Abstract
Despite the onset of neuroblastoma (NB) in most cases takes place at the age of 18 months, this solid tumor is one of the most common malignant neoplasms in the neonatal period. Epidural compression (EC) as a sign of distribution of the tumor process into the spinal channel is a very rare phenomenon among clinical manifestations of the congenital NB. Exactly rarity of this observed condition hardens differential diagnostics of the states manifested with availability of neurological disorders such as cuts (paralyses) during the neonatal period. A similar neurological situation can show the pathology of the nervous system most frequent in this age group, such as malformations, perinatal damage, ischemic and hemorrhagic changes in the spinal arteries, certain genetically determined diseases of the neuromuscular apparatus. In most cases, the main factor that unfavorably affects recovery of movement functions of a child us the EC duration until the moment of commencement of treatment. At the same time, children have favorable oncologic forecast with the congenital NB and EC: The 5‑year survival rate comprises up to 86.2 %. The main objective of this publication is to highlight a rare clinical case; problems of differential diagnostics of neurological status with malformations and perinatal lesions of the nervous system; as well as the problems associated with the conduct of this group of patients. Exactly correctly and timely EC diagnostics shortens the interval from the moment of setting the diagnosis to the commencement of the operation. Suspect availability of malignant neoplasms of the fetus, as well as the development of neurological disorders, possibly with the help of screening methods for diagnosis – ultrasonic examination. Starting from the III trimester of pregnancy, it is possible to visualize paravertebral hyperechoic masses penetrating into the spinal canal. Decreasing of movement activities in extremities in the course of this process is the sign of EC. EC therapy caused with NB include poliochemotherapy, surgical intervention (laminotomy and laminectomy), and radiation therapy. The selection of therapeutic approach remains individual for each oncologic in-patient department due to certain factors. Surgical intervention as the preferred method of treatment aimed at rapid withdrawal of symptoms of spinal cord compression is associated with the risk of postoperative deformities of the spine, retarded growth, prolonged bed rest period, and, possibly, subsequent reconstructive surgery on the spinal column. Nevertheless, surgical intervention is required in the case of rapidly growing neurological deficiency, return of neurological symptoms or absence of tumor shrinkage on the background of chemotherapy. In most cases, chemotherapy is performed in accordance with NB-2004 protocol, however, daily monitoring of the neurological status is required for therapy correction and, if necessary, settlement of the issue of operative intervention. Radiation therapy is very rarely used for treatment of the NB that caused EC due to high risk of development of secondary malignant neoplasms. Irrespectively of selection of the therapeutic approach to treatment of the EC caused with NB, permanent motor deficiency was observed in 50 % of cases. Disorders of functions of pelvic organs were also observed. In order to achieve a favorable outcome in terms of improved neurological status, the attention should be primarily focused on timely diagnosis and the choice of therapy in order to reduce the duration of the EC, which, in turn, has a major influence on the motor deficiency. This article represents a clinical case of development of congenital NB of a child during their first days of life accompanied with the clinic of the spine EC. The issues of differential diagnostics of pathological states of the early infancy period revealed with neurological symptoms were reviewed. On the reasons of arising of such issues may be NB. The issues of the clinic, diagnostics, treatment, and observation of such patients were reviewed separately.
Highlights
Несмотря на то, что дебют нейробластомы (НБ) в большинстве случаев приходится на возраст 18 месяцев жизни, данная солидная опухоль – одно из наиболее часто встречаемых злокачественных новообразований (ЗНО) в неонатальном периоде
The main objective of this publication is to highlight a rare clinical case; problems of differential diagnostics of neurological status with malformations and perinatal lesions of the nervous system; as well as the problems associated with the conduct of this group of patients
Epidural compression (EC) therapy caused with NB include poliochemotherapy, surgical intervention, and radiation therapy
Summary
Что дебют нейробластомы (НБ) в большинстве случаев приходится на возраст 18 месяцев жизни, данная солидная опухоль – одно из наиболее часто встречаемых злокачественных новообразований (ЗНО) в неонатальном периоде. Среди клинических проявлений врожденной НБ эпидуральная компрессия (ЭК), как признак распространения опухолевого процесса в канал спинного мозга, встречается крайне редко. В данной статье представлен клинический случай развития врожденной НБ у ребенка первых дней жизни, сопровождающийся клиникой ЭК спинного мозга.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
More From: Russian Journal of Children Hematology and Oncology
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.