Pediatric Oncology Team Research Articles

8657 Thymic Neoplasia In A Pediatric Patient With LI-Fraumeni Syndrome: A Case Report

Abstract Disclosure: M.A. Souza: None. W.M. Lins: None. D. di Matteo: None. J.L. Marcayata: None. A. Kuhn: None. H. Charcar: None. F. Ledesma: None. M.Q. Almeida: None. F. Jatene: None. B.B. Mendonca: None. M.C. Fragoso: None. Introduction: Li-Fraumeni Syndrome (LFS) is a rare autosomal dominant hereditary cancer predisposition syndrome primarily caused by pathogenic variants in the TP53 tumor suppressor gene. The individuals affected are typically young, with a cancer risk of approximately 90% in lifetime. Parents choosing to test their young children face the dilemma of when to disclose their own or the child's status and when to initiate cancer screening if LFS is detected. This process presents psychosocial challenges for both parents and affected children. Both providers and patients question whether the surveillance benefits outweigh the psychological burden of knowing LFS status and screening-related concerns, creating ambivalence toward genetic testing decisions.Case Report: We describe a 6-year-old male with Li-Fraumeni syndrome carrier of the germline pathogenic variant TP53 c.818G>A (p.R273H) in heterozygous (exon 8).In the family history, there are three relatives with advanced tumor cases, with the grandmother already deceased, the mother undergoing chemotherapy treatment, and the cousin being cancer-free for 10 years.The patient in question had no gestational complications. No consanguinity between parents. No signs or symptoms of hormonal hyperfunction. As proposed in the literature, Li-Fraumeni Syndrome patients should undergo frequent screening for early tumor detection, the patient began screening according to the Toronto protocol in 2018, at the age of 1 year and 7 months, through cranial and thoracic MRI, abdominal ultrasound, and annual hormonal assays. In the thoracic MRI conducted in February 2023, an enlarged and heterogeneously signaling thymus was identified, with a nodular formation (absent in previous exams), measuring about 2.5 cm. Facing suspicion of a thymoma, thymic carcinoma, or lymphoma, a biopsy guided by tomography in March 2023 was done. Histological examination was compatible with primary thymic neoplasia. During follow-up, a significant growth of the mass to 9 cm was observed. The patient underwent surgical treatment in August 2023 with resection of the mediastinal mass, and the anatomopathological examination revealed undifferentiated thymic carcinoma, followed in conjunction with the pediatric oncology team. The dilemma is about the adjuvant radiotherapy since the possibility to acquire a new tumor radio-induced such as sarcoma. We proposed to follow with MRI imaging each 3 months and if minimal signal of recurrence he will underwent radiotherapy. Conclusion: In addition to the intrinsic rarity of Li-Fraumeni Syndrome, this case draws attention to the possibility of a thymic tumor, an extremely rare neoplasm in syndrome carriers. In the literature, only scattered case reports show this association. Furthermore, this case reinforces the importance of systematic screening for investigating possible neoplasms in this susceptible population. Presentation: 6/1/2024

