Learning objectives
To provide an overview of the imaging tools used for the evaluation of pediatric opsoclonus-myoclonus syndrome is essential for early diagnosis.
To discuss the superior sensitivity and specificity of I-MIBG for greater efficacy in the detection of neuroblastoma.
To describe how an initial I-MIBG scan allows for localization of neuroblastoma tumors, which can prevent unnecessary and repetitive radiological testing.
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...
Findings and procedure details
Neuroblastoma is a tumor of sympathetic origin and can have primary sites in the adrenal medulla or along the paravertebral sympathetic chain.
The tumor often presents with distant metastases most commonly in the bone, bone marrow, lymph nodes and liver.
The I-MIBG scan can more precisely localize the tumor lesions in the body.
Additionally, the SPECT-CT helps with localization on the I-MIBG scan. [Fig 1] [Fig 2]
Conclusion
Neuroblastoma is a pediatric tumor of relatively high frequency. Certain cases of occult neuroblastoma can present with the opsoclonus-myoclonus syndrome. In these cases, diagnosis of neuroblastoma requires the I-MIBG scan for proper detection of primary and secondary tumors, as urine and serum catecholamine levels can often be normal. The I-MIBG scan is highly specific and sensitive in detecting neuroblastoma and should be considered the test of choice by radiologists in screening procedures. This test will allow for efficiency in diagnosis and treatment of these pediatric...
Personal information and conflict of interest
S. Kohanzad:
Nothing to disclose
D. Sadowsky:
Nothing to disclose
P. Gerard:
Nothing to disclose
J. Meshekow:
Nothing to disclose
References
Kushner BH. Neuroblastoma: a disease requiring a multitude of imaging studies.Journal of Nuclear Medicine 2004;45:1172-1188.
Parisi MT, Hattner RS, Matthay KK, Berg BO, Sandler ED. Optimized diagnostic strategy for neuroblastoma in opsoclonus-myoclonus. Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine 1993; 11:1922-1926.
Hero B, Schleiermacher G. Update on pediatric opsoclonus myoclonus syndrome. Neuropediatrics 2013; 44:324-329.
Jacobs A, Delree M, Desprechins B, et al. Consolidating the role of I-MIBG-scintigraphy in childhood neuroblastoma: Five years of clinical experience. Pediatric Radiology 1990; 20:157-159.
Bouvier JF, Philip...