Showing papers by "Sue C. Kaste published in 2008"

The ‘Image Gently’ campaign: increasing CT radiation dose awareness through a national education and awareness program

Abstract: ALARA (As Low As Reasonably Achievable) has been a guiding principle for pediatric radiologists for decades. The Society for Pediatric Radiology (SPR) has long been a leader in promoting safety in radiology practice in children. However, the ALARA principle has taken on new meaning in the past several years as the number of CT scans in children has skyrocketed. For example, it is estimated that since the 1980s when CT was beginning its ascendancy there has been up to an 800% increase. CT scans in children provide great benefit in patient care when used appropriately. However, increased use requires a team approach to ensure that only indicated exams are performed and at the Pediatr Radiol (2008) 38:265–269 DOI 10.1007/s00247-007-0743-3

Bone mineral density deficits in survivors of childhood cancer: long-term follow-up guidelines and review of the literature.

Abstract: The development of curative therapy for most pediatric malignancies has produced a growing population of childhood cancer survivors who are at increased risk for a variety of health problems resulting from their cancer or its treatment. Because of the fact that many treatment-related sequelae may not become clinically apparent until the survivor attains maturity or begins to age, the ability of primary care providers to anticipate late effects of treatment is essential for providing timely interventions that prevent or correct these sequelae and their adverse effects on quality of life. Altered bone metabolism during treatment for childhood cancer may interfere with attainment of peak bone mass, potentially predisposing to premature onset of and more severe complications related to osteopenia and osteoporosis. Bone mineral deficits have been reported after treatment for a variety of pediatric malignancies and represent morbidity that can be reduced or prevented through lifestyle changes and attention to other common cancer-related sequelae such as hypogonadism. The Children's Oncology Group long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers provide risk-based surveillance recommendations that are based on expert opinion and review of the scientific literature for potential late effects of pediatric cancer therapy including osteopenia. This review summarizes the existing literature that has defined characteristics of cancer survivors at risk for bone mineral deficits and contributed to the surveillance and counseling recommendations outlined in the Children's Oncology group long-term follow-up guidelines.

Wilms tumour: prognostic factors, staging, therapy and late effects

Abstract: Wilms tumour is the most common malignant renal tumour in children. Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy. Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Societe Internationale d'Oncologie Pediatrique (SIOP) and the Children's Oncology Group (COG). The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy. The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery. This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy. Surgery remains a crucial part of treatment for nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrectomy, when technically feasible, seems reasonable not only in those with bilateral disease but also in those with unilateral disease where the patient has urological disorders or syndromes predisposing to malignancy. Partial nephrectomy, however, is frequently not sufficient for an anaplastic variant of tumour. The late effects for Wilms tumour and its treatment are also reviewed. The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experiencing serious morbidity at 20 years from diagnosis. The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour. Continual international trial development and participation will improve matching of treatment needs with prognosis, reducing long-term complications in the majority. The advent of molecular markers of disease severity and improved functional imaging might help.

Osteonecrosis in Adult Survivors of Childhood Cancer: A Report From the Childhood Cancer Survivor Study

Abstract: Purpose Osteonecrosis (ON) is a potentially serious complication of therapy in survivors of childhood cancer. Our goals were to describe the incidence of ON and identify patient and treatment characteristics associated with elevated risk. Patients and Methods The rate of self-reported ON was determined for 9,261 patients enrolled onto the Childhood Cancer Survivor Study, a cohort of 5-year survivors of childhood cancer diagnosed from 1970 to 1986, and compared with the rate in a random sample of 2,872 siblings of survivors. Survivors with positive responses were reinterviewed to confirm the diagnosis. Results Fifty-two cancer survivors reported ON in 78 joints, yielding 20-year cumulative incidence of 0.43% and a rate ratio (RR) of 6.2 (95% CI, 2.3 to 17.2) compared with siblings, adjusted for age and sex; 44% developed ON in a previous radiation field. The RR was greatest among survivors of stem-cell transplantation for acute lymphoblastic leukemia (ALL), acute myelogenous leukemia (AML), and chronic mye...

