Family and Illness Predictors of Outcome in Pediatric Brain Tumors

Investigated the prediction of cognitive and behavioral outcomesin 63 children with heterogenous brain tumors. Hierarchicalmultiple regression analyses were used to determine how family-relatedvariables added to the prediction of children's outcome overand above illness measures. The best predictors of children'sbehavior problems and adaptive behavior were family and demographicvariables, whereas the best predictors of achievement were illnessand demographic variables. A combination of family and illnessvariables, however, was the best predictor of intellectual functioning.In addition to identifying specific predictors of cognitiveand behavioral outcome in children with brain tumors, theseresults lend initial support for the inclusion of contextualfactors such as family stress, maternal coping, number of parentsin the home, and family SES measures in studies of how diseasefactors affect outcomes in pediatric brain tumor patients.     

Cytopathology of uncommon malignant renal neoplasms in the pediatric age group

Malignant renal neoplasms are common solid tumors in pediatric oncology practice. These include the common Wilms' tumor/nephroblastoma and the uncommon neoplasms such as clear-cell sarcoma of the kidney (CCSK), rhabdoid tumor, renal-cell carcinoma, and others. The aim of this study was to describe in detail the cytopathological features of the histopathologically proven uncommon pediatric renal tumors. Aspirates from Wilms' tumor, which are mesenchyme predominant, show clusters of spindle cells associated with the matrix material. Evidence of rhabdomyoblastic differentiation may be present. CCSK, classic subtype, is characterized by round to oval cells arranged perivascularly and also in sheets and clusters intimately associated with a metachromatic matrix mucopolysaccharide material better appreciated in May-Grunwald-Giemsa (MGG)-stained smears. The cells also have more abundant cytoplasm and may show nuclear grooves. Spindle-cell pattern of CCSK is difficult to diagnose on aspiration cytology. Renal-cell carcinoma of childhood shows similar cytological features as its adult counterpart. Rhabdoid tumor of the kidney is characterized by a monomorphic population of cells with abundant cytoplasm, eccentric nuclei with prominent nucleoli. Intrarenal yolk sac tumor is a rare neoplasm and shows severely pleomorphic cells on aspiration.Awareness of these entities is important for the practicing cytopathologist. Further, non-Wilms' renal malignant neoplasms must be distinguished from the common Wilms' tumor so that appropriate chemotherapy protocols may be instituted in cases where the tumor is in an advanced stage of malignancy.     

Diffuse sclerosing variant of papillary thyroid carcinoma: A clinicopathologic and immunophenotypic analysis of 22 cases

Background: The diffuse sclerosing variant of papillary thyroid carcinoma (DSV-PTC) is an uncommon tumor making up about 2% of all papillary thyroid carcinomas. Previous studies have not comprehensively evaluated these tumors in a large series of patients. Design: Twenty-two cases of DSV-PTC diagnosed between 1970 and 2000 were identified in the files of the AFIP. Histologic and immunohistochemical features were evaluated and patient follow-up was obtained. Results: The tumors affected 14 females and 8 males, aged 6 to 49 yr (mean, 18 yr), with males presenting at a mean older age than females (24 vs 14 yr). Symptoms included an enlarging mass in the thyroid, present for a mean of 9.5 mo. While a dominant tumor was identified in a single lobe, bilateral disease was common (n=16). The dominant mass ranged in size from 1.7 to 5.8 cm in diameter (mean, 3.8 cm). Histologically, all cases demonstrated a papillary carcinoma (conventional, solid, or follicular pattern) diffusely involving the gland. Extrathyroidal extension, lymphocytic thyroiditis, squamous metaplasia, increased fibrosis/sclerosis, and psammoma bodies were present to a variable degree. Both the papillary carcinoma and squamous metaplasia cells were strongly immunoreactive with CK19, thyroglobulin, and TTF-1. An increased number of S-100 protein immunoreactive dendritic cells were recognized. p53 was increased (>15%) in the tumor cells in 12 patients, while Ki-67 was increased in the tumor cells in two patients. Perithyroidal and cervical lymph node metastasis occurred in 18 (82%) patients. All metastases demonstrated histologic features similar to the primary. Complete resection (thyroidectomy in 18 patients) with lymph node dissection, yielded a 95% 5-yr survival without evidence of disease. One patient died of disease after a malignant transformation of the squamous metaplasia into squamous cell carcinoma. Conclusions: The recognition of DSV-PTC can be made with the following features: classic to solid foci of PTC, lymphocytic thyroiditis, squamous metaplasia, increased fibrosis, and innumerable psammoma bodies. DSV-PTC is more biologically aggressive than conventional PTC, but the patients’ survival is not significantly different. This diagnosis should lead the clinician to aggressively manage these patients (thyroidectomy and lymph node dissection) in an effort to achieve an excellent long-term clinical outcome.     

Acceptability of Interventions for Pediatric Pain Management

Examined the effects of pain symptom severity and patient diagnosticstatus on pediatric staffs' acceptability ratings of 6 interventionsused to treat pediatric pain. Results indicated that (a) extinctionwas significantly less acceptable than all other behavioraland pharmacologic interventions, (b) self-management was moreacceptable than all other interventions with the exception ofcontingency management, (c) accelerative procedures and self-managementinterventions were preferred to pharmacologic treatment, (d)patient diagnostic status and pain symptom severity failed tosignificantly influence treatment ratings, (e) self-managementwas the only intervention differentially rated as a functionof pain symptom severity, and (f) pharmacologic treatment wasdifferentially rated as a function of patient diagnostic status.     

