Neuroblastoma in children: What has changed in the 21^st century

Abstract

Among the childhood cancers, neuroblastoma ranks fourth. This tumour is considered the most common extracranial malignant neoplasm in children and was first described in 1865 by the German scientist Rudolf Virchow. Neuroblastoma has the unique ability to undergo increased cell differentiation and transform into ganglioneuroma. In some cases, the disease can be asymptomatic and may undergo spontaneous regression or maturation into a benign tumour. However, neuroblastoma often has an aggressive course with early metastasis. Due to the ambiguity of clinical symptoms, the primary diagnosis of neuroblastoma in children requires pediatricians to be acutely aware of oncological emergencies and initial signs such as increased abdominal size and asymmetry, neurological symptoms, pain, and dysuria. In low-risk patients, minimal therapy, including surgery alone, achieves long-term survival in more than 90% of cases. Achieving similarly high survival rates in the intermediate-risk group is possible only with the use of modern polychemotherapy regimens in combination with surgical treatment and, in some cases, radiation therapy. High-risk patients require a combination of the aforementioned methods together with autologous or allogeneic hematopoietic stem cell transplantation, with long-term overall survival rates not exceeding 50%. The most favorable prognosis is seen in patients with localised disease under the age of one year. This article describes the features of tumour development, the course of the disease, and the evolution of diagnostic and therapeutic strategies from the past to the present.