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PurposeEURAMOS-1, an international randomized controlled trial, investigated maintenance therapy with pegylated interferon alfa-2b (IFN-α-2b) in patients whose osteosarcoma showed good histologic response (good response) to induction chemotherapy.Patients and methodsAt diagnosis, patients age ≤ 40 years with resectable high-grade osteosarcoma were registered. Eligibility after surgery for good response random assignment included ≥ two cycles of preoperative MAP (methotrexate, doxorubicin, and cisplatin), macroscopically complete surgery of primary tumor, < 10% viable tumor, and no disease progression. These patients were randomly assigned to four additional cycles MAP with or without IFN-α-2b (0.5 to 1.0 μg/kg per week subcutaneously, after chemotherapy until 2 years postregistration). Outcome measures were event-free survival (EFS; primary) and overall survival and toxicity (secondary).ResultsGood response was reported in 1,041 of 2,260 registered patients; 716 consented to random assignment (MAP, n = 359; MAP plus IFN-α-2b, n = 357), with baseline characteristics balanced by arm. A total of 271 of 357 started IFN-α-2b; 105 stopped early, and 38 continued to receive treatment at data freeze. Refusal and toxicity were the main reasons for never starting IFN-α-2b and for stopping prematurely, respectively. Median IFN-α-2b duration, if started, was 67 weeks. A total of 133 of 268 patients who started IFN-α-2b and provided toxicity information reported grade ≥ 3 toxicity during IFN-α-2b treatment. With median follow-up of 44 months, 3-year EFS for all 716 randomly assigned patients was 76% (95% CI, 72% to 79%); 174 EFS events were reported (MAP, n = 93; MAP plus IFN-α-2b, n = 81). Hazard ratio was 0.83 (95% CI, 0.61 to 1.12; P = .214) from an adjusted Cox model.ConclusionAt the preplanned analysis time, MAP plus IFN-α-2b was not statistically different from MAP alone. A considerable proportion of patients never started IFN-α-2b or stopped prematurely. Long-term follow-up for events and survival continues.

Background: Technetium‐99 (99mTc) lymphoscintigraphy with blue dye injection is an accepted method for sentinel lymph node (SLN) mapping, but blue dye has known adverse effects, and injection of 99mTc may increase time under anesthesia for pediatric patients. Indocyanine green (ICG) may serve as an adjunct to assist with visibility and identification of SLNs. We hypothesized that sensitivity of ICG was similar to blue dye in SLN biopsies. Methods: Thirty patients (36 procedures with 96 total specimens) underwent preoperative intradermal injection of 99mTc, followed by intradermal injection of isosulfan blue and ICG. Test characteristics of blue dye, ICG, and 99mTc included sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). Results: ICG had a sensitivity of 87% and PPV of 83% for detection of 99mTc‐hot lymph nodes; blue dye had a sensitivity of 44% and PPV of 97%. For detection of pathologically confirmed lymph nodes, ICG had a sensitivity of 84% and a positive predictive value (PPV) of 91%. 99mTc had a sensitivity of 82% and a PPV of 94%. ICG had no significant difference in odds of being positive in pathology‐confirmed lymph nodes compared to 99mTc (odds ratio [OR], 0.818; 95% confidence interval [CI], 0.3–2.172; p =.823) and had higher odds than isosulfan blue (OR, 0.025, 95% CI, 0.001–0.148; p <.001). Conclusion: This study established the efficacy of ICG as an adjunct to SLNB in the pediatric and young adult population. ICG was safe, more efficacious than blue dye, and may obviate the need for lymphoscintigraphy in selected patients resulting in reduced time under anesthesia. This trial in the pediatric and young adult oncology population studied patients undergoing sentinel lymph node biopsy with the use of gold‐standard technetium‐99, isosulfan blue, and indocyanine green. Indocyanine green was found to be safe, more efficacious than blue dye, and noninferior to technetium‐99, potentially obviating the need for nuclear scanning in select patient populations, resulting in reduced time under anesthesia. [ABSTRACT FROM AUTHOR]

