Multiple schwannomas in the radial nerve

We present a case of multiple schwannomas in the radial nerve. The occurrence of multiple schwannomas in a single major nerve is very rare. Magnetic resonance imaging was useful in detecting the tumours. As schwannomas may be multiple without clinical symptoms, we recommend MR imaging of the entire limb when schwannomas occur in a major nerve in the upper extremity.     

Sequential half-body irradiation in childhood

Single-dose half-body irradiation (HBI), introduced for the palliation of pain from widespread bone metasases in adults, has proved to be successful. Relief is obtained in a high proportion of patients, with upwards of two-thirds experiencing complete relief. Onset of response is fast and clinically valuable duration varies from 5 to 20 weeks. Objective evidence of tumor regression is found less frequently. The dose-limiting toxicity has proved to be acute radiation pneumonitis, with bone marrow tolerance of lesser importance, in spite of the fact that many patients received previous local irradiation and/or chemotherpy. Palliative HBI has not become a valuable treatment in pediatric malignancies, because of a shorter metastatic phase. Pediatric tumors usually have shorter cell cycle times and are more responsive to systemic agents. Results are described in selected institutions, where HBI has been used in the treatment of pediatric malignancies. A single institution pilot study was undertaken at the Princess Margaret Hospital involving 17 patients with Ewing's sarcoma of bone, without overt metastases at diagnosis. Results to date have not been obviously different from overall survival in the first intergroup Ewing's sarcoma study. Overall, the treatment has been shown to be well tolerated and can be given entirely on an out-patient basis. When compared on a historical basis with a previous single dose total body irradiation study, the one year survival rate was increased. HBI appears to be a tolerable treatment, when given concurrently with or sequential to local and systemic treatment.     

The involvement of calpain-dependent proteolysis of the tumor suppressor NF2 (merlin) in schwannomas and meningiomas

Neurofibromatosis type 2 (NF2) protein, also known as merlin or schwannomin, is a tumor suppressor, and NF2 is mutated in most schwannomas and meningiomas. Although these tumors are dependent on NF2, some lack detectable NF2 mutations, which indicates that alternative mechanisms exist for inactivating merlin. Here, we demonstrate cleavage of merlin by the ubiquitous protease calpain and considerable activation of the calpain system resulting in the loss of merlin expression in these tumors. Increased proteolysis of merlin by calpain in some schwannomas and meningiomas exemplifies tumorigenesis linked to the calpain-mediated proteolytic pathway.     

Partial seizures associated with antiphospholipid antibodies in childhood

We report antiphospholipid antibody positivity in three of a consecutive series of 23 children presenting partial epileptic seizures. There was no clinical or serological evidence of systemic lupus erythematosus or other connective-tissue disease. Neither computed tomography nor magnetic resonance imaging revealed ischemic alteration. The presence of antiphospholipid antibodies in 3/23 children may indicate that immune-mediated neuronal damage could be a pathogenetic mechanism for partial epilepsy.     

Brain edema in meningiomas is associated with increased vascular endothelial growth factor expression

OBJECTIVE: Vascular permeability factor/vascular endothelial growth factor (VPF/VEGF), an endothelial cell-specific cytokine, induces proliferation of endothelial cells and increases vascular permeability dramatically. All gliomas secrete significant amounts of VEGF, whereas meningiomas are variable in expression. Thus, we sought to determine whether the extent of VPF/VEGF expression in meningiomas correlated with differences in brain edema associated with these tumors. METHODS: Meningioma tissue samples from 37 patients (15 men, average age 65 +/- 13 yr; 22 women, average age 60 +/- 10 yr) who underwent surgery at or were referred to the University of Alabama Hospital were examined retrospectively for the extent of expression of immunoreactive VPF/VEGF. Additionally, peritumoral edema was assessed on a blinded basis radiographically from preoperative magnetic resonance imaging scans. Selected specimens were examined by in situ hybridization to document the source of VPF/VEGF. RESULTS: The predominant meningioma subclassifications were transitional (57%) or meningothelial (27%) subtypes. VPF/VEGF immunoreactivity ranged from 0 to 3.5, with a median value of 2 on a subjective 5-point scale; magnetic resonance imaging-assessed edema ranged in extent from 0 to 4 (subjective 5-point scale), with a median value of 2.5. The correlation of determination (R2) of magnetic resonance imaging-assessed tumor edema rating and VPF/VEGF staining intensity rating was 0.6087 (r = 0.78; P = 0.0001). In situ hybridization localized VPF/VEGF messenger ribonucleic acid in meningioma cells and not in normal parenchymal brain cells. CONCLUSION: These data suggest that meningioma-associated edema may be a result of the capacity of meningioma cells to produce VPF/VEGF locally, leading to increased tumor neovascularization and enhanced vascular permeability.     

