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Biomedical subjects

Sue C Kaste

Publications and source records attributed to Sue C Kaste.

At least 19 recordsLinked to original sources
PubMed2008

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

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.

Abdominal Neoplasms↗
PubMed2007

Pericardial involvement at diagnosis in pediatric Hodgkin lymphoma patients.

BACKGROUND: Because most cases are clinically silent, the incidence, clinical course, and outcome of pericardial involvement in Hodgkin lymphoma are unknown. METHODS: Records of all patients with newly diagnosed Hodgkin lymphoma treated at our institution between 1991 and 2004 were reviewed. Pericardial involvement was identified by computerized tomography (CT) as focal thickening or nodularity present at the time of diagnosis, and by echocardiography as pericardial effusion. Outcomes measured were incidence of pericardial involvement, relapse-free survival, and overall survival. RESULTS: Thirteen of 273 patients (5%) had pericardial involvement. All patients with pericardial involvement had nodular sclerosing tumors versus 183 of 260 patients without pericardial involvement (P = 0.02); 9 (67%) had a bulky mediastinal mass versus 27% (P = 0.002). Two patients required pericardial drainage to drain very large effusions (n = 2). Both patients were symptomatic with either shortness of breath or superior vena cava syndrome. In the 11 cases that did not undergo surgical drainage, the effusion resolved within days after starting chemotherapy. Two patients experienced distant relapse but underwent successful salvage therapy. All 13 patients remain alive and free of disease at a median follow-up of 9.7 years (range, 1.7-12.9 years) with normal cardiac function. CONCLUSIONS: Pericardial involvement by lymphoma is usually asymptomatic unless accompanied by substantial pericardial effusion. In most cases, pericardial involvement resolves with treatment of the underlying malignancy, but close observation for hemodynamic complications is required. A symptomatic effusion, once treated, does not affect survival.

Adolescent↗
PubMed2007

Quantitative bone mineral density assessment in malignant infantile osteopetrosis.

PURPOSE: To investigate the use of quantitative computed tomography (QCT) and dual energy absorptiometry (DXA) for assessing bone mineral density (BMD) in the evaluation of children with malignant infantile osteopetrosis (MIOP). METHODS AND PATIENTS: We retrospectively reviewed QCT- and DXA-determined BMD in six patients with infantile osteopetrosis and correlated BMD measured during the initial evaluation with patient characteristics. RESULTS: Five male and one female infant met the eligibility criteria. BMD was markedly elevated in all patients as determined by each modality, QCT or DXA. For QCT, in which age-specific normal values are known, the BMD was found to be 22.4-32.6 standard deviations above the mean. Using DXA, the estimated BMD of the lumbar spine ranged from 0.45 to 0.8 g/cm(2); children with similarly appearing bone radiographs had quite disparate BMD. While the qualitative trend of BMD among the patients was similar for both measures, the units and numerical values of BMD differed. We found no correlation between BMD results and hematopoiesis observed in the bone marrow or with visual evaluation of the radiographs. CONCLUSION: Both QCT and DXA are effective quantitative measures of BMD in children with MIOP. However, the same modality should be employed for longitudinal evaluation of a given patient. Each technique has unique advantages and disadvantages and may complement one another in the evaluation of MIOP or other bone disorders. Quantitative assessment of BMD in children with MIOP will likely serve to further characterize this disease.

Absorptiometry, Photon↗
PubMed2006

A model for quantitative changes in the magnetic resonance parameters of muscle in children after therapeutic irradiation.

PURPOSE/OBJECTIVE: This study aimed to develop objective models of radiation effects on musculature in children with soft tissue sarcoma using treatment dosimetry and clinical and quantitative magnetic resonance imaging (MRI) parameters that may be used to guide treatment planning or predict side effects. METHODS: In the initial 13 patients undergoing external beam radiation therapy (RT) on a Phase II study of conformal or intensity-modulated RT for the treatment of soft tissue sarcoma approved by an Institutional Review Board, we evaluated quantitative MRI changes in the musculature to assess radiation-related treatment effects. Patients with soft tissue sarcoma, including Ewing's sarcoma, had quantitative T1, T2 and dynamic enhanced MRI (DEMRI) performed before, during (Week 4) and after RT (Week 12). Regions of interest were selected in consistent locations within and outside the high-dose regions (on ipsilateral and contralateral sides when available). Mean RT dose, T1, T2 and DEMRI parameters were calculated and modeled using a mixed random coefficient dose model. RESULTS: The mean doses to the high- and low-dose regions were 56.4 Gy (41.8-75.3 Gy) and 13.0 Gy (0.1-37.5 Gy), respectively. Compared with tissues distant from the tumor bed, maximal enhancement was significantly increased in tissues adjacent to the tumor/tumor bed prior to RT (60.6 vs. 44.2, P=.045) and remained elevated after 12 weeks. T1 was significantly elevated in tissues adjacent to the tumor bed prior to RT (942.4 vs. 759.0, P=.0078). The slope of longitudinal change in T1 was greater for tissues that received low-dose irradiation than those that received high-dose irradiation (P=.0488). The effect of dose on the slope of T2 was different (P=.0333) when younger and older patients are compared. CONCLUSIONS: Acute affects of irradiation in muscle are quantifiable via MRI. These models provide evidence that quantifiable MRI parameters may be correlated with patient parameters of radiation dose and clinical factors including patient age. Long-term follow-up will be required to determine if acute changes correlate with clinically significant late effects.

