[Conventional epiphyseal chondrosarcoma of childhood and adolescence: a case report]. / Konventionelles epiphysäres Chondrosarkom des Kindes- und Jugendalters: ein Fallbericht.

INTRODUCTION: Conventional chondrosarcoma is the second most common primary malignant bone tumor and usually occurs at older adult ages. It is rare in childhood and adolescence. CASE HISTORY: This case report presents the treatment course of a 13-year-old boy with a symptomatic chondrogenic tumor of the right distal femur. Histopathologically, an epiphyseal intermediate-grade chondrosarcoma (G2) was diagnosed. DISCUSSION: Based on the following case, potential radiological and histopathological differential diagnoses, such as chondroblastoma or chondroblastic osteosarcoma, are discussed against the background of current standards in orthopedic oncology.

Diagnostic utility of cerebrospinal fluid analysis in dogs with suspected idiopathic epilepsy.

BACKGROUND: Idiopathic epilepsy (IE) is the most common cause of repeated seizures in dogs. The International Veterinary Epilepsy Task Force consensus guidelines recommend performing magnetic resonance imaging (MRI) of the brain and cerebrospinal fluid (CSF) analysis as part of a tier II diagnosis of IE, and these procedures have documented risks. The aim of this retrospective study was to identify how often dogs with suspected IE have abnormalities on CSF analysis. METHODS: Dogs aged between 6 months and 6 years that were presented with a history of two or more seizures with at least 24 h between seizure episodes, a normal neurologic examination, no evidence of toxic or metabolic causes, a normal MRI scan (including contrast administration) and CSF analysis were included. RESULTS: Eighty-two dogs were included. Of these, nine dogs (10.9%) had abnormalities on CSF analysis: five of nine dogs (55.5%) had albuminocytologic dissociation, three of nine dogs (33.3%) had mild increases in total nucleated cell count (TNCC), and one of nine dogs (11.1%) had mild increase in both total protein and TNCC. Cytology in dogs with elevated TNCC revealed a mononuclear pleocytosis. One of the nine dogs with abnormal CSF had a seizure within the 24 h before investigations, and six of nine dogs had a seizure within 1 month before investigation. CONCLUSION: CSF analysis can play an important role in the diagnostic investigation of the underlying causes of repeated seizures. However, in dogs with a normal inter-ictal neurological examination and MRI scan, it rarely reveals significant abnormalities, and the risk of performing a CSF tap may outweigh the potential diagnostic gain.

[Extranodal B-cell lymphoma in the distal femur with pre-existing CRMO]. / Extranodales B-Zell-Lymphom im distalen Femur bei vorbestehender CRMO.

CRMO is a rare form of nonbacterial osteomyelitis, which is mainly found in children. The diagnosis is complex because bone tumors or lymphoma must also be excluded. The case report describes the development of a solitary femoral lymphoma on the basis of a known CRMO disease as well as the differential diagnostic pitfalls.

Presence of thoracic and lumbar vertebral malformations in pugs with and without chronic neurological deficits.

Congenital vertebral malformations (CVMs) are common in brachycephalic dogs such as the pug, and are often considered incidental findings. However, specific CVMs have been suggested to be associated with neurological deficits in pugs. The objective of this study was to investigate the clinical importance of CVMs in the pug by comparing computed tomography studies of the thoracolumbar spine from pugs without neurological deficits with those from pugs with a confirmed T3-L3 spinal cord lesion and neurological deficits consistent with a chronic T3-L3 myelopathy. A total of 57 pugs were recruited into the study from Sweden (n=33), United Kingdom (n=21) and Norway (n=3); 30 with neurological deficits and 27 without. Focal T3-L3 pathology was confirmed in all pugs with neurological deficits by magnetic resonance imaging (n=29) and/or pathology (n=15). Computed tomography studies of the thoracolumbar spine from pugs with and without neurological deficits were compared to investigate possible associations between presentation of neurological deficits consistent with chronic T3-L3 pathology and signalment variables, presence of CVMs and type of CVMs. Congenital vertebral malformations were as common in pugs with, as in pugs without, neurological deficits. Regardless of neurological status, the majority of pugs (96%) presented with one or more CVM. An association between presence, or type of CVM in the T1-L3 vertebral column, and neurological deficits consistent with T3-L3 pathology could not be confirmed.

C7 vertebra homeotic transformation in domestic dogs - are Pug dogs breaking mammalian evolutionary constraints?

