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ZusammenfassungDie interdisziplinäre Forschungskooperation COACH-Konsortium (Chronic Conditions in Adolescents – Implementation and Evaluation of Patient-centered Collaborative Healthcare) untersucht die psychosoziale Situation von Jugendlichen und jungen Erwachsenen mit chronischen körperlichen Erkrankungen. Zur Untersuchung der psychischen Komorbidität wurden bisher 1.023 Patienten mit Diabetes mellitus Typ 1 im Alter von 12–21 Jahren bei Routinevorstellungen in der Klinik bzw. Ambulanz mittels der Screening-Fragebogen Patient Health Questionnaire (PHQ-9) und Generalized Anxiety Disorder Scale-7 (GAD-7) zu Angst- und Depressionssymptomen befragt. 29,8 % der Jugendlichen und jungen Erwachsenen zeigten ein auffälliges Screening-Ergebnis. Dabei wurden 17,8 % der Fragebogen zu Angstsymptomen und 25,6 % der Fragebogen zu Depressionssymptomen auffällig mit Gesamtscore-Werten ≥ 7 in GAD-7 bzw. PHQ-9 beantwortet. Patienten mit erhöhten Depressions- und Angstwerten wiesen im Mittel einen deutlich höheren medianen HbA1c-Wert als Zeichen einer schlechteren Stoffwechseleinstellung auf (8,33 [8,09; 8,56]) als Patienten mit unauffälligem Screening (7,58 [7,48; 7,68]; p

Zusammenfassung Hintergrund Die Zunahme von Multimorbiditat und Multimedikation in Deutschland fuhrt zu einer steigenden Komplexitat der Gesundheitsversorgung und damit zu vermehrten Sicherheitsrisiken. Vermeidbare unerwunschte Ereignisse zu identifizieren und zu verhindern muss eine hohe Prioritat haben. Bei der Verbesserung der Patientensicherheit spielt die Perspektive von Patientinnen und Patienten eine wichtige Rolle. In dieser Studie wurde eine Befragung zu Wissen, Wahrnehmung und Erfahrungen der Bevolkerung zum Thema Patientensicherheit, dabei vor allem zu subjektiv erlebten Fehlern im Versorgungsgeschehen, durchgefuhrt. Ziel war es insbesondere, den Stand der Patientensicherheit aus Sicht der Patientinnen und Patienten zu erheben und Unterschiede zwischen den Gruppen der subjektiv gut und den subjektiv schlecht zum Thema Patientensicherheit Informierten hinsichtlich der Einschatzung von Risiken und deren Vermeidbarkeit zu analysieren. Methode Im Rahmen des „TK-Monitor Patientensicherheit“ wurden bundesweit computer-assistierte Telefoninterviews durchgefuhrt. Die Rekrutierung erfolgte durch ein mehrstufiges Auswahlverfahren unter Bildung einer geschichteten Stichprobe. Eingeschlossen wurden Erwachsene (≥ 18 Jahre) die uber ausreichend Deutschkenntnisse verfugten. Die Befragung erfolgte mittels eines strukturierten Leitfadens mit 21 Fragen zur Wahrnehmung und Erfahrung mit Patientensicherheit sowie 12 Fragen zu soziodemographischen Daten. Die Ergebnisse wurden deskriptiv und mittels inferenzstatistischen Verfahren ausgewertet. Ergebnisse Von insgesamt 1000 teilnehmenden Personen (51% weiblich) war etwa die Halfte (52%) erwerbstatig und 57% schatzten ihren Gesundheitszustand als „sehr gut“ oder „gut“ ein. Insbesondere Datenschutz, Arzneimittelfehler, Infektionen im Krankenhaus und Diagnose(un)sicherheit wurden als besonders sicherheitsrelevante Problemfelder betrachtet. 55% der Befragten schatzen ihren subjektiven Informationsstand zum Thema Patientensicherheit als „sehr gut“ oder „gut“ ein. Der subjektive Informationsstand war negativ assoziiert mit wesentlichen Outcome-Parametern wie erlittenen Medikationsfehlern oder vermuteten Fehlern bei einer medizinischen Untersuchung oder Behandlung. Auch gaben subjektiv gut Informierte eher an, selbst zu einer sicheren Gesundheitsversorgung beitragen zu konnen. Diskussion Das Thema der Diagnose(un)sicherheit stellt aus Sicht der Bevolkerung eine der grosten personlichen Gefahrdungen dar. Hier zeigt sich ein Bedarf der Befragten an Bearbeitung und Information, der in der Forschungslandschaft in Deutschland bislang zu wenig abgedeckt ist. Bei den Zusammenhangen zwischen subjektivem Informationsstand und Outcome-Parametern bleibt zu analysieren, ob der subjektive Informationsstand den tatsachlichen Informationsstand abbildet, der wiederum fur wirksame eigene Praventionsmasnahmen notig ware. Dieser Frage sollte in weiteren Studien nachgegangen werden. Schlussfolgerung Die Perspektive von Patientinnen und Patienten ist eine wichtige Erganzung in der Betrachtung der Sicherheit der medizinischen Versorgung in Deutschland. Einflussfaktoren auf die subjektive Informiertheit sollten untersucht und die subjektive Informiertheit der Bevolkerung erhoht werden. Weitere, regelmasige Erhebungen sind wunschenswert, um die Thematik zu vertiefen.