Allelic loss at 1p is associated with tumor progression of meningiomas

Next to chromosome 22 anomalies, deletions of the short arm of chromosome 1 have previously been described as the most frequent alteration detected by cytogenetic analysis of meningiomas. To determine the incidence of these deletions, we have analyzed a series of 50 meningiomas for the loss of alleles at four chromosome 1 loci. Thirteen samples displayed LOH for the markers studied; in one instance, the results were compatible with loss of the entire chromosome 1, whereas in the other 12 samples deletions of the short arm were observed. Eleven of the meningiomas had previously been shown to have loss of alleles on chromosome 22, and 12 of them were characterized by increased tumor aggressiveness. These findings suggest that deletion of Ip (or the alteration of a locus located there) might represent a secondary, but nonrandom alteration in meningiomas, perhaps contributing to meningioma tumor progression.     

Multiple skin cancers associated with hydroxyurea therapy

The association of multiple cutaneous cancers and long-term use of hydroxyurea is now being recognized. In this article, we describe the development of multiple skin tumors in two patients who were receiving hydroxyurea therapy. These cases illustrate the late onset of subsequent skin cancers despite discontinuation of therapy. As hydroxyurea continues to have a prominent role in the treatment of myeloproliferative diseases, clinicians must be aware of the increased risk of multiple skin cancers and use preventive and skin cancer screening practices in patients with these diseases.     

Multiple endocrine neoplasia associated with multiple lipomas

BACKGROUND: Multiple endocrine neoplasia type 1 (MEN-1) is characterized by tumors of the parathyroids, the neuroendocrine pancreas-duodenum and the anterior pituitary, but shows also a wide clinical variety of other symptoms. CASE REPORT: We present a case of a 68-year-old woman with a 18 year history of MEN-1 consisting of gastrinoma and primary hyperparathyroidism. Beside these typical symptoms, the patient suffered from thyroid adenoma, malignant kidney tumor and multiple subcutaneous lipomas. RESULT: While the number of autopsies declined from 113 in 1977 to 66 in 1984, the number of diagnostic techniques used increased continuously (94, 107, 118 and 140, amounting to 0.83, 1.34, 1.76 and 2.12 per patient). The premortal detection of abdominal abnormalities increased globally from 16.8 to 32.5%. This increase was largely due to better diagnosis of liver and gallbladder abnormalities which were in most cases of little relevance. CONCLUSION: Whether these non-endocrine tumors are associated to MEN-1 is unclear and has to be tested by examining the chromosomal regions 11q13 and 11q24/25 of the tumors sample, in which the possible MEN-1 involved tumor suppressor genes are located.     

Cortisol reactivity and regulation associated with shame responding in early childhood.

The purpose of this study was to characterize cortisol response and regulation associated with shame responding in early childhood and to examine how general the relation between shame and cortisol is. It was predicted that children responding to task failure with shame would show a larger and more prolonged cortisol response than other children. Participants were 214 children (124 boys, 90 girls) ranging from 3.7 to 4.5 years of age (M = 4.14 years, SD = 0.24). Shame responding was assessed from children's emotion-expressive behavior in response to failing 6 performance tasks, 2 preceding (initial) and 4 following (subsequent) assessment of hypothalamic-pituitary-adrenal axis activation. Cortisol response and regulation associated with failure were assessed from saliva sampled before and 15, 20, 25, 30, and 40 min following the first of the 2 initial failures. For boys and for some girls, high initial shame was associated with greater cortisol reactivity and slower regulation of the cortisol response. For boys, high initial shame and relatively slow regulation of the associated cortisol response predicted subsequent shame responding occurring after recovery of the cortisol response. For girls, high initial shame, but not cortisol response, predicted subsequent shame responding. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Long-term follow-up of childhood epilepsy associated with tuberous sclerosis

BACKGROUND: Fructose-sorbitol (F-S) mixtures can provoke symptoms in irritable bowel syndrome (IBS) patients, and a proportion of IBS patients also have enteric hypersensitivity to distension. We hypothesized, therefore, that sugar malabsorption and fermentation to produce hydrogen gas may provoke symptoms to a greater extent in IBS patients hypersensitive to distension than in those patients without such hypersensitivity. Our aims were therefore to compare, in IBS patients, symptoms and breath hydrogen responses after F-S, on the basis of jejunal sensitivity and jejunal motor function. METHODS: Fifteen female IBS patients (44 +/- 15 years) underwent, on separate occasions, 3-h breath hydrogen analyses after ingesting 10 g lactulose and 25 g fructose with 5 g sorbitol. Jejunal sensitivity and motor function were determined by balloon distension and 24-h manometry studies, respectively. Cumulative symptom scores and breath hydrogen production were analysed on the basis of the presence or absence of jejunal hypersensitivity and dysmotility. RESULTS: Four and seven patients had jejunal hypersensitivity for initial perception and pain, respectively. Eleven, nine, and nine patients had jejunal dysmotility for fasting phase 3, phase 2, and fed motor activity, respectively. Of the patients with symptom provocation after F-S (n = 8 within 3 h, n = 12 within 12 h) or with F-S malabsorption (n = 10), the relative proportion did not differ on the basis of the presence or absence of jejunal hypersensitivity or of motor dysfunction. Symptom scores and hydrogen production also were not different in these subgroups. CONCLUSIONS: Although carbohydrate malabsorption can provoke symptoms in some IBS patients, there is no consistent association between such a phenomenon and the presence of either jejunal hypersensitivity or dysmotility.     