8657 Thymic Neoplasia in a Pediatric Patient With LI-Fraumeni Syndrome: A Case Report

Abstract Disclosure: M.A. Souza: None. W.M. Lins: None. D. di Matteo: None. J.L. Marcayata: None. A. Kuhn: None. H. Charcar: None. F. Ledesma: None. M.Q. Almeida: None. F. Jatene: None. B.B. Mendonca: None. M.C. Fragoso: None. Introduction: Li-Fraumeni Syndrome (LFS) is a rare autosomal dominant hereditary cancer predisposition syndrome primarily caused by pathogenic variants in the TP53 tumor suppressor gene. The individuals affected are typically young, with a cancer risk of approximately 90% in lifetime. Parents choosing to test their young children face the dilemma of when to disclose their own or the child's status and when to initiate cancer screening if LFS is detected. This process presents psychosocial challenges for both parents and affected children. Both providers and patients question whether the surveillance benefits outweigh the psychological burden of knowing LFS status and screening-related concerns, creating ambivalence toward genetic testing decisions.Case Report: We describe a 6-year-old male with Li-Fraumeni syndrome carrier of the germline pathogenic variant TP53 c.818G>A (p.R273H) in heterozygous (exon 8).In the family history, there are three relatives with advanced tumor cases, with the grandmother already deceased, the mother undergoing chemotherapy treatment, and the cousin being cancer-free for 10 years.The patient in question had no gestational complications. No consanguinity between parents. No signs or symptoms of hormonal hyperfunction. As proposed in the literature, Li-Fraumeni Syndrome patients should undergo frequent screening for early tumor detection, the patient began screening according to the Toronto protocol in 2018, at the age of 1 year and 7 months, through cranial and thoracic MRI, abdominal ultrasound, and annual hormonal assays. In the thoracic MRI conducted in February 2023, an enlarged and heterogeneously signaling thymus was identified, with a nodular formation (absent in previous exams), measuring about 2.5 cm. Facing suspicion of a thymoma, thymic carcinoma, or lymphoma, a biopsy guided by tomography in March 2023 was done. Histological examination was compatible with primary thymic neoplasia. During follow-up, a significant growth of the mass to 9 cm was observed. The patient underwent surgical treatment in August 2023 with resection of the mediastinal mass, and the anatomopathological examination revealed undifferentiated thymic carcinoma, followed in conjunction with the pediatric oncology team. The dilemma is about the adjuvant radiotherapy since the possibility to acquire a new tumor radio-induced such as sarcoma. We proposed to follow with MRI imaging each 3 months and if minimal signal of recurrence he will underwent radiotherapy. Conclusion: In addition to the intrinsic rarity of Li-Fraumeni Syndrome, this case draws attention to the possibility of a thymic tumor, an extremely rare neoplasm in syndrome carriers. In the literature, only scattered case reports show this association. Furthermore, this case reinforces the importance of systematic screening for investigating possible neoplasms in this susceptible population. Presentation: 6/1/2024

Integrating Audiovisual Immersion Into Pediatric Radiation Therapy Across Multiple Centers: Methodology, Timeliness, and Cost of the Audiovisual-Assisted Therapeutic Ambience in Radiation Therapy Prospective Multi-Institutional Trial

PurposeThe Audiovisual Assisted Therapeutic Ambience in Radiotherapy (AVATAR) trial was a prospective multicenter study (NCTXXXXXXXX) examining the combination of video immersion with radiotherapy and was successfully conducted through the collaboration of pediatric radiation oncology teams at 10 institutions independent of any preexisting consortium. We sought to analyze and report the methodology of trial conception and development, process map, and cost. Methods and MaterialsThe study enrolled patients aged 3-10 years preparing to undergo radiation therapy, integrated the combination of AVATAR-based video immersion during radiation therapy at each institution, and offered AVATAR use as an alternative to anesthesia, with rates of anesthesia use and outcomes of serial standardized anxiety and quality of life assessments assessed among the 81 children enrolled. A process map was created based on the trial timeline with the following components: study development time (time from conception of the trial to accrual of first patient, including design phase, agreement and approval phase and site preparation phase), and accrual duration time (time from the first to last accrual). Costs and institutional success rates were calculated. ResultsTime from inception of study to last accrual was 3.6 years (1313 days). The study development time was 417 days (31.7%) and accrual duration time was 896 days (68.3%), with the final 50% of accrual occurring in less than 6 months. Equipment cost was approximately $550 per institution and was covered by funding from the lead study institution. All 10 centers were successful with AVATAR implementation, defined as ≥50% of patients able to avoid anesthesia with use of AVATAR, including centers with both photon and proton therapy. ConclusionsThis report elaborates on the methodology and timeline of trial conception and development using data from a previously published supportive care study combining video immersion with radiotherapy among 10 cooperating pediatric oncology institutions. It highlights the potential for multicenter collaborations on prospective trials integrating supportive care therapies with radiotherapy.

Wilms Tumor with Vena Caval Intravascular Extension: A Surgical Perspective.

Wilms tumor (WT) is the most common kidney tumor in pediatric patients. Intravascular extension of WT above the level of the renal veins is a rare manifestation that complicates surgical management. Patients with intravascular extension are frequently asymptomatic at diagnosis, and tumor thrombus extension is usually diagnosed by imaging. Neoadjuvant chemotherapy is indicated for thrombus extension above the level of the hepatic veins and often leads to thrombus regression, obviating the need for cardiopulmonary bypass in cases of cardiac thrombus at diagnosis. In cases of tumor extension to the retrohepatic cava, neoadjuvant therapy is not strictly indicated, but it may facilitate the regression of tumor thrombi, making resection safer. Hepatic vascular isolation and cardiopulmonary bypass increase the risk of bleeding and other complications when utilized for tumor thrombectomy. Fortunately, WT patients with vena caval with or with intracardiac extension have similar overall and event-free survival when compared to patients with WT without intravascular extension when thrombectomy is successfully performed. Still, patients with metastatic disease at presentation or unfavorable histology suffer relatively poor outcomes. Dedicated pediatric surgical oncology and pediatric cardiothoracic surgery teams, in conjunction with multimodal therapy directed by a multidisciplinary team, are preferred for optimized outcomes in this patient population.