Purpose Salivary Gland cancer (SGC) is a rare and heterogenous group of tumors. Standard therapeutic options achieve high local but poor distant control rates, especially in high-grade SGC. The aim of this monocentric study was to evaluate patterns of recurrence and its treatment options (local ablative vs. systemic) in a homogenously treated patient population with high-grade SGC after surgery and radio(chemo)therapy. Methods Monocentric, retrospective study of patients with newly diagnosed high-grade salivary gland cancer. We retrospectively reviewed clinical reports from 69 patients with high-grade salivary gland cancer in a single-center audit. Survival rates were calculated using the Kaplan–Meier method and prognostic variables were analyzed (univariate analysis: log-rank test; multivariate analysis: Cox regression analysis). Results The median time of follow-up was 31 months. After 5 years, the cumulative overall survival was 65.2%, cumulative incidence of local recurrence was 7.2%, whereas the cumulative incidence of distant metastases was 43.5% after 5 years. 30 of 69 patients developed distant metastases during the time of follow-up, especially patients with adenoid cystic carcinoma, salivary duct carcinoma, adenocarcinoma NOS and acinic cell carcinoma with high-grade transformation. The most common type of therapy therefore was chemotherapy (50%). 85.7% of patients with local ablative therapy of distant metastases show disease progression during follow-up afterwards. Conclusion With surgery and radio-chemotherapy, a high rate of loco-regional control is reached, but over 40% of patients develop distant metastases in the further follow-up which usually present a diffuse pattern involving in a diffuse metastases. Therefore, in the future, intensified interdisciplinary combination therapies even in the first-line treatment in certain subtypes of high-grade SGC should be investigated.

Abstract. Most cases of voluntarily stopping eating and drinking (VSED) occur in the context of severe underlying diseases with concomitant dwindling needs for food and fluids. In these cases, VSED resembles the rejection of a medical treatment. While this is the leading difference to suicide, however, there are also situations within VSED in which aspects of suicide prevention must be considered. After defining the relevant terms and presenting a literature review, we outline options of dealing with different death wishes and provide recommendations oriented toward the possibilities of suicide prevention in the context of abstaining from food and fluids.