Background Medulloblastoma is an aggressive central nervous system (CNS) tumor that occurs mostly in the pediatric population. Treatment often includes a combination of surgical resection, craniospinal irradiation (CSI), and chemotherapy. Children who receive standard photon CSI are at risk for cardiac toxicities including coronary artery disease, left ventricular scarring and dysfunction, valvular damage, and atherosclerosis. Current survivorship guidelines recommend routine echocardiogram (ECHO) surveillance. In this multi-institutional study, we describe markers of cardiac dysfunction in medulloblastoma survivors. Methods A retrospective chart review of medulloblastoma patients who had photon beam CSI was followed by ECHO between 1980 and 2010 at Lurie Children's Hospital and Dana-Farber/Boston Children's Hospital. Results During the 30-year study period, 168 medulloblastoma patient records were identified. Included in this study were the 75 patients who received CSI or spinal radiation and ECHO follow-up. The mean age at CSI was 8.6 years (range, 2.9-20), and the mean number of years between radiation therapy (RT) completion and first ECHO was 7.4 (range, 2-16). Mean ejection fraction (EF) was 60.0% and shortening fraction (SF) was 33.8%. Five patients (7%) had abnormal ECHO results: three with EF <50% and two with SF <28%. Conclusion The majority of medulloblastoma patients who received CSI have relatively normal ECHOs post-treatment; however, 7% of patients had abnormal ECHOs. The implication of our study for medulloblastoma survivors is that further investigations are needed in this population with a more systematic, longitudinal assessment to determine predictors and screenings. [ABSTRACT FROM AUTHOR]

Objective: The current study examined the roles of constructive and dysfunctional problem-solving strategies in the relationships between illness uncertainty and adjustment outcomes (i.e., anxious, depressive, and posttraumatic stress symptoms) in caregivers of children newly diagnosed with cancer.Methods: Two hundred thirty-eight caregivers of children (0-19 years of age) newly diagnosed with cancer (2-14 weeks since diagnosis) completed measures of illness uncertainty, problem-solving strategies, and symptoms of anxiety, depression, and posttraumatic stress.Results: A mediation model path analysis assessed constructive and dysfunctional problem-solving strategies as mediators between illness uncertainty and caregiver anxious, depressive, and posttraumatic stress symptoms. Dysfunctional problem-solving scores partially mediated the relationships between illness uncertainty and anxious, depressive, and posttraumatic stress symptoms. Constructive problem-solving scores did not mediate these relationships.Conclusions: The current findings suggest that illness uncertainty and dysfunctional problem-solving strategies, but not constructive problem-solving strategies, may play a key role in the adjustment of caregivers of children newly diagnosed with cancer. Interventions aimed at managing illness uncertainty and mitigating the impact of dysfunctional problem-solving strategies may promote psychological adjustment. [ABSTRACT FROM AUTHOR]

Background: There are minimal data on long-term surgical outcomes of patients who have undergone resection for Wilms tumor (WT) and neuroblastoma (NB).Methods: A retrospective review of patients in a long-term survivor clinic between the years 1967 and 2016 in a pediatric tertiary care hospital (>5 years posttreatment) was performed.Results: Eighty-six survivors of WT and 86 survivors of NB who had ongoing follow-up in the survivors' clinic were identified. The median age at diagnosis was 2.5 years (range, 0.4-15.7 years) with a mean follow-up of 22.3 years (±10.4 years) for WT. The median age at diagnosis for patients with NB was 0.9 years (range, 0.1-8.6 months); mean follow-up of 21.7 years (±7.9 years). Twelve patients with WT (14.0%) had at least 1 repeat laparotomy, 11.1% for bowel obstruction, at a median of 3 months from initial surgery. Twelve patients (14.0%) with NB required laparotomy and 8.1% for bowel obstruction, at a median of 12 years after initial surgery. The incidence of hypertension in patients with WT who had undergone nephrectomy was not outside of population norms. Patients who underwent thoracotomy for a NB have a higher incidence of scoliosis and Horner syndrome.Conclusions: Small bowel obstruction requiring laparotomy is significantly higher than the literature norms for both tumor patient populations and typically occurs in the early postoperative period for patients with WT and remotely in patients with NB. The long-term surgical complications of patients who underwent resection for NB and WT clearly merit follow-up and patient education within multidisciplinary long-term survivorship clinics. [ABSTRACT FROM AUTHOR]