Developmental surveillance and screening practices in a pediatric oncology clinic: Initial progress of a quality improvement study.

Pediatric cancer patients' oncology teams regularly take on a primary care role, but due to the urgent nature of cancer treatment, developmental screenings may be deprioritized. This leaves patients at risk of developmental diagnoses and referrals being delayed. Clarify the current developmental surveillance and screening practices of one pediatric oncology team. Researchers reviewed charts for patients (n=66) seen at a pediatric oncology clinic in a suburban academic medical center to determine engagement in developmental screening (including functioning around related areas such as speech, neurocognition, etc.) and referrals for care in these areas. Developmental histories were collected from all patients through admission history and physical examination (H&P), but there was no routinized follow-up. Physicians did not conduct regular developmental screening per American Academy of Pediatrics guidelines for any patients but identified n=3 patients with needs while the psychology team routinely surveilled all patients seen during this time (n=41) and identified n=18 patients as having delays. Physicians did not routinely screen for development needs beyond H&P and were inconsistent in developmental follow-up/referrals. Integrated psychologists were key in generating referrals for developmental-based care. However, many oncology patients were not seen by psychologists quickly or at all, creating a significant gap in care during a crucial developmental period. The case is made for further routinization of ongoing developmental screening in pediatric oncology care.

Guidelines for outpatient administration of naxitamab: Experience from Atrium Health Levine Children's Hospital.

In this publication, we will share our experience of AE management, provide guidance for appropriate staffing, and the discuss the importance of patient education when treating patients with R/R HR neuroblastoma using naxitamab. Approved treatments for patients with refractory and/or relapsed (R/R) high-risk (HR) neuroblastoma are limited, and there is a high unmet need for new treatment combinations. Naxitamab is a disialoganglioside 2 (GD2)-binding antibody that was approved by the United States Food and Drug Administration in 2020 for use in combination with granulocyte-macrophage colony-stimulating factor for the treatment of patients with R/R HR neuroblastoma in the bone and/or bone marrow and who have demonstrated a partial response, minor response, or stable disease with prior therapy. The pediatric oncology team at Atrium Health Levine Children's Hospital has successfully treated several patients with naxitamab both alone and in combination with chemotherapy, with no patients requiring unplanned overnight hospitalization and few severe adverse events (AEs). To accomplish this, the team at Levine Children's Hospital established standard operating procedures for naxitamab, a therapy defined as high acuity due to the potential for acute AEs with rapid onset and that benefits from continuous monitoring by a nursing team and a dedicated provider. This will provide a practical guide for institutions offering naxitamab to their patients, and ensure successful administration of this high acuity treatment in the outpatient setting.

Implementing an Antibiotic Stewardship Program in National Cancer Institute, Sri Lanka

