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BACKGROUND: Therapeutic options are limited in pediatric CNS malignancies. CheckMate 908 (NCT03130959) is an open-label, sequential-arm, phase 1b/2 study investigating nivolumab (NIVO) and NIVO + ipilimumab (IPI) in pediatric patients with high-grade CNS malignancies. METHODS: Patients (N = 166) in 5 cohorts received NIVO 3 mg/kg every 2 weeks (Q2W) or NIVO 3 mg/kg + IPI 1 mg/kg every 3 weeks (4 doses) followed by NIVO 3 mg/kg Q2W. Primary endpoints included overall survival (OS; newly diagnosed diffuse intrinsic pontine glioma [DIPG]) and progression-free survival (PFS; other recurrent/progressive or relapsed/resistant CNS cohorts). Secondary endpoints included other efficacy metrics and safety. Exploratory endpoints included pharmacokinetics and biomarker analyses. RESULTS: As of January 13, 2021, median OS (80% CI) was 11.7 (10.3-16.5) and 10.8 (9.1-15.8) months with NIVO and NIVO + IPI, respectively, in newly diagnosed DIPG. Median PFS (80% CI) with NIVO and NIVO + IPI was 1.7 (1.4-2.7) and 1.3 (1.2-1.5) months, respectively, in recurrent/progressive high-grade glioma; 1.4 (1.2-1.4) and 2.8 (1.5-4.5) months in relapsed/resistant medulloblastoma; and 1.4 (1.4-2.6) and 4.6 (1.4-5.4) months in relapsed/resistant ependymoma. In patients with other recurrent/progressive CNS tumors, median PFS (95% CI) was 1.2 (1.1-1.3) and 1.6 (1.3-3.5) months, respectively. Grade 3/4 treatment-related adverse-event rates were 14.1% (NIVO) and 27.2% (NIVO + IPI). NIVO and IPI first-dose trough concentrations were lower in youngest and lowest-weight patients. Baseline tumor programmed death ligand 1 expression was not associated with survival. CONCLUSIONS: NIVO ± IPI did not demonstrate clinical benefit relative to historical data. The overall safety profiles were manageable with no new safety signals.
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Neoplasias , Nivolumabe , Humanos , Criança , Nivolumabe/uso terapêutico , Ipilimumab/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias/tratamento farmacológico , BiomarcadoresRESUMO
BACKGROUND: National advisory panels (NAPs) have been established for the care of children and young people (CYP) with cancer in the United Kingdom since 2011, with an increase in panel number in recent years. Their practice has not previously been reviewed; therefore, we sought to evaluate the role, practice and impact of six selected NAPs offering expertise in ependymoma, histiocytosis, leukaemia, neuroblastoma, renal tumours and sarcoma. PROCEDURE: This service evaluation used mixed methodology, including review of NAP documentation, semi-structured interviews with the NAP chairs and an analysis of the cases referred for discussion. RESULTS: Total 1110 referrals were analysed. Results demonstrated the significant scope and amount of work undertaken by the NAPs, largely testament to the commitment of the panel members. Specific roles fulfilled have been highlighted, and NAP recommendations have been shown to influence clinical decision-making and be implemented in the majority of cases. Despite widespread good practice, areas to address have been identified; these include clarity regarding NAP membership, consistency in recommendations, the consideration of holistic information to promote personalised management and the exploration of wider multidisciplinary team roles. CONCLUSIONS: In the context of increasing demand and the escalating number of NAPs, it is timely to consider how service improvement can be facilitated. Best practice guidelines have been formulated as a product of this study, to promote a sustainable and effective model for NAPs. Review and benchmarking national panel performance against these guidelines will drive high standards of care going forward and they should be embedded as standard practice.