Ultrasonic characterization of human cancellous bone in vitro using three different apparent backscatter parameters in the frequency range 0.6-15.0 mhz

Abstract: Ultrasonic techniques based on measurements of apparent backscatter may provide a useful means for diagnosing bone diseases such as osteoporosis. The term "apparent" means that the backscattered signals are not compensated for the frequency-dependent effects of attenuation and diffraction. We performed in vitro apparent backscatter measurements on 23 specimens of human cancellous bone prepared from the left and right femoral heads of seven donors. A mechanical scanning system was used to obtain backscattered signals from each specimen at several sites. Scans were performed using five different ultrasonic transducers with center frequencies of 1, 2.25, 5, 7.5, and 10 MHz. The -6 dB bandwidths of these transducers covered a frequency range of 0.6-15.0 MHz. The backscattered signals were analyzed to determine three ultrasonic parameters: apparent integrated backscatter (AIB), frequency slope of apparent backscatter (FSAB), and time slope of apparent backscatter (TSAB). Linear regression analysis was used to examine the correlation of these ultrasonic parameters with five measured physical characteristics of the specimens: mass density, X-ray bone mineral density, Young's modulus, yield strength, and ultimate strength. A total of 75 such correlations were examined (3 ultrasonic parameters times 5 specimen characteristics times 5 transducers). Good correlations were observed for AIB using the 5 MHz (r = 0.70 - 0.89) and 7.5 MHz (r = 0.75-0.93) transducers; for FSAB using the 2.25 MHz (r = 0.70 - 0.88), 5 MHz (r = 0.79 - 0.94), and 7.5 MHz (r = 0.80 - 0.92) transducers; and for TSAB using the 5 MHz (r = 0.68 - 0.89), 7.5 MHz (r = 0.75 - 0.89), and 10 MHz (r = 0.75 - 0.92) transducers.

Image GentlySM: A National Education and Communication Campaign in Radiology Using the Science of Social Marketing

Abstract: Communication campaigns are an accepted method for altering societal attitudes, increasing knowledge, and achieving social and behavioral change particularly within public health and the social sciences. The Image Gently SM campaign is a national education and awareness campaign in radiology designed to promote the need for and opportunities to decrease radiation to children when CT scans are indicated. In this article, the relatively new science of social marketing is reviewed and the theoretical basis for an effective communication campaign in radiology is discussed. Communication strategies are considered and the type of outcomes that should be measured are reviewed. This methodology has demonstrated that simple, straightforward safety messages on radiation protection targeted to medical professionals throughout the radiology community, utilizing multiple media, can affect awareness potentially leading to change in practice.

Imaging in unilateral Wilms tumour.

Abstract: Wilms tumour is one of the most common malignancies in children, with an excellent prognosis after therapy. There is a very diverse approach to treatment according to geographical location. This variation in therapeutic attitude toward Wilms tumour, particularly between the United States and Europe, has consequences for the choice of imaging modality at diagnosis. In Europe, the International Society of Paediatric Oncology (SIOP) treatment protocol is based on chemotherapy followed by surgery. Imaging (US, CT and MRI), clinical history and examination will help predict whether the findings are consistent with Wilms tumour. Furthermore, in the UK preoperative image-guided biopsy is advised to help identify the small group of patients who, despite typical imaging features of Wilms tumour, have other types of neoplasia that require alternative management. In the United States, the National Wilms Tumor Study (NWTS) advises surgery prior to chemo- and radiotherapy. Hence imaging must provide detailed anatomical information for surgical planning. This article discusses the role of imaging at diagnosis and the relative strengths and weaknesses of the available radiological techniques. We also focus on imaging the lung for metastatic disease and the consequences (to the patient’s ultimate outcome) of CT-diagnosed small pulmonary nodules and discuss the radiological diagnosis and consequences of tumour rupture present at diagnosis.

CRHR1 Polymorphisms Predict Bone Density in Survivors of Acute Lymphoblastic Leukemia

Abstract: Purpose Corticosteroids are a critical component of therapy for acute lymphoblastic leukemia (ALL) but are associated with late effects, such as osteoporosis. Risk factors remain poorly defined. Because CRHR1 polymorphisms have been associated with other corticosteroid effects, our goal was to define whether CRHR1 polymorphisms predict which patients with ALL are likely to develop bone mineral deficits. Patients and Methods The mean bone mineral density z scores of 309 long-term survivors of ALL were determined by quantitative computed tomography of the trabecular lumbar spine. We analyzed whether CRHR1 genotypes, adjusted for sex, ALL treatment regimen, and weight, could predict bone density. Results We found that three single nucleotide polymorphisms (SNPs), all in linkage disequilibrium, were associated with bone density in a sex-specific manner. Bone density was lower in males (P = .001), in nonblack patients (P < .08), in those who were not overweight (P < .001), and in those who received intensive a...