Multiple cutaneous cylindromas associated with parotid and submandibular gland cylindromas

BACKGROUND: Multiple cutaneous cylindromas are uncommonly associated with extracutaneous cylindromas. An association between cutaneous cylindromas and salivary gland cylindromas would probably not be fortuitous. CASE REPORT: A 66-year-old man developed multiple cutaneous cylindromas and cylindromas situated in the parotid and submaxillary gland. Histology was similar for both localizations. DISCUSSION: This association was probably not fortuitous as the histology was similar, other cases have been reported in the literature, and recent complaint concerned the development of multiple cutaneous cylindromas and salivary gland cylindromas. Salivary gland cylindromas should be searched for in patients who develop cutaneous cylindromas as they are more aggressive in nature.     

Factors associated with continuity and changes in disruptive behavior patterns between childhood and adolescence

The relationships between disruptive behaviors in middle childhood (7 to 9 years) and conduct disorder in adolescence (14 to 16 years) were studied in a birth cohort of New Zealand children. Latent class analysis suggested strong behavioral continuity, with children showing early disruptive behaviors having odds of adolescent conduct disorder that were over 16 times higher than children who did not display early disruptive behavior. Nonetheless, in the region of 12% of children showed a discontinuous history, with 5% of children showing an early onset of conduct problems and later remission while 7% showed later onset conduct problems. Children showing discontinuous histories of behavior problems came from backgrounds in which levels of risk were intermediate between those of children who showed a persistent pattern of conduct problems and those who were consistently nonproblem children. Peer factors played an influential role in behavioral change in adolescence, with individuals showing late onset of conduct problems having high rates of affiliation with delinquent peers but those showing remission of problem behaviors in adolescence having relatively low rates of such affiliations.     

Convulsions with meningiomas: incidence and significance

In a study of a consecutive series of 127 surgically treated meningiomas, it was found that 29% of the patients had reported with convulsions as their initial symptom. In this group, surgical excision of the meningioma stopped the convulsions in about half of the patients, but the others continued to have seizures after their operations. Among those patients with meningiomas who did not have preoperative convulsions, about one-sixth (15 patients) developed postoperative seizures. Patients in both groups required prolonged anticonvulsant medication. Factors predisposing to the occurrence of postoperative seizures were the site of the tumor, faulty surgical technique, and a preoperative history of seizures.     

Hormone receptors in vestibular schwannomas

The possibility that steroid hormones play a role in vestibular schwannoma proliferation has been suggested by a number of investigators. There is conflicting information about the presence of steroid hormone receptors in these tumors. The aim of this study was to examine the expression of androgen, progesterone, glucocorticoid and estrogen receptor messenger ribonucleic acid levels (mRNA) in twenty-one vestibular schwannomas by either Northern blot analysis or the polymerase chain reaction (PCR). Glucocorticoid receptor mRNA was expressed in all twenty-one tumors examined. Only two male specimens were positive for androgen receptor mRNA expression by PCR-Southern blot analysis. Thirty-three percent of the schwannomas (7/21) showed a strong band for progesterone receptor mRNA by PCR-Southern blot analysis; there were an equal number of males and females in this group. Estrogen receptor mRNA levels were undetectable in all tumors examined by PCR-Southern blot analysis. These studies suggest that the pattern of steroid receptor expression is different in schwannomas than in meningiomas. Individual vestibular schwannomas need to be examined for their steroid receptor mRNA expression mRNA expression to know whether they will be responsive.     

Cognitive and academic problems associated with childhood cancers and sickle cell disease.

Childhood cancers and sickle cell disease represent some of the most complex medical conditions of childhood, impacting development in all domains. The influence of these conditions on cognitive functioning and academic achievement has particular relevance for the school psychologist, who is poised to promote the positive adaptation of children with these chronic illnesses in the school setting. This article provides a review of the research related to cognitive aspects of pediatric cancer and sickle cell disease. Based on that literature, science-based practice recommendations of relevance to the school psychologist are offered. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Idiopathic constipation in childhood is associated with thickening of the internal anal sphincter

This study was undertaken to search for a rational basis for the use of anal dilatation and internal sphincterotomy as the treatment for chronic intractable constipation in children. Sixteen children, age 5 months to 13 years, who had constipation resistant to conservative treatment were compared with 39 age-matched controls. History and current symptoms were assessed using a standard questionnaire. Internal and external and sphincter morphology was assessed on clinical examination and by anal endosonography, using a 10-MHz rotating endoprobe to provide accurate measurement of the various components of the anal canal. The control group showed a linear correlation between the thickness of the internal anal sphincter and both age and weight, increasing from 0.4 mm in infancy to 0.9 mm in adolescence. Children who had constipation displayed significant thickening of the internal sphincter (range, 0.5 to 1.9 mm, P = .005) which was independent of the length of the history (P = .103). There was no difference in the morphology of the external anal sphincters between the groups. The finding of a hypertrophied internal anal sphincter could provide a rational basis for anal dilatation and internal sphincterotomy as treatment for idiopathic constipation.