Primary alveolar rhabdomyosarcoma of the bone: two cases and review of the literature

BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. The anatomical localization of the tumor may vary, but commonly involve the head/neck regions, male and female urogenital tract or the trunk and extremities. CASE PRESENTATION: Here, we report two challenging cases involving 17- and 9-years-olds males where diffuse and multiplex bone lesions suggested either a hematological disease or a primary bone tumor (mesenchymal chondrosarcoma). Biopsies, proved a massive infiltration of the bone marrow cavity with rhabdomyosarcoma. In both cases, the ARMS subtype was confirmed using FOXO1 break-apart probes (FISH). Radiological examination could not identify primary soft tissue component in any localization at the time of diagnosis in either cases. CONCLUSIONS: Primary alveolar rhabdomyosarcoma of the bone as a subtype of ARMS, seems to be a distinct clinico-pathological entity with challenging diagnostic difficulties and different, yet better, biological behavior in comparison to soft tissue ARMS. However, it is difficult to be characterized or predict its prognosis and long-term survival as only sporadic cases (four) were reported so far

SURFACE LAYER’S SOUND SPEED PROFILES: CLIMATOLOGICAL ANALYSIS AND APPLICATION FOR THE CNOSSOS-EU NOISE MODEL

Noise pollution and exposure are important environmental issues that need to be investigated and regulated. To do this,we need to know about micrometeorology to figure out how noise travels from the source to the receiver. Accordingly, the sound propagation part of the common noise assessment methods (CNOSSOS-EU) developed by the European Commission for different sources of noise needs detailed meteorological databases. Using data from the SYNOP stations maintained by the Hungarian Meteorological Service (HMS) and the ERA5 meteorological reanalysis database, the standard noise propagation conditions are determined. The primary objective of this study is to ascertain the probability distribution of stability classes for a variety of source-receiver orientations, utilizing either 25 or 2 stability classes, and several different aggregation levels. Relative frequencies and year-to-year variability have been calculated for favourable noise propagation conditions where the sound speed profile grows with height (downward refraction condition) and unfavourable noise propagation conditions where the sound speed profile constant or decreases with height (so-called homogeneous conditions). Favourable noise propagation occurs in approximately one-third of cases during the daytime while in approximately two-third of cases during the evening and night-time where the noise exposure is increasing. Furthermore, using the SoundPLANnoise software, sound propagation model calculations were performed on a study area near Budapest, using different values of parameter pf describing the probability of occurrence of favourable conditions on sound propagation during different periods of the day. This area is crossed by Highway 4, which is a major road according to the 49/2002 EU Directive, as it has more than three million vehicles passing on the examined section every year. The results show considerable deviations in annual average A-weighted sound levels calculated using different versions of parameter pf. The largest difference between the A-weighted sound levels calculated with the highest and lowest generated annual pf values was 1.65 dB(A); 1.42 dB(A) and 0.75 dB(A) for day, evening and night periods, respectively.&nbsp

Complete recovery from psychosis upon miglustat treatment in a juvenile Niemann-Pick C patient

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A terc-amino effektus vizsgálata és kiterjesztése: új típusú, biológiailag aktív policiklusos vegyületek előállítása = The study and the extension of the tert-amino effect: Synthesis of new types of polycyclic compounds with biological activities

1. Vizsgáltuk a terc-amino effektus 2. típusának kiterjesztését a reakcióban résztvevő vinil és terc-amino csoportot két külön gyűrűn tartalmazó (kondenzált) bi- és triaril - bifenil/fenilpiridazin, trifenil/difenilpiridazin, naftalin illetve fenilnaftalin - modellvegyületekre; a gyűrűzárási reakciók révén új azepin-, azocin-, azonin- és azecin-anellált gyűrűrendszereket állítottunk elő. E munkánk révén a terc-amino effektus az eddigi ismeretek szerinti alkalmazását lényegesen bővítettük. 2. Antimaláriás hatásirányban nemzetközi együttműködésben vizsgáltuk azocinszármazékok, illetve indolokinolin alkaloidok bi- és triciklusos analógjainak aktivitását. Ez utóbbiak közül két származék is jelentős in vitro aktivitást mutatott. 3. SSAO hatásirányban vizsgáltuk a terc-amino effektussal nyert diciano-szubsztituált azaheterociklusokból előállított aminometil származékok, illetve további vegyületcsaládok (oxim típusú vegyületek, aminometil-piridazinok) aktivitását; együttműködésben kiválasztott SSAO-gátlók további in vivo vizsgálatára került sor gyulladásos modelleken. E vegyületek egyike (SZV-1287) mind in vitro, mind in vivo jelentős SSAO-gátló és gyulladáscsökkentő hatást fejt ki; fejlesztéséről a közeljövőben döntünk. | 1. Novel extensions of the type 2 tert-amino effect have been studied to compounds having the interacting vinyl and tert-amino moieties on two different rings. Namely, cyclizations of bi- and triaryl - biphenyl/phenylpyridazine, triphenyl/diphenylpyridazine, napthalene and phenylnapthalene - model systems were studied, affording novel azepine-, azocine-, azonine- and azecine-fused ring systems. Our studies facilitate novel applications of the tert-amino effect as compared to cyclizations described thus far. 2. In the frame of an international collaboration, the antimalarial activity of azocine derivatives, and bi- and tricyclic analogues of indoloquinoline alkaloids were studied. Two representatives of the latter group exhibited substantial in vitro activity. 3. SSAO activity of aminomethyl derivatives obtained from dicyano-substituted azaheterocycles synthesized via tert-amino effect cyclization and further classes of compounds (oxime derivatives, aminomethyl-pyridazines) were studied; in collaboration in vivo activity of selected SSAO inhibitors were studied in inflammatory models. On selected derivative (SZV-1287) exhibited substantial in vitro and in vivo SSAO inhibitor and anti-inflammatory activity; we are to decide on its further development