Abstract Bacterial infection is a dreaded complication in cancer patients worldwide. In low resource settings where supportive care facilities are less than ideal this takes on even more greater significance. In recent years, the emergence of antimicrobial resistance (AMR) has become a significant problem worldwide and cancer patients are those among most affected. Treatment of infections due to Multi Drug Resistant (MDR) bacteria represents a clinical challenge because therapeutic options are limited. Overuse and misuse of antimicrobials has been acknowledged as a main driver for resistance development and the need to optimize the use of antimicrobials has been stressed. National Cancer Institute is not an exception, children with cancer develop infections due to MDR bacteria when they are neutropenic and mortality and morbidity of those infections are very high. Therefore antibiotic stewardship program (AMSP) was implemented in the pediatric units to optimize clinical outcome while minimizing the unwanted effects, with the rational prescribing of antibiotics. Pediatric Oncology unit comprises of four wards with 150 beds and an intensive care unit with 8 beds. As a preliminary step meetings were conducted to outline a plan of action with the participation of relevant stake holders, microbiology team and pediatric Oncology team. Director of the hospital was also involved to the program. Workshops were conducted to educate and train health care workers and to create awareness on AMR. Antibiogram was established to see the antibiotic resistant patterns of bacterial isolates in the hospital. The audit on antibiotic use was conducted to see the use of antibiotics in the units. Also feedback of the audit was given to the clinicians, it showed deviations from standard practices in the use of antibiotics. It was noted that the antibiotic resistance rates in the unit was very high for both gram negative and gram positive isolates. Clinical practice guideline on fever and neutropenia was developed based on the sensitivity pattern of the antibiogram. The guideline comprised of introduction, basic investigations, antibiotic recommendations, follow-up assessments and antimicrobial dose and frequency. The clinical practice guideline was displayed as a poster in all units near the doctors table. Small booklet was also printed with pediatric dose, duration and route of commonly used antibiotics with their indications. A new drug chart was introduced to patients' bed head ticket with a separate section for antimicrobials including history of allergy, designation of the prescriber, reminder to collect cultures, dose, route, duration and periodic review. Fourteen antibiotics were included in the preauthorization list, which require approval prior to use. Antibiotic resistant rates in the unit was alarmingly high. Therefore, there should be a mechanism for continuous monitoring and auditing. Infection control practices need to be strengthen to prevent the spread of MDR organisms. Better coordination between clinical microbiologist and physicians is required on local resistant patterns of microorganisms. Periodic training of hospital staff should be carried out on AMSP, including adverse reactions, antibiotic resistance and optimal prescribing. Evaluation of AMSP and their perceive level of success needs to be performed periodically.

Optimizing discharge readiness to reduce health disparities for at-risk children with cancer in the Bronx.

407 Background: Transitions in care between hospital and home are overwhelming for patients and families, and often result in medical errors. Children with cancer are at an increased risk of adverse outcomes due to their immunocompromised state. Despite a robust therapeutic armamentarium, unfavorable outcomes secondary to disease- and treatment-related complications remain a challenge, and children with cancer are particularly vulnerable to these outcomes during transitions of care. At our institution, the pediatric oncology team noted that caregivers of patients with new pediatric cancer diagnoses often admitted to confusion regarding infection precautions, central venous catheter care, and medications when returning for follow up appointments. This experience is supported by data showing that caregivers of patients with leukemia feel subjectively unprepared to take care of patients after hospital discharge. The increasing prioritization of outpatient management in healthcare has shifted responsibility for identifying and managing emergencies and complications to caregivers. Methods: We initiated a comprehensive cancer-specific program through the use of quality improvement measures and four plan-do-study-act (PDSA) cycles to ensure consistency of healthcare provider-delivered education and improve caregiver knowledge of homecare instructions in a high-risk patient population. Results: Based on our recent publication, overall retention of key discharge information was greater in the intervention cohort compared to the non-intervention cohort (median total scores: 89 and 63, respectively; p=0.001). Improvements in the proportions of correct responses post-intervention were also observed across all subject matters: from 0.57 to 0.88 for fever guidelines (p=0.059), from 0.71 to 0.78 for signs of sepsis (p=0.65), from 0.57 to 1.00 for accurate choice of on-call number (p=0.004), and from 0.71 to 0.94 for antiemetic management (p=0.14). Conclusions: Our study demonstrated that standardized processes with a multi-disciplinary team and task allocation significantly improves ability of caregivers to retain important information after a new cancer diagnosis, with specific improvement in retention of fever guidelines and on-call contact instructions. During our intervention, it became clear that as team members change (residents changing cycle, oncologist going off service, nurses switching), having one point person is absolutely critical in ensuring discharge education continues. We anticipate that a dedicated patient navigator focused on consistent psychosocial screening and health literacy comprehension of key discharge details would lead to a marked improvement in cancer care delivery for children with cancer in the Bronx; specifically, discharge readiness, medication adherence, family satisfaction, and patient safety for children with cancer in the Bronx.

Incorporating cancer survivorship care in dental education.

Incorporating cancer survivorship care in dental education.