A Contextual Family/Systems Approach to Pediatric Psychology: Introduction to the Special Issue

Introduced the special issue on "Family System in PediatricPsychology" by organizing the papers around a series of themesrelevant to understanding families in pediatric psychology.Themes reflect the relationships between parent and child adjustment,family subsystems, legacies and traditions, social support systems,family interventions, the constancy of change in families, thechallenges of conducting longitudinal research, the inclusionof fathers, and the importance of a competency framework inpediatric family psychology. The paper concludes with recommendationsfor pediatric family psychology, including suggestions for increasingdiversity, expanding methodological approaches, and enhancingfamily competence.     

Fine-needle aspiration cytology of pancreatoblastoma in a young woman: report of a case and review of the literature

Pancreatoblastoma is a rare tumor and has been reported only four times in the cytologic literature, three times in fine-needle aspiration (FNA) biopsy and once in an imprint of resected tumor. We are reporting the fourth case of FNA cytology with immunohistochemical and electron microscopic studies. The patient is a 24-yr-old African American woman, who presented with a pancreatic mass, hepatic masses, and abdominal lymphadenopathy. The aspiration smears of the liver mass showed a biphasic tumor composed of bland-appearing primitive spindled stromal fragments with "spider-web"-like long fibrils interconnecting with sharply angulated islands of cohesive epithelium. At high power, the epithelium is composed of medium-sized cells with round-to-oval vesicular nuclei with fine chromatin and one-to-two small nucleoli. The neuroendocrine component was demonstrated immunohistochemically with synaptophysin and chromogranin expressions. The acinar component and squamoid component were demonstrated ultrastructurally by the presence of 400-600 nm zymogen granules and tonofilaments. The literature was reviewed and the cytological features of all the four cases of pancreatoblastoma are summarized.     

Detection of bloodstream pathogens in a bacille Calmette-Guérin (BCG)-vaccinated pediatric population in Malawi: a pilot study

Children in Malawi receive bacille Calmette-Guérin (BCG) vaccination within the first 3 days of life. Thus, we hypothesized that Malawian children infected with the human immunodeficiency type 1 virus (HIV-1) might be particularly vulnerable to dissemination of the BCG Mycobacterium bovis strain with which they were vaccinated. Following informed consent by parents, we studied children admitted to a Malawi general hospital during the 1998 wet and dry seasons. Blood from cohorts of acutely ill children was cultured for bacteria, including mycobacteria, and fungi, and tested for anti-HIV-1 antibodies. It was shown that non- typhi Salmonella and Escherichia coli were the predominant bloodstream pathogens during the wet and dry seasons, and that bloodstream dissemination of the BCG M. bovis strain is uncommon in HIV-1-infected children who receive the BCG vaccine.     

Behavioral Upset in Medical Patients-Revised: Evaluation of a Parent Report Measure of Distress for Pediatric Populations

Examined the utility of a new parent-report measure designedspecifically for pediatric inpatients, the Behavioral Upsetin Medical Patients-Revised (BUMP-R). The BUMP-R was administeredto 151 mothers of hospitalized children ages 4–12 yearsthe day following the child's hospital admission. The BUMP-Rdemonstrated good internal consistency and a factor analysisrevealed four factors identified as negativity/agitation, amiability,dysphoria, and noncom-pliance. Children exhibiting behavioraldistress at home were more likely to experience adjustment problemsupon hospitalization. Demographic and illness-related variableswere not substantial risk factors for hospital adjustment difficulties.     

A multimethod assessment of behavioral and emotional adjustment in children with asthma

OBJECTIVE: Examine behavioral adjustment and emotion regulation among 6-year-old children with asthma and a group of healthy controls. METHOD: Subjects were 81 children with asthma and 22 healthy controls. Asthma and allergy statuses were confirmed by objective measures. Emotional and behavioral functioning were assessed through parent report, child interview, and child participation in an emotional regulation paradigm. RESULTS: Maternal report revealed more internalizing and total behavior problems for children with asthma compared to controls. Child interview and behavioral observations of emotion regulation yielded no differences between groups. Severity of asthma was related to increased emotional difficulties by clinician interview and observation but not by maternal report. CONCLUSIONS: Two groups of children with asthma who have psychological difficulties include those with increased anxiety and those with poor emotion regulation and more asthma symptoms. Different measures of child adjustment yield a complex picture of the behavioral difficulties associated with pediatric asthma.     

Child illness, the parenting alliance, and parenting stress

Assessed relationship between children's minor illnesses during the first 3 years of life and parenting stress in the 4th year. Also examined whether a good parenting alliance would compensate for or moderate this relationship. Parents of 56 3- to 4-year-olds completed parenting alliance and stress questionnaires. Child morbidity, assessed from medical records, was directly related to mothers' but not fathers' feelings of stress. The parenting alliance was more strongly related to parenting stress for fathers than for mothers. However, child morbidity and parenting alliance interacted in predicting child-related paternal stresses. While fathers in a poor alliance reported more stress, stress was unrelated to their children's illnesses; for fathers in a moderate to strong alliance, illness and stress were positively correlated. Even minor child illnesses appear to be a source of stress for parents.