Orthopedic disorders in children suffering from neuroblastoma with intraspinal extension: the experience of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology

Abstract

Neuroblastoma (NB) is the most common extracranial tumor in children. In 5–15% of cases, the tumor extends into the spinal canal and can potentially cause neurological deficits and orthopedic problems that can develop both at the onset of the disease and at a later time. We analyzed data of 61 patients with NB and intraspinal extension who had been treated at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology over the period from Jan 2012 to Dec 2018. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. The treatment was delivered in accordance with the NB-2004 protocol. In all the children, magnetic resonance imaging and/or computed tomography of the CNS were performed to evaluate intraspinal extension of the tumor as well as the degree of spinal cord compression. The presence of scoliosis and its severity were determined at the baseline and at 2 years after the diagnosis using imaging data and Cobb angle measurement. Scoliosis was classified as mild if the Cobb angle was 10–25°, moderate if it was 25–40°, and severe if it exceeded 40°. In our study, 7/61 (12%) patients were diagnosed with scoliosis at the baseline assessment. The median age at diagnosis was 8.0 (2.3–11.8) months. The male to female ratio was 2.5:1. In 4/7 (57%) patients, the primary tumor was located in the retroperitoneum (outside the major organs), and in 3/7 (43%) patients – in the posterior mediastinum. In this group, 4/7 (57%) patients had INSS stage 2 or 3 tumors, 2/7 (29%) patients had stage 4 disease, and 1/7 (14%) had INSS stage 4S. The majority of patients (5/7 (71%)) were stratified into an observation group. In 6/7 (86%) patients, the tumor extended into the spinal canal involving the thoracic spine. In 6/7 (86%) cases, there was evidence of complete obstruction of the spinal canal. Neurosurgery was performed in 4/7 (57%) patients. All these patients were diagnosed with mild scoliosis at the baseline. At 2 years after the diagnosis, imaging data were available for 38/54 (70%) patients who had not had scoliosis at the baseline. This time, scoliosis was diagnosed in 9/38 (24%) cases. The median age at NB diagnosis was 8.2 (0.8–42.3) months, the male to female ratio was 2:1. In 7/9 (78%) patients, the primary tumor was located in the posterior mediastinum. The majority of patients were stratified into an observation group (7/9 (78%)). In 8/9 (89%) patients, the tumor extended into the spinal canal involving the thoracic vertebrae. In the majority of patients (4/9(44%)), the tumor filled 33 to 66% of the spinal canal. Neurosurgery was performed in 6/9 (67%) patients. In this group, 7/9 (78%) patients were diagnosed with mild scoliosis and 2/9 (22%) patients – with moderate scoliosis. NB with intraspinal extension can lead to various orthopedic problems including scoliosis that can be revealed both at the onset of the disease and at a later time, meaning that this condition requires a multidisciplinary approach involving orthopedic specialists.