The diagnosis, morphological particularities, and surgical technique in a case of intravascular leiomyoma extended to the right heart chambers

Intravenous leiomyoma is a benign smooth muscle cell tumor of uterine origin that may grow into the pelvic veins and the inferior vena cava. It usually affects premenopausal women and the majority (90%) are parous. Because cardiac involvement is present in up to 10% of cases, it may be misdiagnosed as a primary cardiac tumor or a venous thrombus-in-transit. We describe a case of intravascular leiomyomatosis with cardiac extension and the morphological particularities of the removed tumor

Comparative Endoscopic Anatomic Description of the Mitral Valvular Complex: a Cadaveric Study

Background We compared the aortic, left atrial, and apical approaches to visualize the mitral valve with the goal to investigate the endoscopic anatomy and give exact step-by-step descriptions of these views. Materials and Methods The mitral valvular complex of human cadaveric fresh hearts was investigated from three approaches using 0, 30, and 70 degrees rigid endoscopic optics. In 30 cases after the removal of the hearts, the endoscopes were introduced directly into the aortic root through an aortotomy, left atrium through a standard atriotomy, and apex of the heart through a transmural incision. In 10 cases, the in situ visualization was performed using standard surgical approaches, such as partial upper ministernotomy, right and left minithoracotomy. The investigation was performed first with the mitral valve open, then the left ventricle was filled with saline, and the valve was closed by clamping the aorta. Results For the visualization of ventricular surfaces of the mitral leaflets and the subvalvular apparatus, the apical approach was most optimal. The aortic approach had limitations at the posterior leaflet. Using the atrial approach, we did not obtain any direct visual information about the subvalvular apparatus with the valve closed. The atrial surfaces of the leaflets were best visible using both the atrial and apical approaches with the mitral valve open. In the case of a closed valve, the apical approach did not allow for an investigation of the atrial surfaces. The aortic approach was useful to visualize the atrial surface of the posterior leaflet with an opened valve. Conclusion In mitral valve repairs through the left atrium, an additional aortic or apical view could be useful to obtain functional information about the subvalvular apparatus by the sealing probe

Ritka hasűri vérzés a vena lienalis ágrendszeréből várandósság alatt

Az „akut has”, mint kórkép, a várandósság során mind diagnosztikailag, mind terápiásan nagy kihívást jelent a szülész számára a megváltozott anatómiai viszonyok és a képalkotó eljárások korlátozott használhatósága miatt. Közleményünkben bemutatjuk egy harmadik trimeszterbeli gravida hasi vérömlenyének az esetét, melynek vérzésforrása irodalmi ritkaság, a vérzés típusát a felkereshető irodalomban még nem írták le terheseknél. Az esettanulmány megírása a páciens orvosi dokumentációi és az ellátásában résztvevő klinikusok, patológusok leletei és beszámolói alapján történt. A várandósságának 33. hetében járó gravida akut hasi tünetegyüttessel jelentkezett klinikánkon, anamnéziséből trauma kizárható volt. Az anya romló kardiorespiratorikus állapota miatt sürgősségi császármetszéssel élő, koraszülött magzatot segítettünk világra. A műtét során kb. 1500 ml vért találtunk a hasüregben, melynek okát általános sebész bevonásával kerestük. Aktív vérzésforrásként a lép ereinek területéről vénás jellegű szivárgó vérzést találtunk, ezért splenectomia mellett döntöttünk. Az eltávolított szervkomplexum teljes szövettani feldolgozása helyenként a kisebb léphilusi vénaágak izomfalának felrostozódását és fokozatos eltűnését igazolta, a patológus a vérzés okát a meggyengült érfal endothelen keresztüli vérszivárgásának véleményezte, definitív érfalszakadásra utaló elváltozás nem volt azonosítható. A haemoperitoneum várandósság alatt kifejezetten ritka kórkép, azonban mortalitása mind az anyára, mind a magzatra nézve rendkívül magas. Irodalmi adatok alapján a vérzésforrás leggyakrabban a kismedencei szervek területén jelentkezik. Ritkán a lépartéria aneurysmájának rupturája is előfordul terhességben, a lépvénás eredetű vérzés viszont irodalmi ritkaságnak számít, és az összes feldolgozott és közölt esetben az ér szakadásos sérülése volt a fő ok, az esetünkben leírt, ismeretlen etiológiájú, érfalvesztéses hemorrhágiát sehol nem említik. A várandósunk ellátásánál ellátásánál kulcsfontosságú volt a gyorsan elvégzett exploratív laparotomia, mely a kórkép okát felderítette és megszüntette, lehetővé téve az anya és gyermeke túlélését és felépülését. Esetünk rámutat arra, hogy a várandóssági hasűri vérömlenyek kóroktana akár irodalmi ritkaságokat is rejthet, azaz a pontos ok kiderítésével, lumenes vérzésforrás keresésével nem szabad szükségtelen időt veszteni az édesanya és gyermeke életmentő ellátásának rovására