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Leucemia , Neuroblastoma , Sarcoma , Criança , Humanos , Adolescente , Reino UnidoRESUMO
BACKGROUND: The MAPK pathway is an emerging target across a number of adult and pediatric tumors. Targeting the downstream effector of MAPK, MEK1, is a proposed strategy to control the growth of MAPK-dependent tumors. OBJECTIVE: iMATRIX-cobi assessed the safety, pharmacokinetics, and anti-tumor activity of cobimetinib, a highly selective MEK inhibitor, in children and young adults with relapsed/refractory solid tumors. PATIENTS AND METHODS: This multicenter Phase I/II study enrolled patients aged 6 months to < 30 years with solid tumors with known/expected MAPK pathway involvement. Patients received cobimetinib tablet or suspension formulation on Days 1-21 of a 28-day cycle. Dose escalation followed a rolling 6 design. The primary endpoint was safety; secondary endpoints were pharmacokinetics and anti-tumor activity. RESULTS: Of 56 enrolled patients (median age 9 years [range 3-29]), 18 received cobimetinib tablets and 38 cobimetinib suspension. Most common diagnoses were low-grade glioma (LGG; n = 32, including n = 12 in the expansion cohort) and plexiform neurofibroma within neurofibromatosis type 1 (n = 12). Six patients (11 %) experienced dose-limiting toxicities (including five ocular toxicity events), which established a pediatric recommended Phase II dose (RP2D) of 0.8 mg/kg tablet and 1.0 mg/kg suspension. Most frequently reported treatment-related adverse events were gastrointestinal and skin disorders. Steady state mean exposure (Cmax, AUC0-24) of cobimetinib at the RP2D (1.0 mg/kg suspension) was ~ 50 % lower than in adults receiving the approved 60 mg/day dose. Overall response rate was 5.4 % (3/56; all partial responses in patients with LGG). CONCLUSIONS: The safety profile of cobimetinib in pediatrics was similar to that reported in adults. Clinical activity was observed in LGG patients with known/suspected MAPK pathway activation. Cobimetinib combination regimens may be required to improve response rates in this pediatric population. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov NCT02639546, registered December 24, 2015.
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Azetidinas , Neoplasias , Piperidinas , Adolescente , Adulto , Azetidinas/efeitos adversos , Azetidinas/uso terapêutico , Criança , Pré-Escolar , Inibidores Enzimáticos/uso terapêutico , Glioma/tratamento farmacológico , Humanos , Dose Máxima Tolerável , Recidiva Local de Neoplasia , Neoplasias/tratamento farmacológico , Pediatria , Piperidinas/efeitos adversos , Piperidinas/uso terapêutico , Comprimidos , Adulto JovemRESUMO
PURPOSE: International comparisons of patient demographics, tumor characteristics, and survival can shed light on areas for health care system improvement. The International Society of Pediatric Oncology Wilms Tumor 2001 trial/study registered patients through national clinical study groups in Western Europe and Brazil. This retrospective post hoc analysis of the International Society of Pediatric Oncology Wilms Tumor 2001 database aims to make visible and suggest reasons for any variations in outcomes. METHODS: All patients with unilateral Wilms tumor (WT), age > 6 months, treated with preoperative chemotherapy as per protocol, and registered between 2001 and 2011 were eligible. Countries were grouped to give comparable case numbers and geographical representation. Cox univariable and multivariable (MVA) statistics were applied, with the German collaborative group (Gesellschaft für Pädiatrische Onkologie und Hämatologie-Austria, Germany, and Switzerland) as reference for hazard ratios for event-free survival (EFS) and overall survival (OS). RESULTS: A total of 3,176 eligible patients were registered from 24 countries assigned into six groups. Age and histologic risk group distribution were similar across all groupings. The distribution of WT stage varied by country grouping, with 14.9% (range, 11.1%-18.2%) metastatic at diagnosis. Median follow-up was 78.9 months. For localized WT, 5-year EFS varied from 80% (Brazilian group) to 91% (French group; P < .0001), retaining significance only for Brazil in MVA (P = .001). Five-year OS varied from 89% (Brazilian group) to 98% (French group; P < .0001). In MVA, only superior OS in France was significant (P = .001). Five-year EFS/OS for stage IV did not vary significantly. High-risk histology and tumor volume at surgery were significantly associated with increased risk of death in MVA for metastatic disease. CONCLUSION: International benchmarking of survival rates from WT within a large trial/study database has demonstrated statistically significant differences. Clinical interpretation should take account of variation in tumor stage but also treatment factors.
