The incidence of esthesioneuroblastoma in children under 15 years of age is 0.1 per 100.000 children. Distinctive histological features of this tumor are diffuse accumulation of neuron-specific enolase, synaptophysin, chromogranin, and variable expression of cytokeratins. Diagnosis of the tumor includes endoscopic examination of the nasal cavity and nasopharynx, magnetic resonance imaging (MRI) and computed tomography (CT) of the skull base, paranasal sinuses with intravenous contrast. PET-CT is advisable to use for the detection of regional and distant metastases, as well as for suspected relapse. In patients of adult age, a negative effect on the outcome of the disease was detected, the detection of metastases in the lymph nodes of the neck, the presence of tumor cells at the edges of tumor resection and a high degree of malignancy of the tumor according to the Hyams system. Therapeutic approaches depend on the stage of esthesioneuroblastoma by Kadish. In the A-stage, surgical treatment is advisable. In the presence of tumor cells at the edges of the resection or residual tumor, radiation therapy is performed. In case of B-stage, surgical treatment is combined with the mandatory irradiation of the primary tumor area. In patients with the C-stage, neoadjuvant chemotherapy or radiation is performed, followed by a surgical treatment, adjuvant chemotherapy and/or radiation therapy. Patients with D-stage chemoradiation therapy is indicated. There is no consensus on an effective drug regimen. Overall 5-year survival varies significantly depending on the design of the study — 55% to 98%. Further study of the features of the clinical picture, morphological and molecular features and the course of the disease will help to improve our understanding of the nature of the tumor.
Objectives. The relationship between the development of the number of malignant tumors in children and viruses is well known. The etiological role of the EpsteinBarr virus in the development of undifferentiated nasopharyngeal cancer (NPC) has been well studied. The effect of human papillomavirus (HPV) on the occurrence and development of NPC in children is not sufficiently understood.
Aim. The description of the clinical case of the Epstein Barr virus and HPV infection in 16-year-old patient with the consistent development of NPC and oropharyngeal papillomatosis.
Description of the clinical case. The clinical observation of the boy who was diagnosed with NPC. The full clinical effect in the form of reduction of the primary tumor and metastases in the lymph nodes of the neck was noted after chemo- and radiotherapy. 18 months after the cure according to the positron emission tomography, combined with computed tomography, with 18F-deoxyglucose we marked the accumulation of radiopharmaceuticals in the area of the left palatine and lingual tonsils. The examination revealed clinical signs of the soft palate papillomatosis. The resection of the soft palate was carried out. The morphological study confirmed squamous cell papilloma. HPV type 16 DNA was found in the papilloma.
Conclusion. The accumulation of the information concerning childhood pharynx lesions caused by HPV would help to expand understanding of the most common types of the virus in the paediatric population. It would be possible to identify the groups of risk for the development of malignant tumors on the grounds of the detection HPV types of highly carcinogenic risk.
Российский журнал ДЕТСКОЙ ГЕМАТОЛОГИИ и ОНКОЛОГИИ R u s s i a n J o u r n a l o f P e d i a t r i c H e m a t o l o g y а n d O n c o l o g y 83 К л и н и ч е с к и е н а б л ю д е н и я || C l i n i c a l c a s e s
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