Child↗
PubMed2006

St. Jude Children's Research Hospital, Memphis, Tennessee: gestational choriocarcinoma.

This paper describes a recent tumor board presentation conducted at our institution involving an adolescent with gestational choriocarcinoma. Despite its rarity in pediatrics, gestational choriocarcinoma offers unique diagnostic, treatment and off-therapy considerations in adolescent patients who have become pregnant. Key features and findings of the case as well as important management issues will be discussed.

Adolescent↗
PubMed2006

Changes in bone mineral density in survivors of childhood acute lymphoblastic leukemia.

BACKGROUND: There is little information about factors modulating bone mineral density (BMD) in survivors of childhood acute lymphoblastic leukemia (ALL). PROCEDURE: We analyzed data from 57 survivors (26 male, 52 Caucasian) who underwent two serial quantitative computed tomography (QCT) studies of BMD. Using multiple linear regression, we evaluated the association of BMD change with demographic variables, treatment history, hormone therapy, exercise, and tobacco and alcohol use. RESULTS: The median age was 3.4 years (range, 0.9-17.4 years) at diagnosis of ALL; the median age at the first QCT (Study I) was 15.0 years (range, 10.6-31.0 years) and at the second QCT (Study II) was 18.2 years (range, 14.2-35.3 years). Mean height increased 4.7 cm and mean weight increased 8.8 kg between Studies I and II. While the mean BMD increased 9.33 mg/cc (P = 0.003), the BMD Z-score increased only slightly (0.21 SD, P = 0.035). Cortical bone density increased significantly (approximately 25.3 mg/cc; P = 0.001), but the ratio of trabecular to cortical BMD decreased significantly (P = 0.045). Factors independently associated with unfavorable BMD changes included older age at diagnosis (P = 0.001), female sex (P = 0.018), and nutritional supplementation (0.032). Alcohol (P = 0.009) was an unfavorable factor in a univariable analysis. CONCLUSIONS: Bone mineral accretion during adolescence is attenuated in childhood ALL survivors by a comparative deficit in trabecular versus cortical bone deposition. BMD is influenced favorably by exercise in early adolescence and unfavorably by the use of nutritional supplements and alcohol. These results provide new information about behavioral factors that affect bone accrual in survivors of childhood ALL and warrant definitive evaluation in a larger cohort.

Adolescent↗
PubMed2006

Pediatric surface osteosarcoma: clinical, pathologic, and radiologic features.

BACKGROUND: Osteosarcoma (OS) arising from the surface of bone is far less common than its intramedullary counterpart. Although surface OSs share some radiographic and clinical features, they can be divided into three distinct histologic subtypes. PROCEDURE: We reviewed the clinical, radiographic, and pathologic features of 14 cases of pediatric surface OS treated at St. Jude Children's Research Hospital between 1970 and 2003. RESULTS: Seven patients had parosteal, five had periosteal, and two had high-grade surface OS. The median age at diagnosis was 16.2 years (range, 13.6-18.5 years). Nine patients were male; 11 were Caucasian. None had metastatic disease at diagnosis. Primary tumor sites included distal femur (n = 6), mid to proximal femur (n = 4), and mid to proximal tibia (n = 4). All 14 patients were treated with surgery, and 7 (1 with parosteal, 4 with periosteal, 2 with high-grade tumors) received chemotherapy. One patient experienced pulmonary metastasis of periosteal OS 16 months and 43 months after diagnosis; long-term disease-free survival followed resection of the metastatic tumors. Twelve patients remained alive and disease-free a median of 10 years (range, 1.5-25.4 years) after diagnosis. One patient died of high-grade surface OS 1.8 years after diagnosis, and one patient with periosteal OS died of gastric cancer 18.2 years after diagnosis of OS. CONCLUSIONS: The histologic grade predicts the clinical behavior of pediatric surface OS. Complete resection is the treatment of choice regardless of tumor subtype. Whereas chemotherapy is not indicated for parosteal OS, its role in periosteal OS remains controversial.

