A 25-year-old female patient complained of intermittent pseudoradicular, ulnar-accentuated tingling paraesthesias of the right arm, accompanied by swelling and cyanotic skin discolouration of the forearm 9 days following plate osteosynthesis of a clavicle shaft fracture in the middle third (OTA classification type B). CT angiography revealed almost complete obstruction of the lumen of the subclavian vein at the junction between the clavicle and the first rib, which was consistent with a thoracic inlet syndrome. This was triggered by a haematoma of the concomitantly fractured first rib and a cortical screw protrusion in the area of the constriction between the clavicle and the first rib. Revascularisation was achieved by evacuating the fracture haematoma, partial resection of the first rib, and replacement of the two protruding screws. This case demonstrates that screw protrusion must be avoided during plate osteosynthesis of clavicle shaft fractures, particularly in the medial third of the clavicle – due to its proximity to the first rib. Injuries to the subclavicular neurovascular bundle can be comprehensively detected and treated by resection of the first rib.
Forensic age estimation is essential for legal and social decision-making when reliable documentation is lacking. Traditionally, ossification of the medial clavicular epiphysis (MCE) is assessed by visual staging, but norm variants frequently limit classic systems and introduce error and irreproducibility. High-resolution computed tomography (CT) allows for quantitative morphometric assessment, potentially offering support – especially in such cases. Based on the approach of Hua et al. (2014) an open-source workflow for metric age estimation of the medial clavicles using semi-automatic three-dimensional (3D) CT segmentation was developed. Clinical CT scans were pseudonymized, archived in XNAT (Extensible Neuroimaging Archive Toolkit), and 3D models were generated in 3D Slicer. Expert-guided segmentation and alignment enabled extraction of quantitative parameters including planar areas and volumes of epiphyses and metaphyses; area and volume ratios were calculated as dimensionless metrics. It was concluded that morphometric assessment of the medial clavicles via 3D imaging is a promising approach for forensic age estimation. The workflow’s open-source architecture supports transparency and collaborative validation. Future research should validate metric markers and pursue workflow automation, particularly to address anatomically complex cases.
e23328 Background: Musculoskeletal sarcomas are rare and aggressive malignancies requiring multidisciplinary care, which frequently is not available in low- and middle-income countries (LMICs). In Armenia, the centralization of pediatric cancer care in 2019 at the Pediatric Cancer and Blood Disorder Center (PCBDC) marked a crucial turning point with the establishment of a multidisciplinary team (MDT). This was supported by a collaboration with the University Hospital of Münster in Germany. Our study aims to highlight the impact of these initiatives on improving sarcoma care in a resource-limited setting. Methods: A retrospective analysis of MDT meetings from 2019 to 2024 was conducted, reviewing data from 193 cases discussed during 85 meetings. After centralization (2019-2024) the data on patients aged 0-25 years was compared with a historical cohort from three Armenian hospitals (2008-2019) taking care of sarcoma patients before 2019, assessing changes in diagnostic accuracy, treatment practices, and outcomes. Results: The MDT reviewed 193 cases, including 80 pediatric cases (≤18 years). Treatment adherence to international guidelines improved from 49.0% before 2019 to 79.1% after 2019 (p = 0.0001). The review of paraffin blocks increased from 24.0% before 2019 to 88.4% after (p = 0.0001). Of 64 paraffin blocks sent internationally, 34% resulted in diagnostic changes. For the whole cohort, the 3-year overall survival (OS) rates were 60.0% for patients under 18 years and 47.4% for those aged 18 and above. OS was 66.7% for localized disease, compared to 27% for metastatic cases (p = 0.0001). Bone sarcomas had a 3-year OS of 52.4%, while soft tissue sarcomas had 61.0%. The treatment abandonment rate decreased from 22.0% before 2019 to 10.3% after 2019. When comparing 3-year OS before and after centralization, the rates were 45% and 50%, respectively. Among patients who did not abandon treatment, the 3-year OS was 48.7% before centralization and 57.6% after. Conclusions: This study highlights centralized care and collaboration as the lynchpin of advancing musculoskeletal sarcoma care, exemplified by the establishment of PCBDC and the Armenian-German partnership, which have significantly improved outcomes and management of these patients.