Relationships among severity of osteonecrosis, pain, range of motion, and functional mobility in children, adolescents, and young adults with acute lymphoblastic leukemia.

Abstract: Background and Purpose: Up to 38% of children receiving treatment for acute lymphoblastic leukemia (ALL) develop osteonecrosis, often without symptoms. Little is known about the association between the degree of osteonecrosis and functional mobility in this population. The purpose of this study was to examine relationships among the degree of osteonecrosis, pain, range of motion (ROM), and functional mobility in people with ALL. Subjects: Thirty-three subjects aged 5 to 27 years with ALL and osteonecrosis participated. Methods: The extent of osteonecrosis was determined by magnetic resonance imaging (MRI) of the hip and knee according to 2 classification systems, including the Association Research Circulation Osseous (ARCO) and a knee staging scale. Pain, hip and knee ROM, and the Timed Up and Down Stairs (TUDS) Test were used as measures. Results: Correlations were observed between ARCO and hip pain ( r =.34), between hip flexion ROM and hip pain ( r =−.34), and between knee pain and time on the TUDS Test ( r =−.35). Discussion and Conclusion: Physical therapists should consider that people with ALL may have hip or knee osteonecrosis without clinical symptoms. This notion supports the need for MRI in addition to a comprehensive examination of functional mobility.

Bone mineral density deficits in pediatric patients treated for sarcoma.

Abstract: Background Children treated for sarcoma are at risk of treatment-associated deficits in bone mineral density (BMD). We investigated the severity of risk factors for BMD deficits in this patient population. Procedure Using signed-rank test and logistic regression analysis, we retrospectively analyzed the relation of treatment variables and other potential risk factors to BMD (using quantitative computed tomography (QCT)) of 99 patients treated for pediatric sarcoma who had completed therapy at least 1 year previously. Results The study group (38% rhabdomyosarcoma (RMS), 25% osteosarcoma (OS), 24% Ewing-family tumors, and 12% non-rhabdomyosarcoma soft-tissue sarcoma (NRSTS)) represented 22% of the sarcoma survivors treated between 1982 and 2003 who remained in follow-up at St. Jude. These patients underwent QCT between July 1, 1997 and February 5, 2003. Their median age was 8.7 years (range, 0.2–21.3 years) at diagnosis and 17.4 years (range, 3.3–30.2 years) at the time of BMD measurement; 58% were male and 82% Caucasian. Median BMD Z-score was −0.75 (range, −3.33–3.02), and median BMD was 168.0 mg/cc (range, 89.2–264.8 mg/cc). Risk of BMD deficit increased significantly with younger age at diagnosis (P = 0.044) and higher cumulative cyclophosphamide dose (P = 0.007). Patients with lower extremity primary disease had a significantly lower risk of BMD deficits than others. We found no association between BMD and body habitus, primary disease, lifestyle factors, or endocrinopathy. Conclusion A significant subset of sarcoma survivors are at risk of BMD deficits warranting prospective study of BMD to verify our results and refine risk factors contributing to BMD deficits. Pediatr Blood Cancer 2008;50:1032–1038. © 2007 Wiley-Liss, Inc.

Skeletal toxicities of treatment in children with cancer.

Abstract: For many childhood cancer survivors, the future will hold years of pain and disability due to skeletal toxicities resulting from disease, its treatment and predisposing familial factors that contribute to the development of bone mineral density (BMD) deficits and osteonecrosis. Some technical limitations of imaging methods designed to assess BMD in skeletally mature adult patients are amplified when used to assess bone density in growing children and adolescents. Identifying changes in bone marrow that are associated with disease or treatment, and differentiating these that may heal in comparison to those likely to progress, have not been studied extensively in children. Until recently, little emphasis has been placed on the definition, characterization and prospective monitoring of these two major skeletal toxicities in pediatric patients, despite the growing attention being paid to the prevalence and severity of osteoporosis in adults. Further, the disease definitions established for adults are unlikely to apply to growing children. This presentation will discuss many of the issues related to defining, identifying, and classifying deficits in BMD and osteonecrosis, and will likely raise more issues than it answers.

Comparison of gallium and PET scans at diagnosis and follow-up of pediatric patients with Hodgkin lymphoma.