The aim of this study was to investigate sonographic findings of low-grade malignant tumors of the parotid gland. The sonographic findings of all patients treated for T1-T2 low-grade carcinomas of the parotid gland between 2003 and 2018 were retrospectively examined and compared with those of patients with pleomorphic adenomas for the following parameters: definition of tumor margins, echotexture, echogenicity, shape and vascularization. Statistical analysis was performed using the χ2 test. A p value of

Traditional surgical philosophy condemns extracapsular dissection for pleomorphic adenoma as a euphemism for the enucleation of this lesion.The aims of the study were to trace the development of surgical treatment by pleomorphic adenomas of the parotid gland over the last 15 years and explore its effects on the histopathology specimen.The medical records of all cases with pleomorphic adenomas of the parotid gland between 2006 and 2020 were examined for information on age, gender, and type of surgery. An experienced head and neck pathologist re-evaluated the histology slides from all the cases.The study included 844 patients. Our analysis showed an increase in the performance of extracapsular dissection from 52.8% (2006) to 63.3% (2020), and an increase in histopathology specimens with tumours completely covered by healthy tissue from 27.7% (2006) to 50% (2020).Our decision-making process reached its peak in the last study years, in terms of reduced surgical invasiveness and quality of the pathology specimen.Extracapsular dissection has gained a firm hold as an indispensable tool for the experienced parotid surgeon. Proper indication is based on careful selection of cases and the correct interpretation of preoperative features on palpation and imaging.

ZusammenfassungJugendliche und junge Erwachsene mit Typ-1-Diabetes sind häufig von komorbiden psychischen Störungen betroffen. Dabei zeigt die Studienlage zur Verbreitung ein heterogenes und inkonsistentes Bild. Diagnose und Behandlung der somatopsychischen Begleiterkrankungen beeinflussen den Behandlungs- und Krankheitsverlauf sowie die Lebensqualität der Betroffenen. Trotzdem wird dies in der klinischen Praxis selten frühzeitig berücksichtigt. Das multizentrische Verbundprojekt COACH verfolgt das Ziel, die Erkennung und Behandlung psychischer Komorbidität für diese Zielgruppe in der bundesweiten Routineversorgung zu optimieren.

Zusammenfassung Ziel der Studie Befragung psychiatrischer Fachkräfte zu geschlechtsspezifischen Bedarfen depressiver Männer und Behandlungsziele für die stationäre Versorgung. Methodik Semistrukturierte Interviews. Ergebnisse Männergruppen ermöglichen die Reflexion von Geschlechtsrollenerwartungen. Aktivitätsbezogene Angebote helfen betroffenen Männern, ihre Leistungsfähigkeit einzuschätzen und bieten Erfolgserlebnisse. Sie sind im Behandlungsspektrum unzureichend abgebildet. Schlussfolgerung Die Bedeutung normativer Rollenerwartungen sollte in der Ausbildung psychiatrischer Fachkräfte berücksichtigt werden.

