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Background: Although pediatric lower extremity sarcoma once was routinely treated with amputation, multiagent chemotherapy as well as the evolution of tumor resection and reconstruction techniques have enabled the wide adoption of limb salvage surgery (LSS). Even though infection and tumor recurrence are established risk factors for early amputation (< 5 years) after LSS, the frequency of and factors associated with late amputation (≥ 5 years from diagnosis) in children with sarcomas are not known. Additionally, the resulting psychosocial and physical outcomes of these patients compared with those treated with primary amputation or LSS that was not complicated by subsequent amputation are not well studied. Studying these outcomes is critical to enhancing the quality of life of patients with sarcomas. Questions/purposes: (1) How have treatments changed over time in patients with lower extremity sarcoma who are included in the Childhood Cancer Survivor Study (CCSS), and did primary treatment with amputation or LSS affect overall survival at 25 years among patients who had survived at least 5 years from diagnosis? (2) What is the cumulative incidence of amputation after LSS for patients diagnosed with pediatric lower extremity sarcomas 25 years after diagnosis? (3) What are the factors associated with time to late amputation (≥ 5 years after diagnosis) in patients initially treated with LSS for lower extremity sarcomas in the CCSS? (4) What are the comparative social, physical, and emotional health-related quality of life (HRQOL) outcomes among patients with sarcoma treated with primary amputation, LSS without amputation, or LSS complicated by late amputation, as assessed by CCSS follow-up questionnaires, the SF-36, and the Brief Symptom Inventory-18 at 20 years after cancer diagnosis? Methods: The CCSS is a long-term follow-up study that began in 1994 and is coordinated through St. Jude Children's Research Hospital. It is a retrospective study with longitudinal follow-up of more than 38,000 participants treated for childhood cancer when younger than 21 years at one of 31 collaborating institutions between 1970 and 1999 in the United States and Canada. Participants were eligible for enrollment in the CCSS after they had survived 5 years from diagnosis. Within the CCSS cohort, we included participants who had a diagnosis of lower extremity sarcoma treated with primary amputation (547 patients with a mean age at diagnosis of 13 ± 4 years) or primary LSS (510 patients with a mean age 14 ± 4 years). The LSS cohort was subdivided into LSS without amputation, defined as primary LSS without amputation at the time of latest follow-up; LSS with early amputation, defined as LSS complicated by amputation occurring less than 5 years from diagnosis; or LSS with late amputation, defined as primary LSS in study patients who subsequently underwent amputation 5 years or more from cancer diagnosis. The cumulative incidence of late amputation after primary LSS was estimated. Cox proportional hazards regression with time-varying covariates identified factors associated with late amputation. Modified Poisson regression models were used to compare psychosocial, physical, and HRQOL outcomes among patients treated with primary amputation, LSS without amputation, or LSS complicated by late amputation using validated surveys. Results: More study participants were treated with LSS than with primary amputation in more recent decades. The overall survival at 25 years in this population who survived 5 years from diagnosis was not different between those treated with primary amputation (87% [95% confidence interval [CI] 82% to 91%]) compared with LSS (88% [95% CI 85% to 91%]; p = 0.31). The cumulative incidence of amputation at 25 years after cancer diagnosis and primary LSS was 18% (95% CI 14% to 21%). With the numbers available, the cumulative incidence of late amputation was not different among study patients treated in the 1970s (27% [95% CI 15% to 38%]) versus the 1980s and 1990s (19% [95% CI 13% to 25%] and 15% [95% CI 10% to 19%], respectively; p = 0.15). After controlling for gender, medical and surgical treatment variables, cancer recurrence, and chronic health conditions, gender (hazard ratio [HR] 2.02 [95% CI 1.07 to 3.82]; p = 0.03) and history of prosthetic joint reconstruction (HR 2.58 [95% CI 1.37 to 4.84]; p = 0.003) were associated with an increased likelihood of late amputation. Study patients treated with a primary amputation (relative risk [RR] 2.04 [95% CI 1.15 to 3.64]) and LSS complicated by late amputation (relative risk [RR] 3.85 [95% CI 1.66 to 8.92]) were more likely to be unemployed or unable to attend school than patients treated with LSS without amputation to date. The CCSS cohort treated with primary amputation and those with LSS complicated by late amputation reported worse physical health scores than those without amputation to date, although mental and emotional health outcomes did not differ between the groups. Conclusion: There is a substantial risk of late amputation after LSS, and both primary and late amputation status are associated with decreased physical HRQOL outcomes. Children treated for sarcoma who survive into adulthood after primary amputation and those who undergo late amputation after LSS may benefit from interventions focused on improving physical function and reaching educational and employment milestones. Efforts to improve the physical function of people who have undergone amputation either through prosthetic design or integration into the residuum should be supported. Understanding factors associated with late amputation in the setting of more modern surgical approaches and implants will help surgeons more effectively manage patient expectations and adjust practice to mitigate these risks over the life of the patient. Level of Evidence: Level III, therapeutic study. [ABSTRACT FROM AUTHOR]

Fatigue, rated by adolescents as the most distressing symptom experienced during cancer treatment, is essential to the successful clinical care of patients of every culture and nationality. Efforts to provide relief from such symptom, in any area of the world, have been hampered by the lack of reliable and valid instruments used to measure fatigue. Our aims were to examine the semantic, conceptual, and normative equivalence of the Chinese version of the Fatigue Scale-Adolescent (FS-A-C) to the original Fatigue Scale-Adolescent (FS-A) and to estimate the reliability and validity of the FS-A-C. We recruited 51 Taiwanese adolescents in various stages of different types of cancer in this cross-sectional study. Results indicated that the initial panel estimates of semantic, conceptual, and normative equivalence of the FS-A-C with the original instrument (FS-A) were positive. The FS-A-C had acceptable internal consistency (Cronbach alpha =.89) and moderate-to-high content validity (content validity index ranges from 87% to 100%). In addition, the FS-A-C achieved known-groups validity (anemic adolescents reporting higher fatigue than nonanemic adolescents do) and initial construct validity (a significant association between the FS-A-C and the Anxious/Depressed subscale). Its use in measuring the intensity of fatigue in adolescents is likely to yield accurate assessments of their fatigue that could prompt clinical efforts to relieve their fatigue-related distress.