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Neoplasias Renais , Tumor de Wilms , Criança , Feminino , Humanos , Lactente , Neoplasias Renais/diagnóstico , Neoplasias Renais/tratamento farmacológico , Masculino , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Taxa de Sobrevida , Tumor de Wilms/patologia , Tumor de Wilms/cirurgiaRESUMO
The survival of children with diffuse intrinsic pontine glioma (DIPG) remains dismal, with new treatments desperately needed. In a prospective biopsy-stratified clinical trial, we combined detailed molecular profiling and drug screening in newly established patient-derived models in vitro and in vivo. We identified in vitro sensitivity to MEK inhibitors in DIPGs harboring MAPK pathway alterations, but treatment of patient-derived xenograft models and a patient at relapse failed to elicit a significant response. We generated trametinib-resistant clones in a BRAFG469V model through continuous drug exposure and identified acquired mutations in MEK1/2 with sustained pathway upregulation. These cells showed hallmarks of mesenchymal transition and expression signatures overlapping with inherently trametinib-insensitive patient-derived cells, predicting sensitivity to dasatinib. Combined trametinib and dasatinib showed highly synergistic effects in vitro and on ex vivo brain slices. We highlight the MAPK pathway as a therapeutic target in DIPG and show the importance of parallel resistance modeling and combinatorial treatments for meaningful clinical translation. SIGNIFICANCE: We report alterations in the MAPK pathway in DIPGs to confer initial sensitivity to targeted MEK inhibition. We further identify for the first time the mechanism of resistance to single-agent targeted therapy in these tumors and suggest a novel combinatorial treatment strategy to overcome it in the clinic. This article is highlighted in the In This Issue feature, p. 587.
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Neoplasias do Tronco Encefálico , Recidiva Local de Neoplasia , Criança , Humanos , Neoplasias do Tronco Encefálico/tratamento farmacológico , Neoplasias do Tronco Encefálico/genética , Neoplasias do Tronco Encefálico/patologia , Linhagem Celular Tumoral , Dasatinibe/farmacologia , Dasatinibe/uso terapêutico , Quinases de Proteína Quinase Ativadas por Mitógeno , Recidiva Local de Neoplasia/tratamento farmacológico , Estudos Prospectivos , Inibidores de Proteínas Quinases/farmacologia , Inibidores de Proteínas Quinases/uso terapêuticoRESUMO
BACKGROUND: Cannabis is second only to alcohol as a substance of abuse and dependence in the United States. While there is extensive research examining alcohol use and bariatric surgery, there is currently little research and there are no published guidelines specific to cannabis use and bariatric surgery. OBJECTIVES: To identify major themes and general guidelines applied by bariatric surgery psychology clinicians. SETTING: This practice survey was disseminated to bariatric surgery psychologists at various U.S. academic medical centers, hospitals, and private practices. METHODS: An electronic, 35-question survey was sent to 47 bariatric surgery psychologists to collect information on current clinical practice guidelines regarding cannabis use before and after bariatric surgery. RESULTS: The survey questionnaire was completed by 34 (72.34%) bariatric surgery psychologists. The major identified themes included: (1) the lack of a standardized assessment of cannabis use; (2) a requirement for 3 months of abstinence from cannabis before bariatric surgery; (3) recommended lifetime abstinence from cannabis after bariatric surgery; and (4) discussion of cannabis use risks following bariatric surgery, including appetite stimulation, addiction potential, and possible negative impacts on judgment. CONCLUSION: Cannabis use will likely further increase in the United States. This survey highlighted common bariatric surgery psychology practices in the absence of extensive research and published guidelines. These findings suggest a preliminary framework with which to address cannabis use in patients seeking bariatric surgery. It is recommended that professional organizations and societies build on these initial survey findings to develop guidelines for more consistent, evidence-based practice regarding cannabis use and bariatric surgery.