LANGERHANS CELL HISTIOCYTOSIS IN MANDIBLE OF PEDIATRIC PATIENT: A CASE REPORT

The mandible is one of the most affected bones in Langerhans cell histiocytosis (LCH), along with the skull, ribs, and vertebrae. Oral lesions may be the first/only sign of the condition, which increases the importance of early and assertive diagnosis. Our goal is to report a case of a 3-year-old male with LCH in the mandible, highlighting the challenging diagnosis. The patient had a persistent swelling in the right submandibular region and was referred to Erasto Gaertner Cancer Center with a main hypothesis of odontogenic infection/osteomyelitis. Histopathologic analysis of an oral biopsy specimen suggested a central giant cell lesion, but questions remained about the real nature of the lesion. Lymph node biopsy showed multifocal involvement by atypical and eosinophilic histiocyte aggregates and immunohistochemical analysis confirmed the diagnosis of LCH through positive tests for CD1a, CD68, S-100, and vimentin. The patient was treated by the pediatric oncology team with exclusive chemotherapy with a good outcome. The mandible is one of the most affected bones in Langerhans cell histiocytosis (LCH), along with the skull, ribs, and vertebrae. Oral lesions may be the first/only sign of the condition, which increases the importance of early and assertive diagnosis. Our goal is to report a case of a 3-year-old male with LCH in the mandible, highlighting the challenging diagnosis. The patient had a persistent swelling in the right submandibular region and was referred to Erasto Gaertner Cancer Center with a main hypothesis of odontogenic infection/osteomyelitis. Histopathologic analysis of an oral biopsy specimen suggested a central giant cell lesion, but questions remained about the real nature of the lesion. Lymph node biopsy showed multifocal involvement by atypical and eosinophilic histiocyte aggregates and immunohistochemical analysis confirmed the diagnosis of LCH through positive tests for CD1a, CD68, S-100, and vimentin. The patient was treated by the pediatric oncology team with exclusive chemotherapy with a good outcome.

Palliative Care in Pediatric Oncology and Hematopoietic Stem Cell Transplantation.

To summarize pediatric palliative care (PPC) research from 2016 to 2021 as it intersects with pediatric oncology and hematopoietic stem cell transplantation (HSCT). Children and adolescents with cancer who receive PPC have improved quality of life (QOL), symptom burden, advance care planning discussions, rates of hospice enrollment, home deaths, and receive less intensive therapy at the end-of-life (EOL). Parents report improved QOL and preparation for EOL. Though barriers to PPC utilization exist, new clinical models, oncology team education, and growing family awareness are leading to culture change. PPC within pediatric oncology is considered a standard of care. Families are accepting of PPC, as most wish for their children to live as well as possible for as long as possible. Although PPC remains underutilized, PPC should work collaboratively with pediatric oncology and HSCT teams to improve QOL and EOL outcomes of patients with cancer and their families.

Assessing the quality of deliberative stakeholder consultations involving allied health professionals in pediatric palliative care and hematology/oncology in Canada

BackgroundIn this paper we assess the quality of six deliberative stakeholder consultations regarding the implementation of a precision diagnostic for life-threatening pediatric brain tumors. Decision makers who base policy recommendations on the outputs of consultative exercises can presuppose that all deliberants are well informed of the policy issue, that participation in the deliberative process was fair, and that overcoming implementation barriers will necessarily result in practice change. Additional evidence is therefore needed to substantiate the informational quality of the deliberation, measure the equality of participation and study the effects on stakeholder reasoning to appropriately guide uptake of proposed recommendation(s).MethodsUsing the DeVries framework for assessing the deliberative quality, we analyzed data from 44 post-consultation evaluation surveys completed by pediatric oncology and palliative care teams at two tertiary pediatric healthcare centers in Canada. We also conducted turn-taking and word-contribution analyses from the text transcriptions of each deliberation to assess equality of participation using descriptive statistics.ResultsDeliberants agreed the quality of the deliberative process was fair (median ratings ranging from 9–10 out of 10) and the opportunities to receive expert information and discuss with others about the implementation of a new LDT were helpful (9.5 out of 10). While the session improved understanding of the implementation barriers and opportunities, it had marginal effects on deliberants’ reasoning about whether LDTs would change their own clinical practice (3–10 out of 10). Participation was proportionate in at least four of the six deliberations, where no deliberant took more than 20% of total turns and contributed equal to, or less than 20% of total words.ConclusionThe quality assessment we performed demonstrates high informational value and perceived fairness of two deliberative stakeholder consultations involving pediatric palliative care and oncology teams in Canada. Quality assessments can reveal how the process of deliberation unfolds, whether deliberative outputs are the result of equitable participation among deliberants and what, if any, stakeholder voices may be missing. Such assessments should be routinely reported as a condition of methodological rigor and trustworthiness of deliberative stakeholder engagement research.