Consanguineous marriages have a prevalence rate of 24% in Turkey. These carry an increased risk of autosomal recessive genetic conditions, leading to severe disability or premature death, with a significant health and economic burden. A definitive molecular diagnosis could not be achieved in these children previously, as infrastructures and access to sophisticated diagnostic options were limited. We studied the cause of neurogenetic disease in 246 children from 190 consanguineous families, recruited in three Turkish hospitals between 2016 and 2020. All patients underwent deep phenotyping and trio whole exome sequencing, and data were integrated in advanced international bioinformatics platforms. We detected causative variants in 119 known disease genes in 72% of families. Due to overlapping phenotypes 52% of the confirmed genetic diagnoses would have been missed on targeted diagnostic gene panels. Likely pathogenic variants in 27 novel genes in 14% of the families increased the diagnostic yield to 86%. Eighty-two% of causative variants (141/172) were homozygous, 11 of which were detected in genes previously only associated with autosomal dominant inheritance. Eight families carried two pathogenic variants in different disease genes. De novo (9.3%), X-linked recessive (5.2%) and compound heterozygous (3.5%) variants were less frequent compared to non-consanguineous populations. This cohort provided a unique opportunity to better understand the genetic characteristics of neurogenetic diseases in a consanguineous population. Contrary to what may be expected, causative variants were often not on the longest run of homozygosity and the diagnostic yield was lower in families with the highest degree of consanguinity, due to the high number of homozygous variants in these patients. Pathway analysis highlighted that protein synthesis/degradation defects and metabolic diseases are the most common pathways underlying paediatric neurogenetic disease. In our cohort 164 families (86%) received a diagnosis, enabling prevention of transmission and targeted treatments in 24 patients (10%). We generated an important body of genomic data with lasting impacts on the health and wellbeing of consanguineous families, and economic benefit for the healthcare system in Turkey and elsewhere. We demonstrate that an untargeted next generation sequencing approach is far superior to a more targeted gene panel approach, and can be performed without specialised bioinformatics knowledge by clinicians using established pipelines in populations with high rates of consanguinity. The project is supported by TUBITAK (The Scientific and Technological Research Council of Turkey) Project No. 216S771. YO is supported by the Turkish Academy of Sciences’ Young Investigator award, TÜBA-GEBİP (2017). PFC is a Wellcome Trust Principal Research Fellow (212219/Z/18/Z), and a UK NIHR Senior Investigator, who receives support from the Medical Research Council Mitochondrial Biology Unit (MC_UU_00015/9), the Leverhulme Trust (RPG-2018-408), an MRC research grant (MR/S035699/1), an Alzheimer's Society Project Grant (AS-PG-18b-022). This research was supported by the NIHR Cambridge Biomedical Research Centre (BRC-1215-20014). RH was supported by the European Research Council [309548], the Wellcome Investigator Award [109915/Z/15/Z]. the Medical Research Council (UK) [MR/N025431/1]; the Wellcome Trust Pathfinder Scheme [201064/Z/16/Z], the Newton Fund [UK/Turkey, MR/N027302/1], the Lily Foundation and the Evelyn Trust. HL receives support from the Canadian Institutes of Health Research (Foundation Grant FDN-167281), the Canadian Institutes of Health Research and Muscular Dystrophy Canada (Network Catalyst Grant for NMD4C), the Canada Foundation for Innovation (CFI-JELF 38412), and the Canada Research Chairs program (Canada Research Chair in Neuromuscular Genomics and Health, 950-232279). Sequencing and analysis were provided by the Broad Institute of MIT and Harvard Center for Mendelian Genomics (Broad CMG) and was funded by the National Human Genome Research Institute, the National Eye Institute, and the National Heart, Lung and Blood Institute grant UM1 HG008900 and in part by National Human Genome Research Institute grant R01 HG009141. Data was analysed using the RD-Connect Genome-Phenome Analysis platform developed under FP7/2007-2013 funded project (grant agreement nº 305444) and funding from EJP-RD and INB/ELIXIR-ES. The study was further supported by the Horizon 2020 research and innovation program via grant 779257 “Solve-RD” (RH, SB, AT and HL). RH, PFC and YO were supported by an MRC strategic award to establish an International Centre for Genomic Medicine in Neuromuscular Diseases (ICGNMD) MR/S005021/1

This scientific commentary refers to ‘Induced pluripotent stem cell-derived motor neurons of CMT type 2 patients reveal progressive mitochondrial dysfunction’, by Van Lent et al. (doi:10.1093/brain/awab226).