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Cirurgia Bariátrica , Cannabis , Consumo de Bebidas Alcoólicas , Humanos , Prática Psicológica , Inquéritos e Questionários , Estados UnidosRESUMO
INTRODUCTION: High-risk (HR) metastatic (stage IV) Wilms tumours (WTs) have a particular poor outcome. METHODS: Here, we report the results of HR (diffuse anaplastic [DA] or blastemal type [BT]) stage IV WT treated patients according to the HR arm in the SIOP2001 prospective study. RESULTS: From January 2002 to August 2014, 3559 patients with WT were included in the SIOP2001 trial. Among the 525 patients (15%) with metastatic WT, 74 (14%) had stage IV HR-WT. The median age at diagnosis was 5.5 years (range: 1.4-18.3). Thirty-four patients (47%) had BT-WT and 40 (53%) had DA-WT. Five-year event-free survival rates were 44 ± 17% and 28 ± 15% for BT-WT and DA-WT, respectively (p = 0.09). Five-year overall survival rates were 53 ± 17% and 29 ± 16% for BT-WT and DA-WT, respectively (p = 0.03). Metastatic complete response after preoperative treatment was significantly associated with outcome in univariate and multivariate analyses (hazards ratio = 0.3; p = 0.01). Postoperative radiotherapy of metastatic sites might also be beneficial. Forty-three of 74 patients experienced a relapse or progression predominantly in the lungs (80%). The median time to relapse/progression after diagnosis was 7.3 months (range: 1.6-33.3) and 4.9 months (range: 0.7-28.4) for BT-WT and DA-WT, respectively (p = 0.67). This is the first prospective evidence of inferior survival of stage IV BT-WT as compared with historical intermediate-risk WT. Survival of patients with stage IV DA-WT has not improved compared to the previous SIOP93-01 study. CONCLUSION: These results call for new treatment approaches for patients with HR stage IV WT.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Renais/tratamento farmacológico , Recidiva Local de Neoplasia/epidemiologia , Tumor de Wilms/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Dactinomicina/uso terapêutico , Progressão da Doença , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Masculino , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/prevenção & controle , Estadiamento de Neoplasias , Estudos Prospectivos , Taxa de Sobrevida , Fatores de Tempo , Vincristina/uso terapêutico , Tumor de Wilms/mortalidade , Tumor de Wilms/patologiaRESUMO
BACKGROUND: The National Institute for Health and Care Excellence (NICE) guidance for referral of children with suspected cancer was first published in 2005 and updated in 2015. The updated version relied on sparse primary care evidence and published without input from key stakeholders, for example, acute general paediatricians and paediatric haematologists/oncologists. This led to a document that fell short as a practical guide for referring physicians managing children with potentially life-threatening conditions. Following discussions between the Children's Cancer and Leukaemia Group (CCLG, the UK multidisciplinary professional body for healthcare professionals caring for children with cancer) and NICE, it was agreed that a practical supplement should be produced for the 2015 guidance. A prerequisite was evidence gathering from tertiary care to balance the existing primary care evidence, and a Delphi consensus method was therefore convened. METHODS: A CCLG NICE Guidance Committee formulated 25 draft statements for review. The CCLG emailed its paediatric haematologist/oncologist membership (n=179) and 88 responded (49%). To achieve consensus, statements required ≥70% agreement from ≥60% of actual respondents, from the denominator (n=88). RESULTS: Fifteen of 25 original statements were accepted at the first round of voting. Three of 25 statements where >50% did not support were rejected outright. One statement could not be revised without replicating a previously accepted statement. The six remaining statements were revised and a second round of voting undertaken; all six revised statements were accepted. Overall, 21 of 25 statements (84%) met consensus criteria. CONCLUSIONS: This expert opinion should help streamline suspected cancer referral in children and help optimise subsequent outcomes.
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Prova Pericial , Neoplasias/diagnóstico , Guias de Prática Clínica como Assunto , Encaminhamento e Consulta , Criança , Consenso , Técnica Delphi , Detecção Precoce de Câncer , Humanos , Neoplasias/terapia , Fatores de Tempo , Reino UnidoRESUMO
While irinotecan has been studied in various pediatric solid tumors, its potential role in Wilms tumor (WT) is less clear. We evaluated response and outcome of irinotecan-containing regimens in relapsed WT and compared our results to the available literature. Among 14 evaluable patients, one complete response (CR) and two partial responses (PRs) were observed in patients with initial intermediate-risk (CR and PR) and blastemal-type histologies (PR). Two patients were alive at last follow-up showing no evidence of disease. Our results and the reviewed literature suggest some effectiveness of irinotecan in the setting of relapsed WT.
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Camptotecina/análogos & derivados , Neoplasias Renais/tratamento farmacológico , Tumor de Wilms/tratamento farmacológico , Adolescente , Camptotecina/administração & dosagem , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Irinotecano , Masculino , Fatores de RiscoRESUMO
BACKGROUND: Lifestyle factors and genetic information has been found to contribute to the occurrence of lung cancer. This study assessed receptivity to participating in lifestyle programs to reduce cancer risk among unaffected lung cancer family members. We also explored demographic, medical, and psychosocial correlates of willingness to participate in lifestyle programs. METHODS: Family members who are part of a lung Cancer Family Registry were asked to fill out a survey assessing their receptivity to cancer risk reduction programs including preferences for an individual or family-based program. RESULTS: Of the 583 respondents, 85% were "Somewhat" or "Definitely" willing to participate in a lifestyle program. Among those receptive, about half (56%) preferred a family-based approach. Preferred programs included weight management (36%) and nutritional information (30%). Preferred delivery channels were Internet (45%) and mail-based (29%) programs. On multivariate analysis, those definitely/somewhat receptive reported greater exercise self-efficacy scores (p=0.025). CONCLUSION: The majority of the sample was receptive to lifestyle programs that might decrease cancer risk. There was a large preference for family-based weight management and nutritional programs. Further research is indicated to determine how to best incorporate a family-based approach to lifestyle programs for cancer family members.