Resilient health care in global pediatric oncology during the COVID‐19 pandemic

BackgroundIn the face of unprecedented challenges because of coronavirus disease 2019, interdisciplinary pediatric oncology teams have developed strategies to continue providing high‐quality cancer care. This study explored factors contributing to health care resilience as perceived by childhood cancer providers in all resource level settings.MethodsThis qualitative study consisted of 19 focus groups conducted in 16 countries in 8 languages. Seven factors have been previously defined as important for resilient health care including: 1) in situ practical experience, 2) system design, 3) exposure to diverse views on the patient's situation, 4) protocols and checklists, 5) teamwork, 6) workarounds, and 7) trade‐offs. Rapid turn‐around analysis focused on these factors.ResultsAll factors of health care resilience were relevant to groups representing all resource settings. Focus group participants emphasized the importance of teamwork and a flexible and coordinated approach to care. Participants described collaboration within and among institutions, as well as partnerships with governmental, private, and nonprofit organizations. Hierarchies were advantageous to decision‐making and information dissemination. Clinicians were inspired by their patients and explained creative trade‐offs and workarounds used to maintain high‐quality care.ConclusionsFactors previously described as contributing to resilient health care manifested differently in each institution but were described in all resource settings. These insights can guide pediatric oncology teams worldwide as they provide cancer care during the next phases of the pandemic. Understanding these elements of resilience will also help providers respond to inevitable future stressors on health care systems.

Hearing Loss After Radiation and Chemotherapy for Central Nervous System and Head and Neck Tumors in Children

Hearing loss (HL) is a serious secondary effect of treatment for head and neck (H&N) and central nervous system (CNS) tumors in children. Radiation and platinum chemotherapy independently increase the risk of HL; however, combined modality treatment is routinely used and the effect of chemoradiation on HL risk is not well-studied. Using chemotherapy and cochlear radiation (RT) doses, we created a model that predicts for HL, including a nomogram that can be used in the clinical setting to calculate the risk of clinically-significant HL.In this single institution retrospective study, 171 patients with H&N or CNS tumors were treated with radiation, with or without chemotherapy and had longitudinal (≥2) audiological evaluation. SIOP-Boston (SIOP) and Chang grades were assigned to 2,420 hearing assessments of 342 ears; analyses using SIOP grades are presented here. For the nomogram, SIOP grade ≥3 was considered clinically-significant HL (requiring hearing aids). An Andersen-Gill model for recurrent events was used to obtain inverse intensity weights to account for factors explaining the number of assessments. Multivariable weighted ordinal logistic regression was fitted to evaluate the effect of clinicopathologic features on HL. Clustered robust standard errors were calculated to account for intra-patient correlation.Patients underwent a median of 6 (range 2-23) assessments over a median of 3.1 years from diagnosis to last audiogram (range 0.1-15.2 years). Cisplatin was given to 63% of patients and 34% received carboplatin. The mean cochlear doses on the right and left were 36.8 Gy (standard deviation [SD] 16.5) and 37.0 Gy (SD 16.2), respectively. Multivariable regression revealed that mean cochlear dose (odds ratio [OR] 1.04 per Gy, 95% confidence interval [CI] 1.02-1.05, P < 0.001), time since RT (OR 1.2 per year, 95% CI 1.2-1.3, P < 0.001), cisplatin use (OR 5.33, 95% CI 2.9-9.9, P < 0.001), and carboplatin use (OR 2.3, 95% CI 1.27-4.17, P = 0.006) were associated with increasing SIOP grade of HL; age at RT, hydrocephalus, surgery, amifostine, and laterality were not correlated with HL. There was no synergistic effect of RT and cisplatin (interaction term, P = 0.4) or RT and carboplatin (interaction term, P = 0.9). Cumulative incidence of high-frequency HL (> 4 kHz) was > 50% at 5 years post-RT in those who received mean dose > 20 Gy to the cochlea, while incidence of HL across all frequencies continued to increase beyond 5 years post-RT.RT and chemotherapy have an additive, not synergistic, effect on HL risk. Mean cochlear radiation dose, time since radiation, and platinum chemotherapy use were associated with HL. This modelling can guide survivorship care by multidisciplinary pediatric oncology teams with respect to audiology follow-up in an effort to that ensure suitable educational accommodations and assistive devices are in place.

