Type:
Educational Exhibit
Keywords:
Nuclear medicine, Paediatric, PACS, Diagnostic procedure, Neoplasia
Authors:
S. Kohanzad, D. Sadowsky, P. Gerard, J. Meshekow; Valhalla, NY/US
DOI:
10.26044/ecr2021/C-10672
Background
Neuroblastoma is the most common extra-cranial solid tumor in pediatric patients.1 Pediatric opsoclonus-myoclonus syndrome (OMS) is a rare paraneoplastic syndrome associated with 20-50% of occult neuroblastoma cases.2 Patients present with the classic “dancing eyes, dancing feet” as well as behavioral abnormalities.3 The etiology of OMS is thought to be due to an autoimmune response directed against tumor antigens that cross-react against structures in the central nervous system.3 In contrast to neuroblastoma in patients who do not present with OMS, patients with OMS often have tumors that are smaller and located in the paravertebral regions with a lower likelihood of elevated catecholamine levels.3
In recent years, the I-metaiodobenzylguanidine (I-MIBG) scan has shown to be more sensitive and specific in detecting both primary neuroblastoma and metastatic lesions when compared to more traditional radiologic techniques, such as 99Tc-MDP bone scintigraphy, CT scans, and MRI images.4 5 The I-MIBG is taken up and stored in the chromaffin cells, allowing for proper tumor visualization.4 In cases of occult neuroblastoma, the I-MIBG scan is required for a proper diagnosis as serum and urine catecholamine levels can be normal and often misleading. With an accurate diagnosis of neuroblastoma, pediatric patients with OMS can receive proper treatment in the form of immunosuppressive agents, which allow for slight improvement.5