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Wilms' tumor (WT) is a common childhood renal cancer. A 25-year single center UK experience is reported. During 1985-2010, 97 children underwent immediate nephrectomy or delayed resection of tumor after chemotherapy. Survival, morbidity, and late effects following treatment are described. Tumor distribution was: Stage I, 25.7% (n = 25); Stage II, 24.7% (n = 24); Stage III, 26.8% (n = 26); Stage IV, 17.5% (n = 17); and Stage V, 5.2% (n = 5). Immediate nephrectomy was performed in 39% (n = 38) patients with elective delayed resection in 61% (n = 59) cases. Ten patients had cavotomy to excise tumor involving vena cava territory. Two cases required cardiopulmonary bypass. Tumor rupture was recorded in eight (8.5%) total operated cases-after immediate (n = 5/37), 13.5% vs delayed nephrectomy-(n = 3/57), 5.2%; X(2) P = .154. From 2001 onwards, one case of tumor rupture was recorded at this center after the universal adoption of UKW3 and SIOP guidelines advocating preoperative chemotherapy and delayed nephrectomy for all WT. Three treatment-related deaths occurred-hepatic veno-occlusive disease (n = 2) with actinomycin D and a single WT fatality due to vascular injury. Overall survival was 84.5% (82/97 cases). Two patients developed "late malignancies" -thyroid cancer and a basal cell carcinoma. This study demonstrates excellent survival for WT comparable with national outcomes and international cooperative studies. Adverse events with chemotherapy and surgery, including "late onset," second malignancies deserve special consideration.
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Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Neoplasias Renais/terapia , Tumor de Wilms/mortalidade , Tumor de Wilms/patologia , Tumor de Wilms/terapia , Adolescente , Antibióticos Antineoplásicos/administração & dosagem , Antibióticos Antineoplásicos/efeitos adversos , Criança , Pré-Escolar , Dactinomicina/administração & dosagem , Dactinomicina/efeitos adversos , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estadiamento de Neoplasias , Nefrectomia , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
PURPOSE: Cancer is a shared family experience and may provide a "teachable moment" to motivate at-risk family members to adopt cancer prevention and health promotion behaviors. This study explored how a diagnosis of colorectal cancer (CRC) is experienced by family members and may be used to develop a family-based CRC prevention program. Preferences regarding content, timing, and modes of program delivery were examined. Social cognitive theory provided the conceptual framework for the study. METHODS: This study employed mixed methodology (semi-structured interviews and self-report questionnaires). Participants included 73 adults (21 patients, 52 family members) from 23 families (two patients were deceased prior to being interviewed). Most patients (n = 14; 67 %) were interviewed 1-5 years post-diagnosis. Individual interviews were audio-recorded, transcribed, and content analyzed. RESULTS: For many, a CRC diagnosis was described as a shared family experience. Family members supported each other's efforts to prevent CRC through screening, exercising, and maintaining a healthy diet. Teachable moments for introducing a family-based program included the time of the patient's initial cancer surgery and post-chemotherapy. Reported willingness to participate in a family-based program was associated with risk perception, self-efficacy, outcome expectancies, and the social/community context in which the program would be embedded. Program preferences included cancer screening, diet/nutrition, weight management, stress reduction, and exercise. Challenges included geographic dispersion, variation in education levels, generational differences, and scheduling. CONCLUSIONS: CRC patients and family members are receptive to family-based programs. Feasibility concerns, which may be mitigated but not eliminated with technological advances, must be addressed for successful family-based programs.