Abstract: Background Positron emission tomography (PET) and gallium scans facilitate diagnosis and staging, evaluation of response to therapy, and monitoring for relapse in Hodgkin lymphoma (HL), but have not been compared in pediatric HL. Procedure We performed concurrent PET and gallium scans on 44 pediatric HL patients at diagnosis, early response, off chemotherapy, and off-therapy evaluations. PET and gallium scans were compared to each other and to computed tomography (CT) alone to determine whether either modality led to a change in stage or modified the results of the early response evaluation, which was used to determine the radiation dose. Results PET upstaged four patients at diagnosis (2 from stage I to II, one II to III, and one III to IV), but did not lead to a change in therapy in any of them. It changed response category in two patients at early response evaluation, leading to a change in radiation dose for 1 patient (25.5 Gy instead of 15 Gy to the spleen). Gallium did not change the stage of treatment for any patient. The negative predictive values for eventual lymphoma relapse of PET and gallium scans at off therapy were 89% and 83%, respectively; the positive predictive value of PET at off therapy is 29%. Conclusion PET appears to be superior to gallium in pediatric HL; future studies will determine the optimal timing of PET to assess early response and the utility of quantitative interpretation of the avidity of specific nodal sites. Pediatr Blood Cancer 2008;51:198–203. © 2008 Wiley-Liss, Inc.

Airway evaluation and management in 7 children with malignant infantile osteopetrosis before hematopoietic stem cell transplantation.

Abstract: Malignant infantile osteopetrosis (MIOP) is a rare disorder caused by dysfunctional osteoclasts. The classic MIOP features, such as frontal bossing, micrognathia, and small thorax, may place these children at risk for developing obstructive sleep apnea (OSA) and chronic hypoxemia. To objectively document OSA, airway evaluations were performed; results impacted management. We reviewed the records of 7 MIOP patients treated at St Jude. Six underwent polysomnograms during prehematopoietic stem cell transplantation (HSCT) evaluation. To determine the existence of a relationship between OSA and radiologic imaging, initial chest radiographs and bone mineral density studies were reviewed. Pre-HSCT patients had a median apnea-hypopnea index of 17.51 (normal, 0 to 2), with <25% being central events, thus indicating OSA. The median minimal oxygen saturation was 79%, indicating intermittent hypoxemia. Neither chest radiographs nor bone mineral density correlated with severity of OSA. Four patients received tracheostomies before or during HSCT. Three surviving children underwent polysomnograms 1 year after HSCT, and median apnea-hypopnea index was 1.3, indicating near to complete resolution of OSA. Resolution of OSA may have been multifactorial. Using a quantitative approach, we demonstrate that MIOP children have OSA and hypoxemia; thus, these children should have airway evaluations and treatments to potentially reduce the risk of life-threatening pulmonary complications.

Correlation of bone age, dental age, and chronological age in survivors of childhood acute lymphoblastic leukaemia.

Abstract: Background. There is little information about oncotherapy-related dental development in childhood acute lymphoblastic leukaemia (ALL). Objective. The objective of this study was to compare bone age (BA) and dental age (DA) to chronological age (CA) in childhood ALL survivors. Methods. We retrospectively reviewed hand–wrist and panoramic radiographs of patients treated on contemporary single institution protocols for ALL between 1991 and 2004. We recorded patient demographics, therapeutic protocol, CA, DA, and BA. The cohort was divided into three categories based on age at diagnosis ( 9 years). Results. Of 73 patients, 39 (53.4%) were boys; 55 (75.3%) were Caucasian. Median CA at diagnosis was 4.5 years (range: 0.1–11.0 years); time to study was 4.1 years (range: 0.3–11.4 years). BA was normal in 61 (83.6%), delayed in 10 (13.7%), and advanced in 2 (2.7%). DA was normal in 41 (56.9%), delayed in 8 (11.1%), and advanced in 23 (31.9%). Abnormal BA, abnormal DA, and discrepancy between BA and DA are not statistically significantly associated with investigated patient or treatment factors. Conclusions. DA may be altered in 43.1% of patients treated for ALL. A large prospective study is warranted to better define our observations and to determine their impact on dental and orthodontic management.

The use of bone age for bone mineral density interpretation in a cohort of pediatric brain tumor patients

Abstract: Skeletal bone accretion occurs throughout childhood. The integrity of this process can influence future adult bone health and the risk of osteoporosis. Although surveillance of children who are at risk of poor bone accretion is important, the most appropriate method to monitor childhood bone health has not been established. Previous investigators have proposed using bone age (BA) rather than chronological age (CA) when interpreting bone mineral density (BMD) values in children. To investigate the value of BA assessment for BMD measurement in a cohort of children at risk of poor accretion. A cohort of 163 children with brain tumors who completed both a BMD assessment (quantitative computed tomography, QCT) and who had a BA within a 6-month interval were identified. The difference in BMD Z-scores determined by CA and BA was determined. The impact of salient clinical features was assessed. No significant difference between CA and BA Z-scores was detected in the overall cohort (P = 0.056). However, the scores in 18 children (all boys between the ages of 11 years and 15 years) were statistically determined to be outliers from the values in the rest of the cohort. Interpretation of BMD with BA measurement might be appropriate and affect treatment decisions in peripubertal males.

