2011. PATIENTS & METHODS: Data from 25 patients with recurrent soft tissue sarcoma (leiomyosarcoma: n = 8; liposarcoma: n = 5) were used to assess the efficacy and safety of trabectedin 1.5 mg/m(2) given every 3 weeks. RESULTS: Most patients (n = 14) had been heavily pretreated with ≥ 2 previous chemotherapy lines. Eight (32%) patients achieved a partial response according to dimensional and tumor density changes, and seven (28%) patients had stable disease for ≥ 3 months (clinical benefit rate = 60%; n = 15). Median progression-free survival was 6.4 months and overall survival 19.3 months. Common adverse events were fatigue, nausea, anemia and transient transaminase increases. CONCLUSION: Treatment with trabectedin is effective and well tolerated in heavily pretreated soft tissue sarcoma patients. Tapering dexamethasone courses and switching trabectedin administration to an every 4 weeks schedule effectively dealt with persistent fatigue without compromising effectiveness.

Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature.

BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare but frequently fatal sarcoma, and many of its characteristics still require further clarification. METHODS: We retrospectively analysed 41 patients treated at or referred to two regional referral centres in the UK between 1991 and 2012. A review of the current literature was also performed. RESULTS: The median age of presentation was 27 years (range 16 to 45 years), with a male-to-female ratio of 3:1. Ninety percent of patients had disease in the abdomen. The median size of the presenting tumour was 13 cm (range 3.5 to 23 cm), and 80% had metastatic disease at diagnosis, mainly in the liver (33%) and lungs (21%). Time-to-progression (TTP) was 3.9, 2.3 and 1.1 months after first-, second- and third-line chemotherapy, respectively. First-line treatment with VIDE chemotherapy appeared to confer the longest TTP (median 14.6 months). Ifosfamide and doxorubicin resulted in TTP of >3.8 months when used in any-line setting. Eleven patients received targeted agents as part of a clinical trial. After a median follow-up of 14 months, the overall median survival (MS) was 16 months. There was no difference in MS with regards to age, gender, or size of the presenting tumour. Patients with extra-abdominal disease survived longer compared to those with tumours in the abdomen (all still alive vs MS of 15 months; P = 0.0246). Patients with non-metastatic intra-abdominal disease who underwent surgery had an MS of 47 months (16 months for those who did not have surgery; P = 0.0235). Radiotherapy for locoregional control in patients with metastatic intra-abdominal DSRCT was associated with longer survival (MS of 47 vs 14 months; P = 0.0147). CONCLUSIONS: DSRCT is a rare but often fatal disease that mainly affects younger male patients. Those with intra-abdominal DSRCT have a poorer prognosis, although surgical resection for localised disease and radiotherapy in the metastatic setting are associated with improved survival. A patient's age, gender and size of presenting tumour do not have prognostic significance.

Management of malignant ovarian germ cell tumors.

UNLABELLED: Malignant ovarian germ cell tumors are rare, highly curable cancers of young women. The majority of patients can be cured with either fertility-preserving surgery alone or a combination of surgery and chemotherapy. Relapses occur in 10% to 20% of patients, and the significant proportion of them can be salvaged with chemotherapy. There is no evidence that treatment for malignant ovarian germ cell tumors will adversely affect menstrual or reproductive functions, increase future pregnancy loss, or increase the risk of congenital malformations of the fetus. Late effects, such as secondary leukemia, from chemotherapy are reported but rare. TARGET AUDIENCE: Obstetricians & Gynecologists and Family Physicians. LEARNING OBJECTIVE: After completing this CME activity, physicians should be better able to diagnose ovarian germ cell tumors, outline management of malignant ovarian germ cell tumors, and understand the impact of treatment on fertility and late effects.

Management of solid tumours in organ-transplant recipients.

Malignancy is a well-recognised complication of transplantation and can occur de novo, as a recurrence of a pre-existing malignancy, or from transmission of malignancy from the donor. Common de-novo malignancies are those of the skin and the lymphoreticular system. Various solid-organ cancers have also been reported in transplant recipients and each poses a unique management challenge in view of the unusual setting. We review solid-organ cancers in transplant recipients and their management, including surveillance and prevention.