Thorakolithiasis ist eine seltene gutartige Erkrankung. Sie manifestiert sich als ein oder mehrere pleuraständige Rundherde. Häufig sind die Patienten asymptomatisch. Die Differentialdiagnose gelingt auf Grund von Mobilität mit Lageänderung in der Bildgebung und von Verkalkungen. Differentialdiagnosen von verkalkten Thorakolithen sind Granulome, Hamartome, Teratome, kalzifizierte Metastasen, verkalkte Pleuraplaques und Gallensteine im Pleuraraum. Nicht kalzifizierte Herde können wie Pleuratumore und Metastasen imponieren. Die Ätiologie ist unbekannt. Es gibt jedoch verschiedene Hypothesen für die Entstehung u.a. perikardiale Fettnekrosen, Reibung in der Pleurahöhle, infektiöse Genese sowie eine Ansammlung von Staub phagozytierenden Makrophagen.
Pertrochanteric femoral fractures are among the most common injuries in geriatric patients. Intramedullary implants are used to restore patient mobility. We report coil embolisation of an aneurysma spurium of the profunda femoral artery as a complication after distal nail locking of a proximal femoral nail.
ZusammenfassungDie pertrochantäre Femurfraktur ist eine der häufigsten Verletzungen des geriatrischen Patienten. Goldstandard ist die operative Therapie mittels sofort vollbelastungsstabiler Osteosynthese bspw. durch intramedulläre Implantate. In dieser Kasuistik wird die Ausbildung eines Aneurysma spurium der A. profunda femoris als Komplikation der distalen Verriegelung eines proximalen Femurnagels und die interventionell-radiologische Therapie mittels Coil-Embolisation beschrieben.
Cause-specific analysis of the treatment effect on the different component of the event-free survival since randomization.
AbstractPurpose: The phase III, open-label, prospective, multicenter, randomized Ewing 2008R1 trial (EudraCT2008-003658-13) was conducted in 12 countries to evaluate the effect of zoledronic acid (ZOL) maintenance therapy compared with no add-on regarding event-free survival (EFS, primary endpoint) and overall survival (OS) in standard-risk Ewing sarcoma (EWS). Patients and Methods: Eligible patients had localized EWS with either good histologic response to induction chemotherapy and/or small tumors (<200 mL). Patients received six cycles of VIDE induction and eight cycles of VAI (male) or eight cycles of VAC (female) consolidation. ZOL treatment started parallel to the sixth consolidation cycle. Randomization was stratified by tumor site (pelvis/other). The two-sided adaptive inverse–normal four-stage design (planned sample size 448 patients, significance level 5%, power 80%) was changed after the first interim analysis using the Müller–Schäfer method. Results: Between April 2010 and November 2018, 284 patients were randomized (142 ZOL/142 no add-on). With a median follow-up of 3.9 years, EFS was not significantly different between ZOL and no add-on group in the adaptive design (HR, 0.74; 95% CI, 0.43–1.28, P = 0.27, intention-to-treat). Three-year EFS rates were 84.0% (95% CI, 77.7%–90.8%) for ZOL vs. 81.7% (95% CI, 75.2%–88.8%) for no add-on. Results were similar in the per-protocol collective. OS was not different between groups. The 3-year OS was 92.8% (95% CI, 88.4%–97.5%) for ZOL and 94.6% (95% CI, 90.9%–98.6%) for no add-on. Noticeable more renal, neurologic, and gastrointestinal toxicities were observed for ZOL (P < 0.05). Severe renal toxicities occurred more often in the ZOL arm (P = 0.003). Conclusions: In patients with standard-risk localized EWS, there is no additional benefit from maintenance treatment with ZOL.