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Neoplasias Colorretais/prevenção & controle , Neoplasias Colorretais/psicologia , Família/psicologia , Aceitação pelo Paciente de Cuidados de Saúde , Serviços Preventivos de Saúde , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Colorretais/diagnóstico , Neoplasias Colorretais/epidemiologia , Detecção Precoce de Câncer , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Aceitação pelo Paciente de Cuidados de Saúde/psicologia , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Autoeficácia , Inquéritos e Questionários , Adulto JovemRESUMO
OBJECTIVES: To assess modifiable cognitive and behavioral factors following cardiac events and their association to patients' 3-month survival expectations. METHODS: Patients (N = 233, 71% male; mean age 68years) hospitalized following cardiac events completed study packets assessing mood, behavior change, health behavior domains, and medical recommendation adherence at hospital discharge and 3 months later. RESULTS: In univariate analyses, baseline depression, health distress, behavior change, and adherence were associated with positive expectations at follow-up. Multivariate regression analysis found (Adj. R²=0.43) baseline expectations and adherence were significant predictors of expectations for recovery and survival at follow-up (p < .01). CONCLUSION: Patients' perception of adherence following a cardiac event is a potent predictor of later expectations.
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Ponte de Artéria Coronária/psicologia , Comportamentos Relacionados com a Saúde , Infarto do Miocárdio/psicologia , Cooperação do Paciente , Idoso , Depressão/psicologia , Emoções , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estresse Psicológico/psicologia , Inquéritos e QuestionáriosRESUMO
Providing concise, consistent, and individually relevant patient education is critical. At one institution, patients and families attended a chemotherapy education class consisting of an 11-minute DVD and an oncology nurse-facilitated group discussion. Postclass and eight-week follow-up surveys assessing understanding of treatment side effects, self-care management, and confidence in managing side effects were administered. Quantitative and qualitative data suggested the DVD and oncology nurse-facilitated group discussion provided consistent information, flexibility, and expert knowledge in empowering patients and families to self-manage chemotherapy side effects.
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Antineoplásicos/efeitos adversos , Neoplasias/tratamento farmacológico , Relações Enfermeiro-Paciente , Educação de Pacientes como Assunto/métodos , Poder Psicológico , Autocuidado , Humanos , Neoplasias/enfermagem , Neoplasias/psicologiaRESUMO
BACKGROUND: Cancer is a shared family experience that might provide an opportunity for lifestyle change among at-risk family members. The purpose of this study was to assess receptivity and preferences for cancer risk reduction programs among at-risk family members with two or more relatives affected with pancreas cancer. METHODS: We surveyed 401 at-risk family members in an existing pancreatic cancer family registry. Participants completed a mailed survey which examined demographic, medical, and psychosocial correlates of willingness to participate in lifestyle cancer risk reduction programs. Multivariable generalized estimating equation approaches were used to model preferences. RESULTS: Overall, 85% (n = 342) of at-risk family members were receptive to lifestyle cancer risk reduction programs. Participant preferred programs focused on nutrition (36%, n = 116) and weight management (33%, n = 108), with Web/Internet (46%, n = 157) being the most preferred delivery channel. Most respondents preferred to participate in programs with their family or friends (74%, n = 182), rather than alone (25%, n = 85). In multivariable analysis, younger age (p = 0.008) and higher perceived likelihood of developing cancer (p = 0.03) were associated with willingness to participate in lifestyle programs. CONCLUSIONS: Family members of those with pancreatic cancer are receptive to cancer risk reduction programs focusing on nutrition and weight management delivered via the internet. Further research is indicated to determine how to best incorporate a family-based approach when designing lifestyle intervention programs.
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BACKGROUND: Cancer is a shared family experience, and thus the purpose of this study was to assess receptivity and preferences for cancer risk reduction programs among at-risk family members with two or more relatives affected with colorectal cancer (CRC). METHODS: The sample comprised 401 at-risk family members with two or more relatives affected with CRC from the Colon Cancer Family Registry. In March 2009, respondents completed a mailed survey assessing receptivity and preferences for participating in cancer risk reduction programs and evaluated their relationship to demographic, medical, and psychosocial variables. Multivariable generalized estimating equation approaches were used to model preferences. RESULTS: Overall, 81% of respondents were receptive to a lifestyle cancer risk reduction program; of these, about half (54%) preferred to participate with their family. Program preferences included: weight management (36%) and nutrition (31%); delivered through the internet (41%) or mail (39%). In a multivariate model, a greater level of concern about cancer (p<0.001), female gender (p=0.002), and higher education (p=0.016) were significantly correlated with willingness to participate in lifestyle programs. CONCLUSIONS: Family members of those with CRC are receptive to cancer risk reduction programs that focus on weight management and nutrition delivered via the internet or mail. Future research is needed to determine how best to incorporate a family-based approach that addresses the cancer experience when designing lifestyle intervention programs.
