Эндоскопический эндоназальный подход к удалению опухолей полости носа и околоносовых пазух с распространением на основание черепа у детей

Background. Diversity of opinions on the possibility and feasibility of using endoscopic endonasal approaches for the removal of tumors  located in the nasal cavity, paranasal sinuses, and the base of the  skull in pediatric practice, age-related anatomical features of  pediatric patients, as well as little conducted research make it  difficult to elaborate an optimal therapeutic-diagnostic algorithm for  the application of the endoscopic endonasal approach (EEA) in the surgical practice of a pediatric oncologist. Objective . The study is aimed at the development of an optimal algorithm for endoscopic endonasal methods for diagnosis and  surgery of tumors located in the nasal cavity and paranasal sinuses with skull base metastases in pediatric patients. Methods . During the period from 2012 to 2017 at the NMRC of  pediatric oncology, 54 endoscopic endonasal surgeries were  performed in 43 patients aged from 1 month to 16 years. The mean  age of patients at surgery was 8.1 years. The ratio of boys (n=23)  and girls (n=20) in the study group was 1.15:1, or 53.5% and  46.5% respectively. About 10 (18.5%) operative interventions in the biopsy volume were performed regarding the suspicion of a malignant tumor. Removal of a malignant tumor before the beginning of a special treatment was performed in 2 (4.7%)  patients. The first group included 22 (51.1%) patients diagnosed with morphologically confirmed malignant tumor. In 50%  of cases, malignant tumors were detected in children aged 10–14  years. One of the morphological variants of sarcoma was verified in  15 (68.2%) patients, esthesioneuroblastoma (ENB) — in 6 (27.3%),  and Burkitt’s lymphoma — in 1 (4.5%). Surgery as a part of complex treatment was performed in 6 (27.3%) patients with rhabdomyosarcoma (RMS) and at the final stage of treatment the removal of residual tumor was performed in 5 (22.7%) cases. 7  endoscopic endonasal surgeries were performed in 6 patients with  ENB. One of them underwent repeated surgery in 4.1 years due to  the relapse of the disease. In 3 patients in the stage A according to  the Kadish–Morita classification, only operative treatment was  performed. In one case, endoscopic endonasal resection of recurrent osteosarcoma of the maxillary sinus and the inferior orbit wall was performed in the first stage of treatment. In one patient enrolled in  the study, the diagnosis of hemangiopericytoma was morphologically confirmed after the biopsy. Surgery using EEA was performed after neoadjuvant chemotherapy. The second group included patients  (n=21) who had a suspicion of a malignant tumor according to the  results of comprehensive examination; girls predominated — 13  (61.9%). The majority of patients were under 10 years old — 13 (61.9%), the mean age — 7.6 years. In all cases, after the  removal of the tumor, a histological study was performed, confirming the benign nature of the neoplasm. Results. In patients who underwent endoscopic endonasal surgery (n=39), we did not register any case of systemic or local  infections, orbital complications or bleeding associated with unrecognized vascular trauma. Cerebrospinal fluid leakage in  the postoperative period was observed in 2 patients on the 2nd–4th  day. In 22 patients with malignant tumors, 17 (77.3%) patients are  alive without signs of progression and recurrence of the disease, the follow-up period is from 1 month to 5.1 years. Relapses were registered in 5 (22.7%) patients in the period from 1 month to 4.1  years. Lethal outcome occurred in 4 (18.2%) patients, 3 of them (13.6%) — due to the tumor progression, 1 (4.5%) — due to  reasons not associated with tumor progression or toxicity of  treatment. In the group of patients with benign tumors (n=21), all  were alive during the observation period (1 month to 1.6 years).  Relapses were registered in 3 (14.3%) patients. In 2 (9.5%) cases,  relapses occurred in patients with fibrous dysplasia in 7–9 months. In 1 (4.8%) case, the development of recurrent angiofibroma of the nasopharynx was observed. Patients underwent re-operation. The  observation period after the repeated surgery lasted for 1–11 months. Conclusion . The endoscopic endonasal approach for tumor removal in children with the nasal cavity and paranasal sinuses cancer with  scull base metastases was developed basing on the data of modern  medical research and personal clinical experience.