The number of cervical vertebrae in mammals is almost constant at seven, regardless of their neck length, implying that there is selection against variation in this number. Homebox (Hox) genes are involved in this evolutionary mammalian conservation, and homeotic transformation of cervical into thoracic vertebrae (cervical ribs) is a common phenotypic abnormality when Hox gene expression is altered. This relatively benign phenotypic change can be associated with fatal traits in humans. Mutations in genes upstream of Hox, inbreeding and stressors during organogenesis can also cause cervical ribs. The aim of this study was to describe the prevalence of cervical ribs in a large group of domestic dogs of different breeds, and explore a possible relation with other congenital vertebral malformations (CVMs) in the breed with the highest prevalence of cervical ribs. By phenotyping we hoped to give clues as to the underlying genetic causes. Twenty computed tomography studies from at least two breeds belonging to each of the nine groups recognized by the Federation Cynologique Internationale, including all the brachycephalic 'screw-tailed' breeds that are known to be overrepresented for CVMs, were reviewed. The Pug dog was more affected by cervical ribs than any other breed (46%; P < 0.001), and was selected for further analysis. No association was found between the presence of cervical ribs and vertebral body formation defect, bifid spinous process, caudal articular process hypoplasia/aplasia and an abnormal sacrum, which may infer they have a different aetiopathogenesis. However, Pug dogs with cervical ribs were more likely to have a transitional thoraco-lumbar vertebra (P = 0.041) and a pre-sacral vertebral count of 26 (P < 0.001). Higher C7/T1 dorsal spinous processes ratios were associated with the presence of cervical ribs (P < 0.001), supporting this is a true homeotic transformation. Relaxation of the stabilizing selection has likely occurred, and the Pug dog appears to be a good naturally occurring model to further investigate the aetiology of cervical ribs, other congenital vertebral anomalies and numerical alterations.

Sexual selection on the multicomponent display of black morph male Girardinus metallicus (Pisces: Poeciliidae).

Sexually selected displays often include suites of integrated traits. Black morph males of the poeciliid fish Girardinus metallicus perform courtship and aggressive displays that exhibit their conspicuous yellow and black coloration. Body size, gonopodium size and ventral black area are correlated with intermale aggression, which is key for access to mates. A previous study showed that females may prefer dominant males prior to watching them fight; however, that result was obtained in trials that allowed for male-male interactions across partitions, and to date no study has uncovered the traits important in female choice. We performed a more comprehensive investigation of the multicomponent sexual display including measures of male yellow hue, saturation and brightness. We examined the behavior of size-matched males paired to maximize the difference in yellow saturation, and measured female choice exclusive of male-male interactions and chemical cues. We found no female preference for any traits in the multicomponent sexual display. Males with brighter and more saturated yellow coloration were more likely to be dominant, and dominant males courted and attempted copulations more. Our results suggest that yellow coloration is sexually selected; however, the courtship display requires further investigation because we did not identify targets of female preference, and we discuss possible explanations for this finding.

[Notochordal tumors : Benign notochordal tumors and chordomas]. / Notochordale Tumoren : Benigne notochordale Tumoren und Chordome.

Benign notochordal tumors (BNCT) and chordomas are primary bone tumors of the spine with a predominant localization in the sacrum and clival region followed by the vertebral bodies. Besides the most common variant (NOS [not otherwise specified] with hepatoid or renal carcinoma cell-like differentiation) chordomas with chondroid, and polymorphic to anaplastic morphology are described. An unfavorable variant are pediatric chordomas with a loss of INI-1. BNCT and chordomas are characterized by the following immunohistological profile: vimentin+, cytokeratin+/-, epithelial membrane antigen (EMA)+/-, S100 protein+/-, brachyury+. This profile helps to distinguish these tumors from other lesions such as chondrosarcoma, chordoid meningioma, and metastases of carcinoma.

Interval cancers after skin cancer screening: incidence, tumour characteristics and risk factors for cutaneous melanoma.

BACKGROUND: The rate of interval cancers is an established indicator for the performance of a cancer-screening programme. METHODS: We examined the incidence, tumour characteristics and risk factors of melanoma interval cancers that occurred in participants of the SCREEN project, which was carried out 2003/2004 in Schleswig-Holstein, Germany. Data from 350 306 SCREEN participants, who had been screened negative for melanoma, were linked to data of the state cancer registry. Melanoma interval cancers were defined as melanomas diagnosed within 4-24 months after SCREEN examination. Results were compared with melanomas of the pre-SCREEN era (1999-2002), extracted from the cancer registry. RESULTS: The overall relative incidence of melanoma interval cancers in terms of observed/expected ratio was 0.93 (95% CI: 0.82-1.05; in situ: 1.61 (1.32-1.95), invasive: 0.71 (0.60-0.84)). Compared with melanomas of the pre-SCREEN era, the interval melanomas were thinner and had a slightly greater proportion of lentigo maligna melanomas whereas nodular melanomas were less frequent. INTERPRETATION: The results indicate a moderate performance of the SCREEN intervention with an excess of in situ melanomas. In part, the findings might be due to specifics of the SCREEN project, in particular a short-term follow-up of patients at high risk for melanoma.

Priming the Pump for Big Data at Sentara Healthcare.

