一种罕见的儿童肿瘤疾病,表现在耳鼻喉器官上

A. V. Andrianov, M. V. Drozdova, S. Alekseenko, A. A. Vasilyeva, D. Y. Spichak
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摘要

鼻腔、副鼻窦和上呼吸道疾病在所有年龄段儿童的急性病理结构中都占主要地位。儿童腺扁桃体疾病也是如此。儿童肿瘤疾病非常罕见,尤其是耳鼻喉科器官的肿瘤。要知道,在急性耳鼻喉科病变和腺扁桃体疾病的幌子下,可能隐藏着包括癌症在内的罕见疾病。鼻呼吸困难的主诉可能是这种病变的唯一表现。医生的任务是尽早发现癌症病变。鉴别诊断的主要方法是 X 光和内窥镜检查,与年龄无关。神经纤维瘤病(NF)是一组遗传性系统疾病。指的是噬瘤病。特征包括外胚层和中胚层系统形成障碍。皮肤、神经和骨骼系统最常受到影响。恶性肿瘤的形成发展是其特征。这种疾病虽然罕见,但对神经系统造成的癌症负担比其他任何肿瘤性疾病都要大。在这方面,肿瘤的形成可能有不同的定位和临床表现。该病影响多个系统,有皮肤、神经和骨科表现,导致患者残疾或死亡。我们的研究旨在展示一种罕见的肿瘤疾病--神经纤维瘤病,该病的首发症状是一名有家族史的 6 岁儿童的耳鼻喉器官。本临床病例的意义在于,神经纤维瘤罕见地位于颈部结构,累及咽、喉、纵隔和颈部血管。
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A rare oncological disease in a child with manifestation in the ENT organs
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.
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