Background It is necessary to find a careful and reliable medical procedure for forensic age estimation in the living in order to correctly assess age in potentially underage unaccompanied refugees, so that actual unaccompanied minors may receive their deserved judicial privileges. Objective Design and first results of a study concerning forensic age estimation by low-field magnetic resonance imaging (MRI) are presented. Material and methods Prospective examinations of 650 study participants are planned, evenly distributed in groups of 25 individuals per sex across the age bracket of 12-24 years. The developmental stages of distal radius, distal ulna, distal femur and proximal tibia are to be examined using a 0.31 Tesla (T) low-field MRI scanner, and to then be assessed by applying the Vieth classification. Results After assessing the 487 study participants examined to date, it is apparent that the chosen sequences yield the needed information about the ossification of the epiphyses of interest to enable the application of the Vieth classification. The examination procedures are also proving to be smooth and uneventful. Conclusion Forensic age estimation in the living by means of 0.31 T low-field MRI scanners using the Vieth classification is feasible. It is yet to be decided, whether the characteristics of the ossification of the wrist and knee joints allow for a reliable proof of majority beyond reasonable doubt.
EinleitungDie interdisziplinäre Zusammenarbeit war in der Medizin schon immer von enormer Wichtigkeit. Der Wissensfortschritt in der modernen Medizin inklusive des unaufhaltsamen Trends zu Subspezialisierungen macht es für den jeweiligen Facharzt heutzutage unmöglich, die jeweiligen „Partnerdisziplinen“ erschöpfend zu überblicken. Das in der Vergangenheit erlangte Studienwissen bezüglich anderer Fachgebiete ist in vielen Bereichen nicht mehr aktuell. Somit bestand und besteht keine Alternative zu interdisziplinärer Zusammenarbeit. Durch diese entsteht zum einen ein Mehrwert für den behandelnden Arzt, zum anderen – viel wichtiger – für die zu behandelnden Patienten.
Zusammenfassung Hintergrund Ein rechtlich zulässiges medizinisches Altersfeststellungsverfahren mit möglichst schonenden und zuverlässigen Methoden bei unbegleiteten und fraglich minderjährigen Ausländern ist erforderlich, um den tatsächlich Minderjährigen den für sie gesetzlich vorgesehenen besonderen Schutz zukommen zu lassen. Ziel der Arbeit Studiendesign und erste Ergebnisse einer Studie zur forensischen Altersdiagnostik mittels Niederfeld-Magnetresonanztomographie werden vorgestellt. Material und Methoden Geplant ist die prospektive Untersuchung von 650 Studienteilnehmenden, gleichmäßig verteilt innerhalb der Altersgruppe 12 bis 24 Jahre zu je 25 Personen je Geschlecht. Es sollen Entwicklungsstadien von distalem Radius, distaler Ulna, distalem Femur und proximaler Tibia mithilfe eines 0,31-Tesla(T)-Niederfeld-MRT-Scanners erfasst und mittels Anwendung der Vieth-Klassifikation ausgewertet werden. Ergebnisse Nach Auswertung der Untersuchungen der bisher 487 untersuchten Studienteilnehmenden ist ersichtlich, dass die gewählten Sequenzen die für die Vieth-Klassifikation relevanten Charakteristika der Ossifikation der untersuchten Epiphysen erfassen. Die Untersuchungen erweisen sich überdies als einfach in der Durchführung. Schlussfolgerung Untersuchungen zur forensischen Altersschätzung am Lebenden unter Anwendung der Vieth-Klassifikation sind mit Niederfeld-MRT-Scannern bei einer Feldstärke von 0,31 T durchführbar. Es bleibt zu klären, ob die mit einem 0,31-T-Niederfeld-MRT-Scanner nachweisbaren Ossifikationsmerkmale am Handgelenk und am Knie einen zweifelsfreien Volljährigkeitsnachweis erlauben.
