ВРОЖДЕННАЯ НЕЙРОБЛАСТОМА: ОБЗОР ЛИТЕРАТУРЫ И СОБСТВЕННЫЙ ОПЫТ НМИЦ ДГОИ им. ДМИТРИЯ РОГАЧЕВА ПО ПРЕНАТАЛЬНОЙ ДИАГНОСТИКЕ, КЛИНИЧЕСКОМУ ТЕЧЕНИЮ И ТАКТИКЕ ВЕДЕНИЯ ПАЦИЕНТОВ

Abstract

Congenital neuroblastoma (NB) accounts for 5 % of the total number of patients with this diagnosis and is characterized by a favorable remote prognosis. Most patients are stratified into a low-risk group and do not need chemotherapy. However, in some cases, at stage 4S the disease may develop life-threatening symptoms. Objective of the study: development of an algorithm for prenatal diagnosis and management of patients after birth with NB diagnosis. Materials and methods of research: the study includes 29 patients with a diagnosis of «neuroblastoma», first suspected during ultrasound of the fetus. Therapy and dynamic observation were carried out at the National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev (Moscow, Russia) from January 2012 to July 2021 (115 months). The stage of the disease was established in accordance with international criteria (G.M. Brodeur, 1993). Stratification for risk groups and therapy was carried out according to the recommendations of the modified protocol of the German group for the treatment of NB-2004. Results: the median of the gestation period at the time of detection of pathological changes in the fetus in 25 cases was 36 weeks (spread 28–40 weeks). In 4 patients, the exact period of detection of prenatal formation was not specified in medical documents. The most frequently detected pathology was interpreted as a voluminous formation of retroperitoneal space – 20/29 (70%) cases, in 7/29 (24%) – as the formation of the abdominal cavity and in 2/29 (6%) – as a chest formation. The delivery naturally was carried out in 17/29 (57.7%), in 12/29 (42.3%) a cesarean section was performed. Median age at diagnosis of «neuroblastoma» amounted to 1.9 months (spread 0.4–12.7 months). Lesions of the adrenal glands were the most commonly noted – 26/29 (89.7%), including bilateral – 8/26. Primary tumor was localized in the posterior mediastinum in 2 patients (6.9%), in one case retroperitoneally – (3.4%). No adverse cytogenetic markers were detected in any case. The first stage of the disease was established in 17/29 (58.6%) of patients, in one case, at the 2–4th stage, this stage was detected in 9/29 (31%) children. 28 (96.6%) out of 29 children were stratified into the observation group, one (3.4%) patient – into an intermediate risk group. In 3/29 (10.3%) of patients, the development of relapse/progression was noted. All patients are alive with median observation of 46.9 months (spread 3.5–99.6 months). Conclusion: Patients with intrauterine NB have favorable biological and cytogenetic characteristics of the tumor. The multidisciplinary approach in management of such patients from the moment of detection of fetal pathologies to the diagnosis of NB and following dynamic observation result in high survival rates.