An Interprofessional Team-Based Intervention to Address Barriers to Initiating Palliative Care in Pediatric Oncology: A Multiple-Method Evaluation of Feasibility, Acceptability, and Impact

ContextMany children with advanced cancer are not referred to palliative care despite both professional recommendations to do so and bereaved parental preference for earlier support from sub-specialty palliative care. ObjectivesTo assess the feasibility, acceptability, and impact of an adaptive intervention to address individual and team-level barriers to specialty palliative care referrals. MethodsA multiple-method approach assessed feasibility and acceptability among clinicians from pediatric oncology teams at a single institution. Quantitative measures of comfort with palliative care consultations, team cohesion, and team collaboration were conducted before and after the intervention. Number of palliative care consults were examined before, during, and after sessions. Intervention satisfaction surveys and qualitative interviews were conducted after the intervention. ResultsTwenty-six team members (90% of consented) attended at least one intervention session with 20 (69%) participants completing 75% or more sessions. The intervention was modified in response to participant feedback. After the intervention, participants reported greater team cohesion, comfort discussing palliative care consultation, team collaboration, process satisfaction, and decision satisfaction. Participants agreed that the training was useful, effective, helpful, and worthwhile, that they would use the skills, and that they would recommend the training to other providers. The numbers of palliative care consults increased before intervention sessions were conducted, but did not significantly change during or after the sessions. In the interviews, participants reported overall favorably regarding the intervention with some participants reporting changes in practice. ConclusionAn adaptive intervention to reduce barriers to initiating palliative care for pediatric oncology teams is feasible and acceptable.

Integrating a palliative approach into the healthcare provided by the French-African Pediatric Oncology Group's pilot units. Insights from a 3-year training program

Working alongside local stakeholders, members of the French-African Pediatric Oncology Group developed a 3-year program to train pediatric oncology teams from 15 French-speaking countries in Africa in using analgesics and providing palliative care. This program was rolled out in three phases: initial training, in situ assessment, and advanced training in selected topics. To access this program, multidisciplinary teams had to come up with a project to improve their existing palliative care and pain management practices, and commit themselves to implementing it. All the teams invited agreed to take part in the program, which explicitly broached a subject that is often avoided in oncology teaching. The first phase was rolled out in 2017, with 65 trainees from 19 units attending one of three sessions held in Dakar, Senegal, Abidjan, Côte d’Ivoire, and Rabat, Morocco. The subsequent assessment revealed that only half the teams had started to implement their projects. The advanced training phase was therefore adjusted accordingly. A collective training session held in Marseille was attended by 15 trainees from seven teams whose projects were already underway, while in situ mentoring was provided for six other teams, through French–African twinnings in four cases. The length and openness of the program meant that we were able to identify and share the units’ diverse realities, and fine-tune their projects accordingly, as well as plan ways of continuing the training both locally and collectively.

Enrollment barriers of adolescents and young adults (AYA) on the non-chemotherapy arm of ARST1321.