Adolescent↗
PubMed2006

QCT versus DXA in 320 survivors of childhood cancer: association of BMD with fracture history.

PURPOSE: To assess agreement on diagnosis of diminished bone mineral density (BMD) and correlation between BMD values obtained by dual-energy X-ray absorptiometry (DXA) and quantitative computed tomography (QCT) in childhood cancer survivors. PATIENTS AND METHODS: We retrospectively reviewed lumbar spine QCT and DXA studies for BMD in patients who underwent both imaging studies within a 24-hr period. We determined correlation between BMD values and agreement on diagnosis of diminished BMD obtained by both modalities. Diminished BMD was defined as two or more SDs below mean for age- and gender-matched reference values. We evaluated the relationship of BMD values determined by each modality to self-reported fracture history in the 160 (50%) patients with available reports. RESULTS: Of 320 patients, 56% (178) were male; 87% (277) were white. Median age was 16.4 (range, 5.1-36.0) years. Median BMD Z-score was -1.43 (range, -5.96 to 3.20) by QCT and -1.30 (range, -5.50 to 2.80) by DXA. Correlation between QCT- and DXA-determined BMD values was significant but low, and agreement on diminished BMD was fair (kappa = 0.32). There was no association between BMD measured by either QCT or DXA and self-reported traumatic fracture history. Male gender was associated with doubling the traumatic fracture risk (P = 0.0499). CONCLUSIONS: Quantitative computed tomography and DXA may give discrepant results when used to assess bone health in childhood cancer survivors, especially in those of non-white race. This inconsistency in indicators of BMD deficiency may complicate clinical decision-making. Consecutive use of a single modality is recommended to provide reliable longitudinal information.

Absorptiometry, Photon↗
PubMed2006

Retention of survivors of acute lymphoblastic leukemia in a longitudinal study of bone mineral density.

Attrition in longitudinal studies of survivors of childhood cancer reduces these studies' statistical power, introduces bias and threatens internal and external validity. This study investigated the variables associated with dropout of survivors of acute lymphoblastic leukemia in a trial investigating the effect of vitamin D and calcium supplementation and nutritional counseling on bone mineral density (BMD). Twenty-five participants withdrew from the study. Common reasons given for withdrawing were intolerance of the study drug, family hardship and schedule conflicts. Few statistically and clinically significant differences identified participants who completed the study. Nurses need to be aware of the reasons that participants withdraw from clinical trials, as they are in a strategic position to encourage patients to participate in health promotion studies.

Adaptation, Psychological↗
PubMed2006

MRI of knee osteonecrosis in children with leukemia and lymphoma: Part 2, clinical and imaging patterns.

OBJECTIVE: This aim of this study was to explore the relationship between MRI findings and clinical symptoms of knee osteonecrosis in children and to determine the significance of this relationship. Such information is important for early diagnosis of this frequent complication and for choosing an appropriate treatment strategy. MATERIALS AND METHODS: Osteonecrotic lesion size and location were determined in a retrospective analysis of MR images of the knee obtained in our institution during the past 10 years. Association between MRI findings and clinical symptoms expressed by the knee scores was tested for a subgroup of our patient population who had clinical evaluation of the knees in the orthopedic clinic within 6 weeks of their MRI studies. RESULTS: In 80% of patients, osteonecrosis was bilateral. Lesions were distributed as follows: femur and tibia, 66% of the knees; femur alone, 26%; and tibia alone, 8%. Clinical symptoms of knee osteonecrosis were associated with lesions involving the articular surface of the distal femur, large lesions, and involvement of any part of the tibia. MR images showed milder osteonecrosis in patients who were less than 10 years old at the time of primary diagnosis. CONCLUSION: Osteonecrosis affecting the knees of children treated for leukemia and lymphoma is mostly asymptomatic. Children who are 10 years old or older have signs of more advanced osteonecrosis. Clinical symptoms often lag MRI presentation and may develop late in the course of the disease. Routine MRI evaluation is recommended for timely diagnosis of treatment-induced osteonecrosis.

Adolescent↗
PubMed2006

MRI of knee osteonecrosis in children with leukemia and lymphoma: Part 1, observer agreement.