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Hypercalcaemia is a rare life-threatening complication of paediatric cancer that is commoner in haematological than solid malignancies and associated rarely with acute renal failure. Often refractory to medical therapy, control of hypercalcaemia in children with solid tumours may necessitate excision of localised tumours or urgent chemotherapy for metastatic ones. We present a child with refractory hypercalcaemia, bulky chemosensitive metastatic tumours and acute renal failure in whom chemotherapy posed high-risk of tumour lysis syndrome (TLS). Resection of the metastatic tumours successfully normalised the hypercalcaemia and represents a practical alternative control strategy in cases at high risk of TLS.
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Disgerminoma/cirurgia , Hipercalcemia/prevenção & controle , Síndrome de Lise Tumoral/prevenção & controle , Injúria Renal Aguda/epidemiologia , Criança , Disgerminoma/epidemiologia , Disgerminoma/secundário , Feminino , Humanos , Fatores de Risco , Síndrome de Lise Tumoral/etiologiaRESUMO
We report a case of Peutz-Jeghers syndrome (PJS) in a 2-year old with precocious puberty secondary to a Sertoli-Leydig cell tumour. Family history of PJS and other neoplasms were discovered. The tumour was excised and the STK11 gene deletion identified in both patient and father. Screening revealed hamartomatous gastric polyps, which were removed. Current recommendations for screening of children with PJS begin at age 8 years, based on reported occurrence of complications 1. This report illustrates the importance of considering early screening, along with close clinical review and patient/parent education, for detection of life threatening neoplasms and complications.
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Mucosa Gástrica/patologia , Hamartoma/diagnóstico , Neoplasias Ovarianas/diagnóstico , Síndrome de Peutz-Jeghers/diagnóstico , Pólipos/diagnóstico , Tumor de Células de Sertoli-Leydig/diagnóstico , Neoplasias Gástricas/diagnóstico , Quinases Proteína-Quinases Ativadas por AMP , Pré-Escolar , Feminino , Mucosa Gástrica/cirurgia , Deleção de Genes , Hamartoma/complicações , Hamartoma/genética , Hamartoma/cirurgia , Humanos , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/genética , Síndrome de Peutz-Jeghers/complicações , Síndrome de Peutz-Jeghers/genética , Pólipos/genética , Pólipos/cirurgia , Proteínas Serina-Treonina Quinases/genética , Tumor de Células de Sertoli-Leydig/complicações , Tumor de Células de Sertoli-Leydig/genética , Neoplasias Gástricas/complicações , Neoplasias Gástricas/genética , Neoplasias Gástricas/cirurgiaRESUMO
We report a 6-year-old male with left-sided ptosis, aniscoria and an initially missed slow growing left-sided neck mass, which was surgically excised when he was 9 years old and confirmed to be a paraganglioma. Seven years later he developed recurrent symptoms and was found to have a recurrence in the anterior mediastinum. We also report on all cases of cervical paragangliomas registered with the Manchester Children's Tumour Registry (MCTR) for the 50-year period 1954-2004. Paragangliomas are very rare tumours in the head and neck but should be considered in the differential diagnosis of neck masses especially when presenting with Horner syndrome. Recurrent symptoms and signs of hypertension herald recurrence. As these tumours can form part of a familial syndrome, long-term follow-up is necessary. Family members should be screened for early detection.
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Neoplasias de Cabeça e Pescoço/cirurgia , Neoplasias do Mediastino/cirurgia , Recidiva Local de Neoplasia/cirurgia , Paraganglioma/cirurgia , Sistema de Registros , Adolescente , Criança , Diagnóstico Diferencial , Feminino , Neoplasias de Cabeça e Pescoço/diagnóstico , Humanos , Lactente , Masculino , Neoplasias do Mediastino/diagnóstico , Recidiva Local de Neoplasia/diagnóstico , Paraganglioma/diagnósticoRESUMO
This exploratory study examined frequency of self-reported cervical and breast cancer screening rates in a multiethnic sample of 331 female university students. In general, rates of lifetime screening were fairly low, with only 41% reporting having ever had a PAP examination and 44% reporting having undergone at least one clinical breast examination. Screening rates differed by ethnicity. Non-Hispanic White and African-American women had higher screening rates than Asian/Pacific Islander and Latina women. The significantly lower rate of screening among the latter groups is of particular concern given the higher cancer mortality rates typically observed in those groups.