Zusammenfassung Einleitung Die interdisziplinäre Zusammenarbeit war und ist insbesondere in der Gegenwart – bedingt durch den enormen Wissensfortschritt in der Medizin – von herausragender Bedeutung. Die Diagnostik und Therapie von benignen und malignen Knochentumoren stellt alle beteiligten Fachdisziplinen aufgrund der Seltenheit dieser Tumoren häufig vor Herausforderungen. Das Ziel dieses Artikels ist es, die Bedeutung der Interdisziplinarität in der Diagnosefindung von Knochentumoren herauszuarbeiten. Methoden Es handelt sich um eine Übersichtsarbeit zur Bedeutung der Interdisziplinarität bei der Diagnostik von Knochentumoren. Aufgrund der nur umschriebenen Literatur zu diesem Thema veranschaulichen wir anhand von Fallbeispielen die Notwendigkeit der interdisziplinären Zusammenarbeit. Ergebnisse und Diskussion Dieser Artikel verdeutlicht, dass eine interdisziplinäre klinisch-radiologische Betrachtung von „leave-me-alone lesions“ und eine klinisch-radiologisch-pathologische Korrelation von biopsierten Knochentumoren erfolgen muss. Alle Befunde müssen im Rahmen einer gemeinsamen interdisziplinären Konferenz in ein diagnostisches Gesamtbild eingefügt werden, sodass nicht selten die Diagnose am Ende interdisziplinär erarbeitet wird. Schlussfolgerung Für den „Kliniker“ ist es unabdingbar zu wissen, dass die Diagnose eines Knochentumors oft nicht allein durch den Pathologen gestellt werden kann und zwingend der interdisziplinären Zusammenarbeit bedarf.
INTRODUCTION:Due to substantial advances in all medical disciplines, interdisciplinary cooperation is of major relevance in modern medicine. Given the rarity of benign and malignant bone tumours, diagnosis and therapy of these lesions is especially challenging. Focusing on typical cases, it is the aim of this article to illustrate the necessity of interdisciplinary cooperation. METHODS:The scope of this article is to highlight the particular significance of interdisciplinarity in the diagnosis of bone tumours. To this end, we illustrate the interdisciplinary approach in typical clinical cases. RESULTS AND DISCUSSION:The article demonstrates that a combined clinico-radiological approach is essential in the diagnosis of leave-me-alone lesions, and it underlines the essential role of an interdisciplinary correlation of clinical context, radiology and pathology in the interpretation of bone tumour biopsies. It documents the experience that all clinical aspects and diagnostic findings need to be synoptically integrated in a joint interdisciplinary tumour board to eventually find the correct diagnosis. CONCLUSION:It is generally accepted that the diagnosis of a bone tumour can often not be made by the pathologist alone but essentially requires interdisciplinary cooperation.
In order to find a reliable method to correctly assess majority in both sexes by MRI, a study was conducted to evaluate the applicability of the recently presented Vieth classification in wrist MRI, after it had originally been proposed for knee MRI. After receiving a positive vote by the ethics committee, the left-hand wrists of 347 male and 348 female volunteers of German nationality in the age bracket 12–24 years were scanned. Before conducting the prospective, cross-sectional examinations, an informed consent was obtained from each volunteer. A 3.0 T MRI scanner was used, acquiring a T1 turbo spin-echo sequence (TSE) and a T2 TSE sequence with fat suppression by spectral presaturation with inversion recovery (SPIR). The images were assessed by applying the Vieth classification. Minimum, maximum, mean ± standard deviation, and median with lower and upper quartiles were defined. Intra- and interobserver agreements were determined by calculating the kappa coefficients. Differences between the sexes were analyzed using the Mann-Whitney U test. By applying the unmodified Vieth classification with corresponding schematics, it was possible to assess majority in both sexes via the epiphyseal-diaphyseal fusion of the distal radius and in males also via the epiphyseal-diaphyseal fusion of the distal ulna. The Mann-Whitney U test implied significant sex-related differences for all stages. For both epiphyses, the intra- and interobserver agreement levels were very good (κ > 0.8). If confirmed by further studies, it would be possible to determine the completion of the 18th year of life in both sexes by 3.0 T MRI of the wrist and using the Vieth classification. • The Vieth classification allows determining majority in males and females alike based on the distal radius’ epiphysis by 3.0 T MRI of the wrist. • The Vieth classification also allows determining majority in males based on the distal ulna’s epiphysis by 3.0 T MRI of the wrist, but not in females. • The presented data can be deemed referential within certain discussed boundaries.
