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PL
Wstęp: Przyzwojaki są rzadkimi nowotworami neuroendokrynnymi. Lokalizują się głównie w kłębku szyjnym, otworze szyjnym i nerwie błędnym. Charakteryzują się bogatym unaczynieniem, powolnym wzrostem oraz przeważnie łagodnym przebiegiem, jednak często powodują szereg uciążliwych objawów. Cel: Celem niniejszej pracy jest analiza kierunków szerzenia się oraz korelacji z obrazem klinicznym tych guzów, umiejscowionych w okolicy otworu szyjnego. Materiały i metody: Analiza obrazu klinicznego i przebieg leczenia 6 pacjentów (4 kobiety i 2 mężczyzn), w wieku od 36 do 72 lat, operowanych w listopadzie 2022 roku w Klinice Otorynolaryngologii Warszawskiego Uniwersytetu Medycznego. Zwrócono szczególną uwagę na zależność między morfologią guzów, obrazem klinicznym oraz przebiegiem leczenia. Wyniki: Wśród objawów u czterech z sześciu pacjentów występował niedosłuch, u trzech z sześciu zawroty głowy, szumy uszne i dolegliwości bólowe. U jednego pacjenta stwierdzono guz opuszki żyły szyjnej, rozrastający się do przestrzeni przygardłowej. U dwóch chorych guz zajmował jamę bębenkową. U jednego pacjenta wrastał do dołu tylnego czaszki. Zajęcie zatoki esowatej stwierdzono w dwóch przypadkach. W najbardziej zaawansowanym przypadku guz zajmował tętnicę, żyłę szyjną wewnętrzną, zatokę esowatą i tylny dół czaszki. U pięciu chorych guz zajmował opuszkę żyły szyjnej wewnętrznej, jednak była ona zajęta w różnych częściach. Kierunek wzrostu guza może być wynikiem pierwotnej lokalizacji ciałka przyzwojowego w relacji do struktur otworu szyjnego. Wnioski: Obraz kliniczny opisanych pacjentów przedstawia różnorodność rozwoju guzów otworu szyjnego. Określenie i przewidzenie kierunku wzrostu przyzwojaków może stanowić ważny element w planowaniu leczenia, szczególnie chirurgicznego. Ich rozwój wymaga dalszych badań, które pozwoliłyby określić dokładniej zarówno miejsce ich powstania, jak i potencjalne ryzyko kliniczne dla pacjentów.
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Vagus nerve paragangliomas

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EN
Introduction: Vagus nerve paragangliomas are slow-progressing tumors. Histopathologically, however, they are classified as malignancies. Vagus nerve paragangliomas are rare tumors accounting for 3–25 of all paragangliomas within the head and neck region and about 0.5% of all tumors of this type. Aim: Presentation of the clinical picture, diagnostic management and treatment strategies of vagus nerve paragangliomas. Materials and methods: The study presents a retrospective analysis of the clinical presentation, diagnosis, treatment and postoperative course of 11 patients treated for vagus nerve paraganglioma at the Department of Otorhinolaryngology, Head and Neck Surgery at the Medical University of Warsaw between 2011 and 2020. The study sample included 7 female and 4 male patients aged 36 to 72 years. Results: The predominant symptom as observed in the study group was a neck mass. Foreign body sensation in the throat and dysphagia were also common. Preoperative diagnosis is based mainly on imaging studies due to the characteristic radiological features of vagus nerve paragangliomas and their location relative to neighboring anatomical structures. Surgical treatment was pursued in 10 patients. Three different surgical access routes, namely the cervico-parotid, cervical, and cervico-infratemporal access, were followed depending on the location of the tumor. Vagus nerve injuries occurred during tumor excision surgeries in 7 cases. Injuries to cranial nerves IX and XII occurred in 7 patients. Injuries to cranial nerves XI and VII occurred in 1 case each. As a part of the treatment for postoperative complications, 3 patients with vagus nerve paralysis underwent fat augmentation of the affected vocal fold. Reinnervation involving ansa cervicalis was performed in two patients. One patient with vocal fold paralysis, dysphagia, and tongue deviation was subjected to type I thyroplasty. Conclusions: Among the available options for the management of vagus nerve paragangliomas, surgical methods seem to provide the most effective solutions due to the wide range of access techniques and adaptability to individual cases. Due to the slow growth, a “wait and scan” approach can be applied. Surgical interventions, as a treatment of choice, are associated with damage to the vagus nerve necessitating either swallowing rehabilitation or reconstructive procedures within the larynx and pharynx.