Introduction: Objectives were used to describe guardian proxy‐report and child self‐report quality of life (QoL) during chemotherapy for pediatric acute myeloid leukemia (AML) patients. Methods: Patients enrolled on the phase 3 AML trial AAML1031 who were 2‐18 years of age with English‐speaking guardians were eligible. Instruments used were the PedsQL Generic Core Scales, Acute Cancer Module, and Multidimensional Fatigue Scale. Assessments were obtained at the beginning of Induction 1 and following completion of cycles 2‐4. Potential predictors of QoL included the total number of nonhematological grade 3‐4 Common Terminology Criteria for Adverse Event (CTCAE) submissions. Results: There were 505 eligible guardians who consented to participate and 348 of their children provided at least one self‐report assessment. The number of submitted CTCAE toxicities was significantly associated with worse physical health summary scores (β ± standard error (SE) −3.00 ± 0.69; P < 0.001) and general fatigue (β ± SE −2.50 ± 0.66; P < 0.001). Older age was significantly associated with more fatigue (β ± SE −0.58 ± 0.25; P = 0.022). Gender, white race, Hispanic ethnicity, private insurance status, risk status, bortezomib assignment, and duration of neutropenia were not significantly associated with QoL. Discussion: The number of CTCAE toxicities was the primary factor influencing QoL among children with AML. Reducing toxicities should improve QoL; identifying approaches to ameliorate them should be a priority. [ABSTRACT FROM AUTHOR]

Background: Survivors of Hodgkin lymphoma (HL) in childhood have an increased risk of subsequent malignant neoplasms (SMNs). Herein, the authors extended the follow-up of a previously reported Late Effects Study Group cohort and identified patients at highest risk for SMNs to create evidence for risk-based screening recommendations.Methods: The standardized incidence ratio was calculated using rates from the Surveillance, Epidemiology, and End Results program as a reference. The risk of SMN was estimated using proportional subdistribution hazards regression. The cohort included 1136 patients who were diagnosed with HL before age 17 years between 1955 and 1986. The median length of follow-up was 26.6 years.Results: In 162 patients, a total of 196 solid SMNs (sSMNs) were identified. Compared with the general population, the cohort was found to be at a 14-fold increased risk of developing an sSMN (95% confidence interval, 12.0-fold to 16.3-fold). The cumulative incidence of any sSMN was 26.4% at 40 years after a diagnosis of HL. Risk factors for breast cancer among females were an HL diagnosis between ages 10 years and 16 years and receipt of chest radiotherapy. Males treated with chest radiotherapy at age <10 years were found to be at highest risk of developing lung cancer. Survivors of HL who were treated with abdominal/pelvic radiotherapy and high-dose alkylating agents were found to be at highest risk of developing colorectal cancer and females exposed to neck radiotherapy at age <10 years were at highest risk of thyroid cancer. By age 50 years, the cumulative incidence of breast, lung, colorectal, and thyroid cancer was 45.3%, 4.2%, 9.5%, and 17.3%, respectively, among those at highest risk.Conclusions: Survivors of childhood HL remain at an increased risk of developing sSMNs. In the current study, subgroups of survivors of HL at highest risk of specific sSMNs were identified, and evidence for screening provided. [ABSTRACT FROM AUTHOR]

Adolescent and young adult (AYA) sarcoma patients do not fare as well as their younger counterparts. A variety of factors theorized to underlie such disparate outcomes have been identified including distinct tumor and host biology. Compared to younger patients, AYA patients often develop genetically distinct tumors and are more likely to suffer characteristic therapy-related toxicities. Compounding factors faced by AYA patients include education, finances, employment, and obstacles to treatment adherence. Lack of clinical trial participation among AYA patients has slowed the establishment of optimized age-specific treatment protocols and hindered the collection of biospecimens for scientific investigation. The relative rarity of sarcomas among adult cancers may limit the familiarity of oncologists with state of the art diagnostic and therapeutic aspects of sarcomas in young adults. Among other interventions, improved enrollment on clinical trials is a critical step in addressing the challenges faced by AYA patients. Further insight into unique tumor and host biology among AYA patients is also an important need. [ABSTRACT FROM AUTHOR]

