Spontaneous resolution of unifocal Langerhans cell histiocytosis of the skull : potential role of ultrasound in detection and imaging follow-up

Langerhans cell histiocytosis is a tumor-like condition characterized by idiopathic proliferation of Langerhans cells. The disease may involve the skeleton as well as other organs systems. Bone involvement may be solitary or multifocal. Unifocal osseous Langerhans cell histiocytosis may involve virtually any bone, with the calvarium being most frequently involved. Plain radiography, computed tomography and magnetic resonance imaging are the most used techniques for detection and characterization of the lesion. The use of ultrasound is less known, although it may be a valuable technique in detection and follow-up of superficially located lesions such as calvarial lesions. This case report describes an 8-year-old girl, in whom the lesion was initially detected by ultrasound. Furthermore, ultrasound was used to evaluate spontaneous resolution of the lesion. The knowledge of ultrasound characteristics may be important to avoid unnecessary radiation and gadolinium administration, particularly in a pediatric population

Personal distress and sympathy differentially influence health care professional and parents’ estimation of child procedure-related pain

Objective. Caregivers’ pain estimations may have important implications for pediatric pain management decisions. Affective responses elicited by facing the child in pain are considered key in understanding caregivers’ estimations of pediatric pain experiences. Theory suggests differential influences of sympathy versus personal distress on pain estimations; yet empirical evidence on the impact of caregivers’ feelings of sympathy versus distress upon estimations of pediatric pain experiences is lacking. The current study explored the role of caregiver distress versus sympathy in understanding caregivers’ pain estimates of the child’s pain experience.  Design, Setting, Subjects and Methods. Using a prospective design in 31 children undergoing consecutive lumbar punctures and/or bone marrow aspirations at Ghent University Hospital, caregivers’ (i.e., parents, physicians, nurses, and child life specialists) distress and sympathy were assessed before each procedure; estimates of child pain were obtained immediately following each procedure.  Results. Results indicated that the child’s level of pain behavior in anticipation of the procedure had a strong influence on all caregivers’ pain estimations. Beyond the impact of child pain behavior, personal distress explained parental and physician’s estimates of child pain, but not pain estimates of nurses and child life specialists. Specifically, higher level of parental and physician’s distress was related to higher child pain estimates. Caregiver sympathy was not associated with pain estimations.  Conclusions. The current findings highlight the important role of caregivers’ felt personal distress when faced with child pain, rather than sympathy, in influencing their pain estimates. Potential implications for pain management are discussed

The relationship between parental catastrophizing about child pain and distress in response to medical procedures in the context of childhood cancer treatment: A longitudinal analysis

Objective Children with leukemia frequently undergo invasive medical procedures, such as lumbar punctures (LPs) and bone marrow aspirations (BMAs). To date, cross-sectional evidence indicates that LP/BMA procedures continue to elicit distress over the course of treatment in children and parents.  Method  The current study used prospective analyses investigating in 28 children diagnosed with leukemia, the course of parental and child distress when confronted with consecutive LP/BMA procedures and potential moderation by catastrophic thinking. Parents' level of catastrophic thoughts was assessed before the first treatment-related LP/BMA, while child and parent distress was reported on after each LP/BMA procedure.  Results  Whereas parental distress decreased over time among low catastrophizing parents, LP/BMA procedures remained highly distressing for high catastrophizing parents. Child distress during LP/BMA procedures increased over time and was positively related with parental distress.  Conclusion  These findings stress the importance of targeting child and parent distress as early as possible in treatment.&nbsp

Spatiotemporal Pattern of Neuroinflammation After Impact-Acceleration Closed Head Injury in the Rat

Inflammatory processes have been implicated in the pathogenesis of traumatic brain damage. We analyzed the spatiotemporal expression pattern of the proinflammatory key molecules: interleukin-1β, interleukin-6, tumor necrosis factor-α, and inducible nitric oxide synthase in a rat closed head injury (CHI) paradigm. 51 rats were used for RT-PCR analysis after CHI, and 18 for immunocytochemistry. We found an early upregulation of IL-1β, IL-6, and TNF-α mRNA between 1 h and 7 h after injury; the expression of iNOS mRNA only revealed a significant increase at 4 h. After 24 h, the expression decreased towards baseline levels, and remained low until 7 d after injury. Immunocytochemically, IL-1β induction was localized to ramified microglia in areas surrounding the primary impact place as well as deeper brain structures. Our study shows rapid induction of inflammatory gene expression that exceeds by far the primary impact site and might therefore contribute to tissue damage at remote sites

Dilemmas in the Management of an Infant with Neuroblastoma Metastasized to the Muscles

The risk stratification of infants with metastatic neuroblastoma (NB) has evolved over time from stage 4/M or IVs/4S/MS/Ms according to various staging systems. Despite these developments for some genetic aberrations, the prognostic value and the impact of soft tissue metastases in infants are not fully understood, nor well described in the different classification systems, hampering the definitions to uniformly treat patients and predict prognosis. A literature review on staging of infants with M/MS disease was performed at the occasion of the diagnosis of NB in an 8-month-old boy who presented with atypical metastatic sites in soft tissue and an aberrant tumor biology. The definitions of stage 4/4S/4s/M/MS/Ms were evaluated and compared to enable tumor risk stratification and inform management. International NB groups use different criteria for defining stage of infants with metastasized NB, resulting in differences in management. Limited literature is available on soft tissue metastases, especially muscular metastases, and is poorly incorporated into management guidelines mainly due to the lack of data. The uncertain prognosis of rare genetic aberrancies may add to the difficulties in treatment decisions. In some rare cases of NB in infants, the international treatment classification is not sufficient for staging and treatment decisions. Based on tumor progression, biology of unknown significance and a lack of evidence to classify a child under 12 months with NB and multiple muscular metastases, the patient was treated as stage 4/M and intermediate-risk protocols with a favorable outcome