In der WHO-Klassifikation der Knochentumoren aus dem Jahre 2020 ist es zu einigen Änderungen gekommen. Konzeptionell wurden die klein-rundzelligen Sarkome in einem gesonderten Kapitel zwischen den Weichgewebs- und Knochentumoren gebündelt. Aufgrund neuerer molekularer Befunde wurden einzelne diagnostische Kategorien wie die der fibrohistiozytären Tumoren aufgegeben, andererseits wurden neue Entitäten in die Klassifikation mit aufgenommen. Schließlich wurden systematische Änderungen in der Terminologie der Klassifikation kartilaginärer Tumoren vorgenommen, und die Bewertung des biologischen Verhaltens einiger Läsionen wurde modifiziert. Diese Übersichtsarbeit stellt die wesentlichen Änderungen mit Fokus auf die bei der Diagnostik und Therapie ossärer Prozesse elementare interdisziplinäre Zusammenarbeit zusammen.
Ewing sarcoma, a highly aggressive bone and soft-tissue cancer, is considered a prime example of the paradigms of a translocation-positive sarcoma: a genetically rather simple disease with a specific and neomorphic-potential therapeutic target, whose oncogenic role was irrefutably defined decades ago. This is a disease that by definition has micrometastatic disease at diagnosis and a dismal prognosis for patients with macrometastatic or recurrent disease. International collaborations have defined the current standard of care in prospective studies, delivering multiple cycles of systemic therapy combined with local treatment; both are associated with significant morbidity that may result in strong psychological and physical burden for survivors. Nevertheless, the combination of non-directed chemotherapeutics and ever-evolving local modalities nowadays achieve a realistic chance of cure for the majority of patients with Ewing sarcoma. In this review, we focus on the current standard of diagnosis and treatment while attempting to answer some of the most pressing questions in clinical practice. In addition, this review provides scientific answers to clinical phenomena and occasionally defines the resulting translational studies needed to overcome the hurdle of treatment-associated morbidities and, most importantly, non-survival.
Osteoma is a benign bone forming tumor predominantly arising on the surface of craniofacial bones. While the vast majority of osteomas develops sporadically, a small subset of cases is associated with Gardner syndrome, a phenotypic variant of familial adenomatous polyposis caused by mutations in the APC gene resulting in aberrant activation of WNT/β-catenin signaling. In a sequencing analysis on a cohort of sporadic, non-syndromal osteomas, we identified hotspot mutations in the CTNNB1 gene (encoding β-catenin) in 22 of 36 cases (61.1%), harbouring allelic frequencies ranging from 0.04 to 0.53, with the known S45P variant representing the most frequent alteration. Based on NanoString multiplex expression profiling performed in a subset of cases, CTNNB1-mutated osteomas segregated in a defined "WNT-cluster", substantiating functionality of CTNNB1 mutations which are associated with β-catenin stabilization. Our findings for the first time convincingly show that osteomas represent genetically-driven neoplasms and provide evidence that aberrant WNT/β-catenin signaling plays a fundamental role in their pathogenesis, in line with the well-known function of WNT/β-catenin in osteogenesis. Our study contributes to a better understanding of the molecular pathogenesis underlying osteoma development and establishes a helpful diagnostic molecular marker for morphologically challenging cases.
While the prognosis of patients with Ewing sarcoma (EwS) is improving, little is known about the frequency of pain and its risk factors in survivors of EwS. This study aims to analyse the prevalence and risk factors of pain and its predictive value for recurrence.