Background: Human flora are the most common cause of bacteremia in immunocompromised patients. Activities of daily living (ADL), including oral care and daily chlorhexidine gluconate bathing, can lower the risk of infection. Methods: To address ADL compliance in our pediatric oncology and bone marrow transplant patients, we adopted the ADL 1-2-3 initiative: daily chlorhexidine gluconate bath and linen change, at least 2 activities per day, and oral care 3 times per day. Using the Model for Improvement we created a standardized ADL process that involved all providers. Interventions included addressing ADL 1-2-3 compliance during rounds, establishing accountability in care delivery, an oral care order set and algorithm, daily text message reminders, and physician intervention with noncompliant and high-risk patients. Results: With our interventions, we increased our median compliance with the all-or-none ADL 1-2-3 initiative from 25% to 66% in 90 days. We have sustained our median compliance to 75% sixteen months after implementation. The greatest impact on compliance was seen with text message reminders to staff to complete and document the ADL 1-2-3 components, designated roles and responsibilities, and physician discussion with noncompliant and high-risk patients. Discussion: Oral care algorithm and order set, daily text message reminders, and physician intervention with noncompliant and high-risk patients has improved our compliance. Units where compliance with ADL participation is low can benefit from incorporating elements from this ADL 1-2-3 initiative. [ABSTRACT FROM AUTHOR]

A case study is presented of a 12-year-old female presented to the emergency department with abdominal pain and also reported increasing thirst, polyuria, nausea, and sleepiness over the same period. It mentions that tumor markers for ovarian germ cell, sex cord-stromal, and epithelial tumors were negative. It mentions that final diagnosis revealed hypercalcemia due to small-cell carcinoma of the ovary hypercalcemic type (SCCOHT) of the right ovary.

Purpose: Health related quality of life (HRQL) assessments during therapy for pediatric cancer provide valuable information to better understand the patient experience. Our objective was to determine the impact of a patient-reported outcome (PRO) coordinator on HRQL questionnaire completion rates during a pediatric acute myeloid leukemia (AML) trial. Methods: AAML1031 is a multicenter Children’s Oncology Group therapeutic trial for de novo AML with a secondary aim to assess HRQL of children and adolescents treated with chemotherapy and hematopoietic stem cell transplantation (HSCT). Parents/guardians are the primary respondents and four questionnaires are administered at eight time points. The questionnaires are the PedsQL 4.0 Generic Core Scales, PedsQL 3.0 Acute Cancer Module, PedsQL Multidimensional Fatigue Scale, and the Pediatric Inventory for Parents. To improve response rates, a central PRO coordinator was instituted and reminded sites about upcoming and delinquent questionnaires. The proportion of HRQL questionnaires completed were compared prior to, and following institution of the PRO coordinator. This analysis evaluated the first five assessment time points. Results: There were231 families who consented to participate in the HRQL aim. Overall response rates for all questionnaires were 73–83%. At time point 1, within 14 days of chemotherapy initiation, post-PRO coordinator completion rates were significantly higher for three of four questionnaires. However, the effect was not sustained and at time point 4, one month following last chemotherapy or HSCT, completion rates were significantly lower post-PRO coordinator for all four questionnaires. Conclusion: Addition of a central PRO coordinator did not result in sustained improvement in HRQL questionnaire completion rates. Efforts to improve response rates must consider other strategies. [ABSTRACT FROM AUTHOR]