Canine atopic dermatitis (AD) is an inflammatory and pruritic skin disease and in most cases associated with IgE antibodies against environmental allergens. To date, the only causative therapeutic option is allergen immunotherapy (AIT). Offending allergens for AIT can be identified by intradermal testing (IDT) or serum allergen-specific IgE testing. The aim of the study was to evaluate positive IDT results considering the atopic dogs' clinical history.An IDT was performed on 48 atopic dogs and their owners completed a detailed questionnaire about the seasonal course of their pruritus. Results of IDT were correlated with the seasonal occurrence of pruritus.The most common positive IDT reactions were observed to mite allergens (33.3-62.5 %). Prevalence of positive reactions to individual tree, grass and weed pollen ranged between 8.3 % and 25 %. Moulds and epithelial allergens produced positive reactions in only 0-6.3 %. A correlation between positive IDT reactions and course of pruritus could neither be found for perennial nor for seasonal allergens.The evaluation of IDT reactions may not be an optimal method for identification of clinically relevant allergens in canine AD.The results of this study emphasise the importance of considering clinical history in addition to allergy test results in the formulation of an allergen extract for desensitisation.Die kanine atopische Dermatitis (AD) ist eine entzündliche und juckende Hautkrankheit, bei der in den meisten Fällen IgE-Antikörper gegen Umweltallergene auftreten. Bis heute stellt die Allergen-Immuntherapie (AIT) die einzige kausale Therapie dar. Am Krankheitsgeschehen beteiligte Allergene können durch Intrakutantests (IKT) oder Serumtests auf allergenspezifisches IgE identifiziert werden. Ziel dieser Studie war herauszufinden, ob positive IKT-Ergebnisse mit der Anamnese der atopischen Hunde korrelieren.Bei 48 atopischen Hunden, bei denen ein IKT durchgeführt wurde, füllten die Hundebesitzer einen detaillierten Fragebogen über den jahreszeitlichen Verlauf des Juckreizes ihrer Hunde aus. Die Antworten in den Fragebögen wurden in Beziehung zu den Resultaten des IKT gesetzt.Die häufigsten positiven Reaktionen wurden gegen Hausstaubmilben festgestellt (33,3–62,5 %). Die Prävalenz positiver Resultate gegen die getesteten Pollen von Bäumen, Gräsern und Kräutern lag zwischen 8,3 % und 25 %. Schimmelpilze und Epithelien führten bei 0–6,3 % zu positiven Reaktionen. Positive IKT-Ergebnisse gegen ganzjährige und saisonale Allergene korrelierten nicht mit dem Auftreten von Juckreiz.Die Bewertung von Reaktionen im IKT ist möglicherweise nicht die optimale Methode für die Bestimmung der klinisch relevanten Allergene bei kaniner AD.Die Ergebnisse dieser Studie bestätigen, dass bei der Auswahl von Allergenen für die AIT die klinische Anamnese zusätzlich zu den Ergebnissen des Allergietests berücksichtigt werden muss.

Etwa 2 % der Bevolkerung leiden unter Psoriasis, die langst nicht mehr eine rein dermatologische Erkrankung, sondern vielmehr eine Systemerkrankung darstellt. Demnach mussen auch haufig Begleiterkrankungen sowie eine potenzielle Gelenkbeteiligung erfasst und ggf. eine interdisziplinare Patientenbetreuung organisiert werden. Der Einsatz von validierten Scores stellt eine wichtige Erganzung der Anamnese dar. Therapeutisch kommen intensivierte Lokaltherapien zum Einsatz und kurzfristig z. B. im Schub auch Phototherapien. Neben altbewahrten, konventionellen Systemtherapeutika (u. a. Fumarate, Methotrexat [MTX]) wachst der Markt an Biologika und „small molecules“ etc. stetig. Aktuell stehen neben dem oralen PDE(Phosphodiesterase)-4-Hemmer Apremilast zielgerichtete Therapien zur Blockierung von TNF(Tumor-Nekrose-Faktor)-α, IL(Interleukin)-17A, IL-17-Rezeptor und IL-23 zur Verfugung. Das geeignete Praparat sollte auf den Patienten zugeschnitten sein und auch Aspekte der Wirtschaftlichkeit berucksichtigen und dabei den Gedanken der moglichen Komorbiditatspravention nicht verlieren.

Introduction Microvascular free tissue transfer is a technique for reconstruction of large defects in head and neck surgery. Failure due to microvascular thrombosis can lead to microvascular damage or flap loss. Recombinant tissue-type plasminogen activator (Alteplase) is still an off-label use but it can help to rescue free flaps when embedded in a salvage algorithm. Presentation of case A 39-year-old patient with received a tumor resection and reconstruction by a radial forearm flap of the left palate. Postoperatively a venous flap thrombosis occurred and immediate surgical revision was done. Initially eperfusion of the flap could not be achieved even after mechanical removal of the thrombus. Then a thrombolysis with Alteplase, which was applied directly into the radial artery, was done. The flap was salvaged and is now completely integrated into the mucosa. Flap salvage procedure was performed according to our free flap salvage algorithm. Discussion Thrombolysis with Alteplase for free flap salvage is not a common method. Pedicle thrombosis cannot be predicted. Important procedures during surgical intervention when thrombosis occurs are careful reopening, removal of thrombus, flushing with heparin. Since these procedures failed, surgeons decided to employ Alteplase to optimally rescue the flap. Conclusion The present case shows that pharmacological thrombolysis with Alteplase is an effective ultima ratio in free flap salvage with venous thrombosis, although it is still considered offlabel use. Early detection of flap failure and a clear salvage algorithm are important for successful surgical revisions.