Sister Mary Joseph's nodule as presenting sign of a desmoplastic small round cell tumor.

Abstract: Umbilical metastases, also named Sister Mary Joseph's nodules, are well documented in the adult population and most often represent an underlying intra-abdominal malignancy, usually a carcinoma of gastrointestinal or gynecologic origin. They are indicative of widespread abdominal disease and are associated with a poor prognosis. An extensive review of the literature reveals only two such presentations in the pediatric population. A 14-year-old male presented with an umbilical mass, which was found to be a metastatic lesion of a desmoplastic small round cell tumor (DSRCT) of the abdomen. The diagnosis of an intra-abdominal malignancy, most commonly a DSRCT, should be considered in the presence of an umbilical mass in a child.

18F-PET–CT in extracranial paediatric oncology: when and for whom is it useful?

Abstract: Until recently, diagnostic imaging methods have provided either functional (e.g. radionuclide imaging techniques) or anatomical (e.g. cross-sectional imaging) information. Current technologic advancements now provide merged spatial resolution and physiological assessment in the form of PET–CT, which may increase the accuracy of tumour staging, distinguish between benign and malignant lesions, and assess the early response to treatment in patients with measurable tumours. Although F-PET/PET–CT has been established for a number of adult diseases, it has, until now, been lessextensively explored in paediatric patients. The growing use of PET–CT imaging in paediatrics suggests that this technique contributes significantly to the care of paediatric oncology patients. This review will discuss the preliminary uses of PET–CT in extracranial paediatric oncology, address key aspects to consider in imaging children and adolescents, and discuss potential issues specific to this patient population.

Femoral Resurfacing in Young Patients with Hematologic Cancer and Osteonecrosis

Abstract: Resurfacing hemiarthroplasties were performed to treat advanced osteonecrosis of 20 femoral heads in 14 patients (median age, 19.8 years; range, 15.1–27.4 years), treated for hematologic cancer in childhood or adolescence. Seven hips in five patients were revised to total hip arthroplasties (THA) because of pain; three of these showed radiographic loosening of the femoral head resurfacing component. The median time from resurfacing to revision was 2.4 years (range, 0.9–4.8 years). Marginal Cox-regression analysis, adjusting for correlations owing to bilateral involvement, showed positive association of revision-free survival of the prosthesis with patient’s age; time from resurfacing to the end of anticancer therapy, end of glucocorticosteroid therapy; percentage of joint space at the last radiograph; and size of the lesion has a negative association with revision-free survival. Because of this study’s exploratory nature, p values were not adjusted for the number of statistical comparisons. Among 14 patients, the probability of not requiring resurfacing prosthesis revision was 66% (SE, ±15%; 95% CI, 44%–100%) at 3 years. Osteonecrosis of the femoral head in young patients treated for hematologic cancer in childhood or adolescence poses a serious challenge to the orthopaedic surgeon. The data of this preliminary study suggest that in selected patients resurfacing hemiarthroplasty may delay the need for THA for 3–7 years. Level of Evidence: Level II, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Lymphoma — Controversies in Imaging the Chest

Abstract: In the United States each year, approximately three cases of pediatric Hodgkin’s disease and four cases of pediatric non-Hodgkin’s lymphoma occur per 100,000 members of the population (Leventhal and Donaldson 1993). In such cases, diagnostic imaging is an integral part of staging and monitoring disease progression. However, staging and assessing disease activity in the chest is particularly difficult in pediatric patients because of the presence of the thymus, the lack of mediastinal fat, and the small size of anatomic structures. Thymic rebound or regrowth further complicates disease assessment. This chapter addresses staging complications caused by thymic tissue, the distinction between lymphoma-related pulmonary nodules and those related to other processes, and methods of differentiating between recurrent active disease and residual quiescent disease. The strengths and weaknesses of available imaging techniques are incorporated into each section.

Differential diagnosis and work-up of elevations of alkaline phosphatase following therapy for pediatric cancer.

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