Background A large cohort of Hodgkin lymphoma (HL) survivors exist. With patients transitioning from pediatric to adult care, practitioners should be aware of potential complications. The aim of this study was to describe the long-term complications of patients who had staging laparotomy for the treatment of HL. Methods After institutional review board approval, a retrospective review of hospital records at our institution was performed. Data extracted included demographics, treatment course and long-term postoperative complications. Results 24 patients with HL underwent staging laparotomy from 1971 to 1994 with median follow-up of 27.9 years. Six (33%) had intraabdominal disease. Three patients (17%) required four repeat laparotomies for bowel obstruction. Of these, one had radiation to the inguinal region for local control, one had mantle radiation. Five patients developed a second malignancy. There were no documented cases of postsplenectomy sepsis. Other late effects that were unlikely related to surgery included pulmonary fibrosis (4), heart failure (2), hypothyroidism (4), and dysphagia (3). One patient died of metastatic adenocarcinoma. Conclusions Long-term follow-up of patients who underwent staging laparotomy for HL revealed an increased incidence of repeat laparotomy and secondary malignancy. This underscores the importance of a high index of suspicion and screening in this population. Level of Evidence Level III. [ABSTRACT FROM AUTHOR]

Background: Health related quality of life (HRQL) assessments during therapy for pediatric cancer are important. The objective of this study was to describe reasons for failure to provide HRQL assessments during a pediatric acute myeloid leukemia (AML) clinical trial. Methods: We focused on HRQL assessments embedded in a multicenter pediatric AML clinical trial. The PedsQL 4.0 Generic Core Scales, PedsQL 3.0 Acute Cancer Module, PedsQL Multidimensional Fatigue Scale, and Pediatric Inventory for Parents were obtained from parent/guardian respondents at a maximum of six time points. Children provided self-report optionally. A central study coordinator contacted sites with delinquent HRQL data. Reasons for failure to submit the HRQL assessments were evaluated by three pediatric oncologists and themes were generated using thematic analysis. Results: There were 906 completed and 1091 potential assessments included in this analysis (83%). The median age of included children was 12.9 years (range 2.0 to 18.9). The five themes for non-completion were: patient too ill; passive or active refusal by respondent; developmental delay; logistical challenges; and poor knowledge of study processes from both the respondent and institutional perspective. Conclusions: We identified reasons for non-completion of HRQL assessments during active therapy. This information will facilitate recommendations to improve study processes and future HRQL study designs to maximize response rates. [ABSTRACT FROM AUTHOR]

Abstract: Posterior mediastinal masses have widely variant presentations and a broad differential diagnosis. We describe an adolescent boy presenting with a posterior mediastinal mass, associated with abdominal pain and pleural effusion. Radiologic, operative, and pathologic findings are reviewed. This patient was found to have torsion of an extralobar pulmonary sequestration, of which only 2 other cases have been reported in the literature. Although radiologic findings of a systemic arterial supply to the mass may be absent, with patients presenting with this constellation of symptoms, a high index of suspicion of a sequestration should be maintained. [Copyright &y& Elsevier]

Objective: To determine the diagnostic yield versus complications from endoscopy in children after hematopoietic stem cell transplantation (HSCT). Design: Data from 191 patients were reviewed, then separated into procedures within the first 100 days after HSCT (when the risks for complications and prevalence of acute graft-versus-host disease [GVHD] are highest) and those performed beyond 100 days. Results: Visible endoscopic lesions were found in 63 of 198 (32%) esophagogastroduodenoscopies (EGDs) and 36 of 220 (16%) sigmoidoscopies. Acute GVHD was present in 38 of 121 (31%) biopsy specimens from EGDs within the first 100 days, 15 of 73 (21%) samples from EGDs after 100 days, 52 of 136 (38%) sigmoid biopsy specimens before 100 days, and in 25 of 82 (31%) samples after 100 days. Non-GVHD histologic abnormalities were present in 36 of 124 (29%) biopsy samples from EGDs before 100 days, 32 of 74 (43%) specimens after 100 days, 14 of 136 (10%) sigmoid specimens before 100 days, and 11 of 84 (13%) samples beyond 100 days. Complications: Complications occurred in 13 procedures (3.1%): 8 (4.2%) EGDs, 4 (2.0%) sigmoidoscopies, and 1 (5.5%) colonoscopy. Intestinal bleeding occurred in 12 of the 13 procedures. Thrombocytopenia was a statistically significant association (p < 0.01). One death occurred after splenic flexure perforation. Conclusions: GI abnormalities other than acute GVHD occurred in children after HSCT. Acute GVHD was diagnosed most commonly on sigmoid biopsy. Postprocedure hemorrhage was related to thrombocytopenia. [Copyright &y& Elsevier]