Background: Adenovirus (ADV) infections are associated with significant morbidity and mortality after hematopoietic stem cell transplantation (HSCT). The virus is endemic in the general pediatric population and frequently causes severe disease in immunocompromised patients, especially children. We report our experience with cidofovir (CDV) for treatment of ADV infection in 57 HSCT patients, median age 8 years (range 0.5-26)., Methods: Peripheral blood was prospectively screened weekly on all patients for ADV by quantitative real-time PCR for the first 100 days post-HSCT or longer if clinically indicated. Cultures for viral pathogens were performed from other involved sites. Upon detection of ADV by PCR, culture or tissue histopathology, CDV was given intravenously at 5 mg/kg weekly for 2 consecutive weeks, then every 2 weeks until 3 consecutive ADV-negative samples were documented from all previously invoved sites., Results: The clinical manifestations of ADV infection were: diarrhea (53%), fever (21%), hemorrhagic cystitis (12%), and pneumonitis (11%). Eight patients (14%) presented with disseminated disease. CDV treatment resulted in complete resolution of clinical symptoms in 56 (98%) patients in whom the virus became undetectable by all methods. One patient died due to ADV pneumonitis. No cases of dose-limiting nephrotoxicity were observed. CONCLUSIONS. Cidofovir appeared safe and effective for the treatment of ADV infection in this predominantly pediatric HSCT population. Vigilant surveillance and early treatment with CDV can prevent the poor outcomes associated with ADV disease. A larger prospective study is needed to further determine the role of CDV in the treatment of ADV after HSCT.

Purpose: To determine the incidence, etiology and outcome of graft failure in pediatric allogeneic bone marrow transplant (BMT) recipients., Patients and Methods: Patients with primary or secondary graft failure were identified by database review. A retrospective chart review was performed. Etiologic factors were identified and assessed for statistical significance., Results: 309 children underwent allogeneic BMT during the time interval studied. Four cases of primary graft failure and 7 cases of secondary graft failure occurred. Nonmalignant diagnosis, lower total nucleated cell (TNC) dose, and conditioning without total body irradiation were associated with a higher incidence of graft failure. Donor source, donor/recipient CMV status, CD34+ cell dose, and alloimmunization were not associated with graft failure., Conclusions: Graft failure is a relatively uncommon occurrence in pediatric patients. Autologous reinfusion may allow time to prepare the patient for a second transplant and decrease complications associated with aplasia. More immunosuppressive conditioning regimens may decrease the incidence of graft failure, particularly in patients with non-malignant diseases or those with lower stem cell doses. More frequent monitoring of chimerism by VNTR analysis may detect late graft failure earlier and allow for more rapid intervention.

Purpose: Treatment of acute myeloid leukemia (AML) is associated with substantial adverse effects, including neutropenia and infection. Viridans streptococci (VS) are a primary cause of infection and pneumonia in patients with neutropenia. The authors determined the incidence, clinical features, and complications of VS sepsis in children receiving chemotherapy for AML., Methods: The authors retrospectively reviewed the records of 172 patients treated on their institutional protocols AML91 (n = 95) and AML97 (n = 77) and identified 36 patients who had VS sepsis., Results: The 1-year cumulative incidence of VS sepsis was significantly higher in AML97 than in AML91. Patients with favorable cytogenetic features (ie, t(9;11), t(8;21), or inv(16)) had a significantly higher incidence of infection than did other patients. VS sepsis developed at various times after chemotherapy was initiated, and patients remained febrile for a median of 15 days. Twelve patients (33%) experienced hypotension, 10 (28%) acute respiratory distress syndrome, and 6 (17%) fungal infection. Twenty-three patients (64%) required intensive care, 21 (58%), oxygen therapy, and 7 (19%), vasopressor medications. One patient died of pulmonary aspergillosis after VS sepsis. The 3-year cumulative incidence of aspergillosis was higher in patients with VS sepsis than in those without., Conclusions: Although antibiotic therapy rapidly resolved VS sepsis, complications associated with this infection remained life-threatening in children receiving chemotherapy for AML.

The purpose of this study was to develop and test the Role-Related Meaning Scale for Staff in Pediatric Oncology (RRMS) to determine the internal consistency and the content and construct validity of this two-phase instrument. During phase 1 (item generation, content validation, and initial field testing), 23 nurses from two cancer centers participated, and during phase 2 (instrument testing), 89 nurses from one pediatric research center participated. The nurses completed either the RRMS only (phase 1) or six instruments including the RRMS (phase 2) to assess the following research variables: role-related meaning, group cohesion, organizational commitment, work satisfaction, and intent to leave. The RRMS was revised after phase 1 because the results yielded a ceiling effect and three overlapping items. The Cronbach alpha for the phase 2 total RRMS was.83, and four of the five hypothesized relations were confirmed (P =.04). Therefore, the RRMS was concluded to be an internally consistent instrument that has content validity and beginning construct validity. Future studies will examine whether the RRMS adequately measures the change in meaning brought about by interventions designed to increase role-related meaning among nurses.