EN
Introduction: Paragangliomas are rare neuroendocrine tumors. Most frequently located at the carotid body, jugular foramen and vagus nerve. They are hypervascular, slow-growing and generally benign, but they can cause significant complications due to the associated mass effect and compression or even erosion of the local structures. Aim: The aim of this study is to analyze the directions of spreading and correlation with the clinical characteristics of these tumors located in the jugular foramen area. Materials and methods: Analysis of the clinical presentation and treatment of 6 patients (4 women and 2 men), aged 36 to 72, who underwent surgery in November 2022 at the Department of Otorhinolaryngology of the Medical University of Warsaw. Particular attention was paid to the relationship between tumor morphology, clinical manifestations and course of treatment. Results: Four out of six patients developed hypoacusis, three out of six suffered from vertigo, tinnitus and pain. One patient had a tumor of the bulb of the jugular vein spreading to the parapharyngeal space. In two patients, the tumor occupied the tympanic cavity. In one patient, it grew into the posterior cranial fossa. Sigmoid sinus involvement was found in two cases. In the most advanced case, the tumor involved the internal carotid artery, internal jugular vein, sigmoid sinus, and posterior cranial fossa. In five patients, the tumor involved the bulb of the internal jugular vein, but in different parts. The direction of tumor growth may be a result of the original location of the paraganglionic body in relation to the structures of the jugular foramen. Conclusions: The clinical manifestations of the described patients shows the diversity of paragangliomas development. Determining and predicting the direction of growth may play an important role in planning treatment, especially surgical. The development of these tumors requires more research to determine exactly where they form, as well as the potential risk of a disease-causing event.
EN
Introduction: More than 5% of the world’s population experience hearing impairment. The most common form is presbycusis (age-related hearing loss; ARHL). It affects almost one in three people over the age of 65. The hair cells of the cochlea play an important role in the process of sound registration. Genetic mutations, aging and environmental factors can cause damage that contributes to the hearing loss. Methods and results: The currently explored research directions include drug treatments, gene therapies, and stem cell therapies. To date, no significant differences in the therapeutic effect depending on the route of corticosteroid administration have been demonstrated in patients with moderate to severe hearing loss. New dexamethasone-containing hydrogel formulations, as well as lipid formulations, thermosensitive polymers, and nanoparticles, have been developed to achieve high drug concentrations in the inner ear structures. Otoprotective effects of antioxidants or substances that modify the toxic effects of e.g. cisplatin, are also being studied. Attempts at auditory cells’ regeneration seem promising in hearing loss research. Substances that regulate the central mechanisms of the Notch and Wnt pathways are being explored to this end. The genetic determinants of presbycusis suggest that interference at the level of specific genes may be a promising option for the treatment of this condition. With the CRISPR/Cas9 technology, the functions of inner ear genes can be effectively studied by disrupting normal gene alleles. The CRISPR/Cas9 complexes developed to target specific genes are delivered using cationic lipids, proteins, and viral vectors. They are then transported through the round window membrane by diffusion, without the need to surgically disrupt the inner ear. The potential of using antisense oligonucleotides to treat hereditary deafness caused by hair cell degeneration has also been established. Another research direction is related to stem cells being used for the development of in vitro 3D models of the human inner ear. Studies are also pursued to identify the mechanisms underlying the formation of cochlear organoids from pluripotent cells as well as determine the critical time points and events for cochlear sensory epithelial development and targeted hair cell differentiation. Conclusions: In summary, significant progress has been made over the past decade in the search for novel therapies for sensory hearing loss. This line of research remains an ambitious and important area for further exploration.
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