Affiliation:
1. Saint Petersburg Research Institute of Ear, Throat, Nose and Speech
2. Saint Petersburg Research Institute of Ear, Throat, Nose and Speech;
Children’s City Multidisciplinary Clinical Center of High Medical Technologies named after K.A. Rauhfus;
North-Western State Medical University named after I.I. Mechnikov
3. Children’s City Multidisciplinary Clinical Center of High Medical Technologies named after K.A. Rauhfus
Abstract
Diseases of the nose, paranasal sinuses and upper respiratory tract occupy a leading place in the structure of acute pathology in children of all ages. This also applies to adenotonsillar problems in children. Oncological diseases in children are quite rare, especially those localized in the ENT organs. It must be remembered that under the guise of acute ENT pathology and adenotonsillar problems, quite rare diseases, including cancer, can be hidden. A complaint of difficulty in nasal breathing may be the only manifestation of this pathology. The doctor’s task is to identify cancer pathology at the earliest possible stage. The main methods for differential diagnosis are x-ray and endoscopic examination, regardless of age. Neurofibromatosis (NF) is a group of systemic diseases that are inherited. Refers to phakomatoses. Features include disturbances in the formation of systems of ectodermal and mesodermal origin. The skin, nervous and skeletal systems are most often affected. The formation of the development of malignant neoplasms is characteristic. The disease is rare but results in a greater cancer burden on the nervous system than any other neoplastic disease. In this regard, tumor formations may have different localization and clinical manifestations. The disease affects multiple systems with cutaneous, neurological and orthopedic manifestations that lead to disability or mortality of the patient. The purpose of our study was to demonstrate a rare oncological disease – neurofibromatosis, the first manifestations of which were manifestations in the ENT organs of a 6-year-old child with a family history. The interest of the presented clinical case lies in the rare localization of neurofibroma with localization in the structures of the neck with the involvement of the pharynx, larynx, mediastinum, and neck vessels.
Reference29 articles.
1. Kiselyov AS, Vavilova AA. Rare diseases in practice of otorhinolaryngologist of outpatient clinic. Some aspects of diagnostics. Rossiiskaya Otorinolaringologiya. 2022;119(4):113–122. (In Russ.) https://doi.org/10.18692/1810-4800-2022-4-113-122.
2. Turieva BB, Kokorina OV, Achba RR, Samoilov AA, Selina EV, Dvoryanchikov BB. Multiple osteomas of paranasal sinuses. Rossiiskaya Otorinolaringologiya. 2022;21(6):126–132. (In Russ.) https://doi.org/10.18692/1810-4800-2022-6-126-132.
3. Harina DV. Comparative characteristics of morbidity in terms of the incidence of children aged 0 to 3 years who attend and do not attend preschool institutions. International Journal of Applied and Basic Research. 2018;11(1):80–84. (In Russ.) Available at: https://applied-research.ru/ru/article/view?id=12453.
4. Kryukov AI, Nosulya EV, Kim IA, Perich B. The benign tumours and tumourlike conditions of the sino-nasal region in the children. Russian Rhinology. 2019;27(1):41–48. (In Russ.) https://doi.org/10.17116/rosrino20192701141.
5. Chekan VL, Shulepova EA, Tishkevich ES. A case of congenital neurofibroma of the nose. Otorhinolaryngology. Eastern Europe. 2023;13(2):241–248. (In Russ.) https://doi.org/10.34883/PI.2023.13.2.037.