OBJECTIVE: The aim of this study was to determine whether a new MRI-based staging system for osteonecrosis of the knee in pediatric patients could be used with an acceptable level of intra- and interobserver agreement. MATERIALS AND METHODS: We conducted a retrospective analysis of MRI studies of the knee performed in a single institution between April 1994 and July 2003. Knee osteonecrosis was identified in 168 children with a primary diagnosis of hematologic malignancy. This substantial number prompted us to design a staging system for use with pediatric patients. To assess interobserver reliability of two primary observers in using the system, they reviewed and interpreted the same 36 imaging studies of randomly chosen patients. For the assessment of intraobserver reproducibility, each observer rereviewed 16 studies. A senior observer coded potential causes of disagreement between the primary observers. RESULTS: Interobserver agreement was substantial: the kappa value was 0.66 (95% confidence interval [CI], 0.58-0.75) in locations where the observers had to record only the presence or absence of a lesion, and the weighted kappa value was 0.65 (95% CI, 0.59-0.72) in locations where they had to classify the extent of involvement. The presence of marrow edema, punctate foci of altered signal, and mottled marrow changes was associated with a higher level of disagreement between the primary observers. CONCLUSION: Our proposed classification system, developed specifically for use with MRI, was used with substantial intra- and interobserver agreement. We think its use can contribute to a standardized approach to the interpretation of MRI findings in pediatric osteonecrosis of the knee.

Adolescent↗
PubMed2005

Patterns of treatment failure in pediatric and young adult patients with Hodgkin's disease: local disease control with combined-modality therapy.

PURPOSE: Refinement in managing pediatric Hodgkin's disease (HD) requires understanding of factors associated with local treatment failure. This study defines the cumulative incidence (CI) of local failure (LF) and prognostic factors for pediatric patients treated for HD with combined-modality therapy (CMT). PATIENTS AND METHODS: We enrolled 195 patients onto two prospective studies at St Jude Children's Research Hospital between 1990 and 2000. Patients received CMT with chemotherapy (vinblastine, doxorubicin, methotrexate, and prednisone [VAMP]; vinblastine, etoposide, prednisone, and doxorubicin; or VAMP/cyclophosphamide, vincristine, and procarbazine) and involved-field radiation therapy delivered to initial site(s) of disease on the basis of early response. Sites of disease involvement, treatment, and sites of failure were confirmed from the patients' medical record, imaging, and radiotherapy treatment records. We estimated the overall survival, event-free survival, and CI of LF. RESULTS: With a median follow-up of 7.6 years, the CI of LF was 10.9% and 11.6% at 5 and 10 years, respectively. Twenty-seven (14%) of 195 patients experienced recurrence of HD, and 22 (81%) of those experienced LF. Bulky mediastinal disease greater than one third transthoracic diameter predicted a higher incidence of LF, but did not predict failure in the mediastinum. Male sex, low initial hemoglobin, and bulky mediastinal disease were prognostic indicators of LF. Attenuation of radiation dose to 15 Gy based on response provides excellent infield control. CONCLUSION: CMT provides excellent local disease control in children and young adults with HD. LF remains a primary site of disease recurrence, with male sex, low initial hemoglobin, and bulky mediastinal disease predicting for LF.

Adolescent↗
PubMed2005

MR safety and compatibility of a noninvasively expandable total-joint endoprosthesis.

A noninvasively expandable total-joint endoprosthesis is now available for pediatric patients; the prosthesis can be lengthened by external application of a magnetic field. We investigated the risks of unintentional heating or lengthening of the prosthesis during MR imaging and evaluated the effect of the device on the diagnostic efficacy of MR imaging of surrounding tissues. We performed MR imaging at 1.5 T by using standard pulse sequences and pulse sequences with high-gradient and high-radiofrequency duty cycle. MR imaging caused no measurable change in prosthesis length, and the temperature of the prosthesis increased by less than 1 degrees C during repeated 14-min exposures. Despite significant signal loss and image distortion around the prosthetic joint, clinically useful images were obtained as close as 12 cm from the ends of the prosthetic stems, measured toward the body of the device. Thus, the prosthesis can be safely exposed to MR imaging pulse sequences at 1.5 T, and the visualization of some tissue surrounding the device is clinically useful.

Bone Neoplasms↗
PubMed2005

PET/CT in the evaluation of childhood sarcomas.

OBJECTIVE: Our objective was to review our preliminary experience with PET/CT in evaluating childhood sarcomas including rhabdomyosarcoma (n = 28), the Ewing's sarcoma family of tumors (n = 14), nonrhabdomyosarcoma soft-tissue sarcoma (n = 9), osteosarcoma (n = 8), chondrosarcoma (n = 1), and embryonal sarcoma (n = 1). CONCLUSION: We found PET/CT useful in depicting an unknown primary rhabdomyosarcoma and detecting unsuspected and unusual metastatic sites of childhood sarcomas. It was useful in monitoring response to chemotherapy, radiation therapy, and radiofrequency ablation and aided the postoperative evaluation of tumor resection sites.

Child↗