Spatially Resolved Proteomic and Transcriptomic Profiling of Anaplastic Lymphoma Kinase-Rearranged Pulmonary Adenocarcinomas Reveals Key Players in Inter- and Intratumoral Heterogeneity

Pulmonary adenocarcinomas (pADCs) with an ALK rearrangement are a rare cancer subtype, necessitating comprehensive molecular investigations to unravel their heterogeneity and improve therapeutic strategies. In this pilot study, we employed spatial transcriptomic (NanoString GeoMx) and proteomic profiling to investigate seven treatment-naïve pADCs with an ALK rearrangement. On each FFPE tumor slide, 12 smaller and 2-6 larger histopathologically annotated regions were selected for transcriptomic and proteomic analysis, respectively. The correlation between proteomics and transcriptomics was modest (average Pearson's r = 0.43 at the gene level). Intertumoral heterogeneity was more pronounced than intratumoral heterogeneity, and normal adjacent tissue exhibited distinct molecular characteristics. We identified potential markers and dysregulated pathways associated with tumors, with a varying extent of immune infiltration, as well as with mucin and stroma content. Notably, some markers appeared to be specific to the ALK-driven subset of pADCs. Our data showed that within tumors, elements of the extracellular matrix, including FN1, exhibited substantial variability. Additionally, we mapped the co-localization patterns of tumor microenvironment elements. This study represents the first spatially resolved profiling of ALK-driven pADCs at both the gene and protein expression levels. Our findings may contribute to a better understanding of this cancer type prior to treatment with ALK inhibitors

Region Specific Differences of Claudin-5 Expression in Pediatric Intracranial Ependymomas: Potential Prognostic Role in Supratentorial Cases

Ependymomas are common pediatric brain tumors that originate from the ependyma and characterized by poor prognosis due to frequent recurrence. However, the current WHO grading system fails to accurately predict outcome. In a retrospective study, we analyzed 54 intracranial pediatric ependymomas and found a significantly higher overall survival in supratentorial cases when compared to infratentorial tumors. Next we performed region-specific immunohistochemical analysis of the ependyma in neonatal and adult ependyma from the central canal of spinal cord to the choroid plexus of lateral ventricles for components of cell-cell junctions including cadherins, claudins and occludin. We found robust claudin-5 expression in the choroid plexus epithelia but not in other compartments of the ependyma. Ultrastructural studies demonstrated distinct regional differences in cell-cell junction organization. Surprisingly, we found that 9 out of 20 supratentorial but not infratentorial ependymomas expressed high levels of the brain endothelial tight junction component claudin-5 in tumor cells. Importantly, we observed an increased overall survival in claudin-5 expressing supratentorial ependymoma. Our data indicates that claudin-5 expressing ependymomas may follow a distinct course of disease. The assessment of claudin-5 expression in ependymoma has the potential to become a useful prognostic marker in this pediatric malignancy