Today's healthcare organizations are facing significant demands with respect to managing population health, demonstrating value, and accepting risk for clinical outcomes across the continuum of care. The patient's environment outside the walls of the hospital and physician's office-and outside the electronic health record (EHR)-has a substantial impact on clinical care outcomes. The use of big data is key to understanding factors that affect the patient's health status and enhancing clinicians' ability to anticipate how the patient will respond to various therapies. Big data is essential to delivering sustainable, highquality, value-based healthcare, as well as to the success of new models of care such as clinically integrated networks (CINs) and accountable care organizations.Sentara Healthcare, based in Norfolk, Virginia, has been an early adopter of the technologies that have readied us for our big data journey: EHRs, telehealth-supported electronic intensive care units, and telehealth primary care support through MDLIVE. Although we would not say Sentara is at the cutting edge of the big data trend, it certainly is among the fast followers. Use of big data in healthcare is still at an early stage compared with other industries. Tools for data analytics are maturing, but traditional challenges such as heightened data security and limited human resources remain the primary focus for regional health systems to improve care and reduce costs. Sentara primarily makes actionable use of big data in our CIN, Sentara Quality Care Network, and at our health plan, Optima Health. Big data projects can be expensive, and justifying the expense organizationally has often been easier in times of crisis. We have developed an analytics strategic plan separate from but aligned with corporate system goals to ensure optimal investment and management of this essential asset.

A Rare Case of Isolated Cerebral Sarcoidosis Presenting as Suprasellar Mass Lesion with Salt-Wasting Hypopituitarism.

Background Sarcoidosis is a systemic disorder of unknown origin characterized by noncaseating granulomas. Clinical symptoms due to central nervous system (CNS) involvement occur in 5 to 7% of all cases; subclinical involvement is more frequent. Sole CNS involvement is very rare. Case Report A 25-year-old man presented with increasing polyuria and polydipsia over 8 weeks. Magnetic resonance imaging (MRI) revealed a supra- and infra-chiasmatic pre-thalamic mass lesion 1.0 × 1.4 × 1.4cm in diameter. Microsurgical biopsy verified a necrotizing noncaseating epithelioid cell tumor indicative for neurosarcoidosis. All symptoms dissolved within 3 months under stringent corticoid therapy. Conclusion Intracranial mass lesions as the primary and only manifestation of neuronal sarcoidosis are rare. Because conservative treatment is safe and effective, surgery is limited to biopsy and the alleviation of pressure-related symptoms to preserve neurologic function.

[Coronary atherosclerosis and progression to unstable plaques : Histomorphological and molecular aspects]. / Arteriosklerose der Koronararterien und Plaque-Progression : Histomorphologische und molekularbiologische Aspekte.

Atherosclerosis causes clinical symptoms through luminal narrowing by stenosis or by precipitating thrombi that obstruct blood flow to the myocardium (coronary artery disease), central nervous system (ischemic stroke) or lower extremities (peripheral vascular disease). The most common of these manifestations of atherosclerosis is coronary artery disease, clinically presenting as either stable angina or acute coronary syndromes. Atherosclerosis is a mainly lipoprotein-driven disease, which is associated with the formation of atherosclerotic plaques at specific sites of the vascular system through inflammation, necrosis, fibrosis and calcification. In most cases, plaque rupture of a so-called thin-cap fibroatheroma leads to contact of the necrotic core material of the underlying atherosclerotic plaque with blood, resulting in the formation of a thrombus with acute occlusion of the affected (coronary) artery. The atherosclerotic lesions that can cause acute coronary syndromes by formation of a thrombotic occlusion encompass (1) thin-cap fibroatheroma, (2) plaque erosion and (3) so-called calcified nodules in calcified and tortuous arteries of aged individuals. The underlying pathomechanisms remain incompletely understood so far. In this review, the mechanisms of atherosclerotic plaque initiation and progression are discussed.

[Novel molecular aspects of chordomas]. / Neue molekulare Aspekte der Chordome.

Chordomas are rare and slowly growing malignant bone tumors which mostly occur in adults. These bone tumors are characterized by epithelial and mesenchymal aspects. It is suggested that they arise from remnants of the notochord because they are found along the axial skeleton (e.g. clival, spinal and sacrococcygeal locations). It appears that cytogenetic aberrations are not randomly found in this tumor group. Loss of chromosomal material (e.g. 1p, 3p, 10q, 13q and 14q) is more frequently found than gain of material (e.g. 7q, especially 7q33). Several studies demonstrated brachyury expression (T; 6q27) as a possible candidate gene in the oncogenesis of chordomas (e.g. knock down in the chordoma cell line U-CH1). So far therapy consists of complete resection and irradiation, e.g. with carbon ions. Targeting therapy is not yet established in routine protocols but phase II studies with tyrosine kinase inhibitors have shown partial response of tumors and, in some studies stabilization of the disease has been described.