Purpose of Review: This review aims to evaluate new insights into allergen immunotherapy (AIT) and review the mechanism, different protocols used, and response rate of this treatment option for canine atopic dermatitis. Recent Findings AIT is the only etiological treatment available for canine atopic dermatitis. Selection of allergens is based on clinical history in conjunction with positive reactions on intradermal and serum testing. During decades AIT was applied subcutaneously (SCIT) using aqueous or aluminum-precipitated solution of allergens, but in the last few years, new routes like sublingual (SLIT), intralymphatic (ILIT), and epicutaneous administration are on the rise. AIT combines a satisfactory success rate (around 70%) with a good safety profile. Adverse effects include mainly increased pruritus and anaphylactic reactions are rarely seen in animals. Pilot studies with different adjuvants to further increase safety and efficacy have been published. Summary: AIT is a safe and effective therapy for canine atopic dermatitis. However, as most of the published studies are case series with often rather small numbers of animals included, more and larger randomized studies of immunotherapy are needed in a clinical setting to provide guidance on the optimal selection of allergens, adjuvants, dosing, success rate, and adverse effects of the various protocols of immunotherapy in dogs.

Zusammenfassung Hintergrund Mangelnde Therapietreue ist ein weitverbreitetes Versorgungsproblem in der Anti-VEGF(Vascular Endothelial Growth Factor)-Behandlung von Patienten mit neovaskulärer altersabhängiger Makuladegeneration (nAMD). Im Gegensatz zum Ausmaß mangelnder Therapietreue gibt es bislang keine ausreichende Evidenz zu den Ursachen. Die ANDROMEDA-Studie wurde initiiert, um die Einflussfaktoren der Therapietreue von nAMD-Patienten zu untersuchen. Der Fokus der Studie liegt auf patientenberichteten Endpunkten, da die Wahrnehmungen und Erfahrungen der Patienten für die Untersuchung der vielfältigen Aspekte von Therapietreue von enormer Bedeutung sind. Ziel der Arbeit In dieser Publikation werden das Design der Studie sowie die Entwicklung eines neuen Patientenfragebogens zur Erfassung von Therapiehürden vorgestellt. Material und Methoden Anfang 2019 wurde die prospektive, nichtinterventionelle Beobachtungsstudie zur Erfassung der Therapietreue von nAMD-Patienten und Anti-VEGF-Behandlung gestartet. Es ist geplant, deutschlandweit 1000 Patienten in 120 Studienzentren für 24 Monate zu beobachten. Dabei werden zu Beginn sowie nach 4, 12 und 24 Monaten Patienteninterviews zur allgemeinen und visusbezogenen Lebensqualität, zur Behandlungszufriedenheit und zu möglichen Therapiehürden geführt. Sämtliche Visiten werden im Rahmen der klinischen Routine durch die Studienzentren dokumentiert. Bislang existierte zur Erfassung patientenseitiger Begleitumstände und potenzieller Hürden der Anti-VEGF-Therapie kein geeignetes Instrument, weshalb ein spezifischer Patientenfragebogen, der „Patient Questionnaire for Longitudinal Assessment of Adherence Factors to Intravitreal (anti-VEGF) Therapy“ (LAF-IVT), entwickelt wurde. Vor dem Einsatz in dieser Studie wurde der von einem Expertenpanel entwickelte Fragebogen mittels qualitativer Interviews hinsichtlich seiner kognitiven Eigenschaften geprüft. Ergebnisse Die Ergebnisse der Studie werden Anfang 2023 erwartet. Die kognitive Prüfung des LAF-IVT bestätigte die Einsatzfähigkeit des neuen Fragebogens. Die Praktikabilität und Aussagekraft des neuen Instruments können nach Abschluss der quantitativen Datenerhebung beurteilt werden. Diskussion Die von Patienten erlebten Symptome, Hürden, Lasten und Lebensqualitätseffekte einer Therapie beeinflussen die Therapietreue und damit die Therapieergebnisse. Ein besseres Verständnis der Patientensichtweisen und -erfahrungen ist die Grundlage für eine dauerhafte Optimierung der Versorgung.