Phase II study of irinotecan in combination with temozolomide (TEMIRI) in children with recurrent or refractory medulloblastoma: a joint ITCC and SIOPE brain tumor study

BackgroundThis multicenter phase II study investigated temozolomide + irinotecan (TEMIRI) treatment in children with relapsed or refractory medulloblastoma.MethodsPatients received temozolomide 100–125 mg/m2/day (days 1–5) and irinotecan 10 mg/m2/day (days 1–5 and 8–12) every 3 weeks. The primary endpoint was tumor response within the first 4 cycles confirmed ≥4 weeks and assessed by an external response review committee (ERRC). In a 2-stage Optimum Simon design, ≥6 responses in the first 15 evaluable patients were required within the first 4 cycles for continued enrollment; a total of 19 responses from the first 46 evaluable patients was considered successful.ResultsSixty-six patients were treated. Seven responses were recorded during stage 1 and 15 in the first 46 ERRC evaluated patients (2 complete responses and 13 partial responses). The objective response rate during the first 4 cycles was 32.6% (95% confidence interval [CI], 19.5%–48.0%). Median duration of response was 27.0 weeks (7.7–44.1 wk). In 63 patients evaluated by local investigators, the objective response rate was 33.3% (95% CI, 22.0%–46.3%), and 68.3% (95% CI, 55.3%–79.4%) experienced clinical benefit. Median survival was 16.7 months (95% CI, 13.3–19.8). The most common grade 3 treatment-related nonhematologic adverse event was diarrhea (7.6%). Grade 3/4 treatment-related hematologic adverse events included neutropenia (16.7%), thrombocytopenia (12.1%), anemia (9.1%), and lymphopenia (9%).ConclusionsThe planned study primary endpoint was not met. However, its tolerability makes TEMIRI a suitable candidate chemotherapy backbone for molecularly targeted agents in future trials in this setting

Clinical value of SPECT/CT for evaluation of patients with painful knees after total knee arthroplasty- a new dimension of diagnostics?

<p>Abstract</p> <p>Background</p> <p>The purpose of our study was to evaluate the clinical value of hybrid SPECT/CT for the assessment of patients with painful total knee arthroplasty (TKA).</p> <p>Methods</p> <p>Twenty-three painful knees in patients following primary TKA were assessed using Tc-99m-HDP-SPECT/CT. Rotational, sagittal and coronal position of the TKA was assessed on 3D-CT reconstructions. The level of the SPECT-tracer uptake (0-10) and its anatomical distribution was mapped using a validated localization scheme. Univariate analysis (Wilcoxon-Mann-Whitney, Spearmean`s-rho test, p < 0.05) was performed to identify any correlations between component position, tracer uptake and diagnosis.</p> <p>Results</p> <p>SPECT/CT imaging changed the suspected diagnosis and the proposed treatment in 19/23 (83%) knees. Progression of patellofemoral OA (n = 11), loosening of the tibial (n = 3) and loosening of the femoral component (n = 2) were identified as the leading causes of pain after TKA.</p> <p>Patients with externally rotated tibial trays showed higher tracer uptake in the medial patellar facet (p = 0.049) and in the femur (p = 0.051). Patients with knee pain due to patellofemoral OA showed significantly higher tracer uptake in the patella than others (p < 0.001).</p> <p>Conclusions</p> <p>SPECT/CT was very helpful in establishing the diagnosis and guiding subsequent management in patients with painful knees after TKA, particularly in patients with patellofemoral problems and malpositioned or loose TKA.</p

Long Time to Diagnosis of Medulloblastoma in Children Is Not Associated with Decreased Survival or with Worse Neurological Outcome

International audienceBACKGROUND: The long time to diagnosis of medulloblastoma, one of the most frequent brain tumors in children, is the source of painful remorse and sometimes lawsuits. We analyzed its consequences for tumor stage, survival, and sequelae. PATIENTS AND METHODS: This retrospective population-based cohort study included all cases of pediatric medulloblastoma from a region of France between 1990 and 2005. We collected the demographic, clinical, and tumor data and analyzed the relations between the interval from symptom onset until diagnosis, initial disease stage, survival, and neuropsychological and neurological outcome. RESULTS: The median interval from symptom onset until diagnosis for the 166 cases was 65 days (interquartile range 31-121, range 3-457). A long interval (defined as longer than the median) was associated with a lower frequency of metastasis in the univariate and multivariate analyses and with a larger tumor volume, desmoplastic histology, and longer survival in the univariate analysis, but not after adjustment for confounding factors. The time to diagnosis was significantly associated with IQ score among survivors. No significant relation was found between the time to diagnosis and neurological disability. In the 62 patients with metastases, a long prediagnosis interval was associated with a higher T stage, infiltration of the fourth ventricle floor, and incomplete surgical resection; it nonetheless did not influence survival significantly in this subgroup. CONCLUSIONS: We found complex and often inverse relations between time to diagnosis of medulloblastoma in children and initial severity factors, survival, and neuropsychological and neurological outcome. This interval appears due more to the nature of the tumor and its progression than to parental or medical factors. These conclusions should be taken into account in the information provided to parents and in expert assessments produced for malpractice claims