e19214 Background: ARST1321(PAZNTIS): A Phase II/III Randomized Trial of Preoperative Chemoradiation or Preoperative Radiation Plus or Minus Pazopanib (NCT02180867) was the first National Clinical Trials Network (NCTN) study co-developed by pediatric (COG) and adult (NRG Oncology) consortia anticipating enrollment of adolescent and young adult (AYA) cancer patients. ARST1321 had two treatment cohorts, enrolling patients ≥ 2 years of age to either chemotherapy (C) (chemoradiation ± pazopanib) or non-chemotherapy (NC) (radiation ± pazopanib) arms. It was anticipated adults would contribute the majority of enrollments on the NC arm based on prior enrollment patterns. While the C arm accrued as anticipated (with high enrollment of adults and children), the NC arm had low enrollment leading to premature closure. We report on AYA accrual (defined as 15-39 years) to the NC arm and a survey aiming to identify barriers to enrolling AYA patients onto ARST1321. Methods: Our survey was emailed to 161 adult, surgical, and radiation oncologists at large sarcoma centers. A link was sent to an online questionnaire via SurveyMonkey Inc. and responses were reviewed on their platform. Results: 33 patients enrolled on the ARST1321 NC arm, of which 24% were AYA. 25% of AYA enrollments were from non-COG adult cooperative groups. This trial arm was closed in October 2017 after 3.25 years of accrual below anticipated rates. The survey response rate was 31% with a 70% completion rate. Almost half of respondents were medical oncologists with most seeing 50-200 new sarcoma cases/year at an academic institution and 30% in a pediatric environment (divided equally between radiation and surgical oncologists). 70% of respondents have a joint collaboration with their pediatric oncology team with 23% having a joint clinic. 70% of respondents’ sites opened ARST1321 and anticipated 1-5 patients would be eligible for the NC cohort. However, 42% of respondents’ sites had zero patients enrolled on that arm. The most common reasons for not opening the study and/or not enrolling patients on the NC arm included: lack of interest, disagreement with the therapy, lack of a site investigator, premature study closure, patient/family decision, competing trials, insufficient reimbursement, and regulatory delays. Conclusions: Our survey highlights multiple barriers to enrollment of AYA onto cross-NCTN consortia clinical trials spanning the age spectrum. The information obtained will help inform investigators aiming to effectively design, enroll, and conduct similar trial efforts for AYA in the future.

Oral and dental considerations in pediatric cancers.

Oral health care is an integral component of interprofessional collaborative care for children and adolescents diagnosed with cancer. The current review highlights the phases of cancer therapy when dental interventions and palliative care are necessary for children diagnosed with cancer. Contemporary research and review articles pertinent to the oral and dental complications during pediatric cancer therapy and late effects in pediatric cancer survivors were identified by PubMed/MEDLINE search. Best practice guidelines set forth by specialty organizations were also included. The literature search was limited to articles published in the English language. Baseline oral and dental health assessment should occur before initiation of cancer therapy to prevent debilitating complications during the immunosuppressed phase. Counseling on preventive oral health practices is imperative during cancer treatment. Ideally, all dental treatment should be completed before initiation of immunosuppressive therapy. Palliative care and treatment for mucositis, opportunistic oral infections, pain, and other oral complications associated with cancer therapy should be provided as necessary. Survivors of childhood cancers present with unique craniofacial and dental anomalies, dependent on the type of cancer treatment and age at the time of treatment. Pediatric dentists and pediatric oncology teams work collaboratively to screen for and treat dental and oral diseases. As the survival rates of childhood cancers improve, it is essentialfor the dental profession to provide the individualized care necessary for this vulnerable population. The oral health profession also reinforces health practices congruent with cancer prevention and cancer screening.

Seven Types of Uncertainty When Clinicians Care for Pediatric Patients With Advanced Cancer

ContextClinicians deciding whether to refer a patient or family to specialty palliative care report facing high levels of uncertainty. Most research on medical uncertainty has focused on prognostic uncertainty. As part of a pediatric palliative referral intervention for oncology teams we explored how uncertainty might influence palliative care referrals. ObjectivesTo describe distinct meanings of the term “uncertainty” that emerged during the qualitative evaluation of the development and implementation of an intervention to help oncologists overcome barriers to palliative care referrals. MethodsWe conducted a phenomenological qualitative analysis of “uncertainty” as experienced and described by interdisciplinary pediatric oncology team members in discussions, group activities and semistructured interviews regarding the introduction of palliative care. ResultsWe found that clinicians caring for patients with advanced cancer confront seven broad categories of uncertainty: prognostic, informational, individual, communication, relational, collegial, and inter-institutional. Each of these kinds of uncertainty can contribute to delays in referring patients to palliative care. ConclusionVarious types of uncertainty arise in the care of pediatric patients with advanced cancer. To manage these forms of uncertainty, providers need to develop strategies and techniques to handle professionally challenging situations, communicate bad news, manage difficult interactions with families and colleagues, and collaborate with other organizations.

How I approach expressing condolences and longitudinal remembering to a family after the death of a child.

Bereaved families fear their child being forgotten by those who knew their loved child, including their child's oncology team. Thoughtfully timed, family-centric condolences shared by pediatric oncology team members have the potential to extend our compassion and kindness toward a family during the darkness of grief. Well-intended medical teams sometimes feel "at a loss" in terms of what to say to a grieving family and how or when to say it. This paper provides a tangible overview of written or verbal condolence communication in a format that can be personalized to the provider and the patient's family.