Cardiovascular disease is an important contributor to morbidity and mortality in people living with HIV . The immature platelet fraction (IPF) is increased in HIV-negative patients with cardiovascular disease and evidence suggests that an enlarged IPF is associated with adverse cardiovascular events. In this multi-center observational study, we aimed to investigate how the IPF in people living with HIV is influenced by antiretroviral therapy and cardiovascular disease. Subjects without cardiovascular disease that received antiretroviral therapy showed a smaller IPF accompanied by lower D-dimer and C-reactive protein (CRP) levels compared to therapy-naïve subjects (mean IPF: 2.9% vs. 3.9%

Recently, we discovered the recurrent genomic rearrangement [t(4;9)(q13;q31)] enabling upregulation of the transcription factor Nuclear Receptor Subfamily 4 Group A Member 3 (NR4A3) through enhancer hijacking as the oncogenic driver event in acinic cell carcinoma (AciCC) of the salivary glands. In the current study, we evaluated the usefulness of NR4A3 immunostaining and NR4A3 fluorescence in situ hybridization (FISH) in the differential diagnosis of AciCC, comparing a total of 64 AciCCs including 17% cases with high-grade transformation, 29 secretory (mammary analog) carcinomas (MASC), and 70 other salivary gland carcinomas. Nuclear NR4A3 immunostaining was a highly specific (100%) and sensitive (98%) marker for AciCC with only 1 negative case, whereas NR4A3 FISH was less sensitive (84%). None of the MASCs or other salivary gland carcinomas displayed any nuclear NR4A3 immunostaining. The recently described HTN3-MSANTD3 gene fusion was observed in 4 of 49 (8%) evaluable AciCCs, all with nuclear NR4A3 immunostaining. In summary, NR4A3 immunostaining is a highly specific and sensitive marker for AciCC, which may be especially valuable in cases with high-grade transformation and in "zymogen granule"-poor examples within the differential diagnostic spectrum of AciCC and MASC.

OBJECTIVES/HYPOTHESIS The aim of this study was to identify potential associations between epidemiologic, morphologic, and histopathologic features in pleomorphic adenomas (PAs) of the parotid gland in order to extract information about the natural course and biologic behavior of these lesions on the basis of a single-center series of 845 cases within a period of 15 years. STUDY DESIGN Retrospective study in a tertiary academic center. METHODS For this study, an experienced head and neck pathologist critically re-evaluated the histological slides of the pathological specimens of all patients who underwent a parotidectomy for PA of the parotid gland between 2006 and 2020. RESULTS A total of 845 cases made up our study sample. Our analysis showed a statistically significant association of the histologic subtype with younger age (P = .001) and maximal diameter (P = .044), with the hypocellular type being encountered more often in younger patients and in smaller lesions. The same subtype was significantly associated with an incomplete capsule (P = .001), pseudopodia (P = .006), and satellite nodules (P = .001). An incomplete capsule was associated with the presence of pseudopodia (P = .001) and satellite nodules (P = .001). CONCLUSION It seems that various histologic subtypes have different capsule-producing properties. Apparently, over the course of time, tumor material builds a finger-like projection still inside the capsule, separates itself from the parenchyma with fibrous tissue still remaining enclosed within the capsule (pseudopodium), slowly penetrates the capsule (incomplete capsule), and leaves the main lesion taking a part of the capsule with it (satellite nodules). Laryngoscope, 2021.

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.