Ki67 index is an independent prognostic factor in epithelioid but not in non-epithelioid malignant pleural mesothelioma: a multicenter study

BACKGROUND: Estimating the prognosis in malignant pleural mesothelioma (MPM) remains challenging. Thus, the prognostic relevance of Ki67 was studied in MPM. METHODS: Ki67 index was determined in a test cohort of 187 cases from three centres. The percentage of Ki67-positive tumour cells was correlated with clinical variables and overall survival (OS). The prognostic power of Ki67 index was compared with other prognostic factors and re-evaluated in an independent cohort (n=98). RESULTS: Patients with Ki67 higher than median (>15%) had significantly (P<0.001) shorter median OS (7.5 months) than those with low Ki67 (19.1 months). After multivariate survival analyses, Ki67 proved to be-beside histology and treatment-an independent prognostic marker in MPM (hazard ratio (HR): 2.1, P<0.001). Interestingly, Ki67 was prognostic exclusively in epithelioid (P<0.001) but not in non-epithelioid subtype. Furthermore, Ki67 index was significantly lower in post-chemotherapy samples when compared with chemo-naive cases. The prognostic power was comparable to other recently published prognostic factors (CRP, fibrinogen, neutrophil-to-leukocyte ratio (NLR) and nuclear grading score) and was recapitulated in the validation cohort (P=0.048). CONCLUSION: This multicentre study demonstrates that Ki67 is an independent and reproducible prognostic factor in epithelioid but not in non-epithelioid MPM and suggests that induction chemotherapy decreases the proliferative capacity of MPM

Sessilis recidív rectumpolip miatt végzett endoszkópos submucosus dissectio. Esetismertetés és irodalmi áttekintés = Removal of sessile recidiv rectal polyp with endoscopic submucosal dissection. Case report and review of the literature

Az intraluminalis invazív endoszkópia fejlődésével a nagy, sessilis polipok, a muscularis mucosa rétegét nem meghaladó malignus tumorok eltávolítása jelent napjainkban kihívást. A korábban ilyen indikációval végzett mucosectomiák különböző formáinál nehézséget okozott a mélységi és laterális reszekciós szélek kontrollja, a 20 mm-nél nagyobb elváltozások pedig csak piecemealtechnikával voltak eltávolíthatók. Beteganyag: 64 éves nőbeteg nagy, korábban piecemealtechnikával, majd mucosectomiával eltávolított sessilis rectumpolipjának helyén észlelt recidív elváltozás eltávolítását végeztük endoszkópos submucosus dissectiót alkalmazva. Eredmények: Az 55 percig tartó beavatkozás során csak minimális vérzés jelentkezett. A beavatkozás során szövődményt nem észleltünk, a beteg egynapos obszerváció után panaszmentesen távozott. A szövettani vizsgálat szerint az elváltozás eltávolítása vertikálisan és laterálisan is az épben történt. Következtetés: Az endoszkópos submucosus dissectio olyan elváltozások radikális eltávolítására is alkalmas, melyek a korábban alkalmazott technikákkal nem voltak sikeresen reszekálhatók. | With the development of flexible endoscopy, removal of the large sessile polyps and superficial malignant tumors that do not exceed the layer muscular mucosa has become today’s major challenge. Earlier in various types of mucosectomy performed with such indication it was difficult to control the depth and the lateral margin of the resection surface. Tumors larger than 20 mm could only be removed with the application of the piecemeal technique. Patient and method: 64-year-old female patient’s large sessile polyp had been removed earlier with piecemeal technique followed by mucosectomy. On the area of these interventions a recidiv adenoma was found and for this reason endoscopic submucosal dissection was applied. Result: The procedure took 55 minutes, and only small volume of bleeding was detected during the intervention. The postoperative period was uneventful, one day later the patient left the hospital. Vertical and lateral resection surface were free of tumor histologically. Conclusion: The endoscopic submucosal dissection is suitable for the removal of the large sessile polyp, which could not be successfully removed with earlier techniques