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Diagnostic value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography imaging in pediatric opsoclonus myoclonus ataxia syndrome presenting with neuroblastoma

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Abstract

Background

Early precision diagnosis and effective treatment of opsoclonus myoclonus ataxia syndrome (OMAS) patients presenting with neuroblastoma can prevent serious neurological outcomes.

Objective

To assess the diagnostic value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging in pediatric OMAS with neuroblastoma.

Materials and methods

A retrospective evaluation of 45 patients diagnosed with OMAS who underwent 18F-FDG PET/CT was performed. A univariate analysis was performed to compare clinical characteristics between OMAS with and without neuroblastoma. Univariate and multivariate logistic regression analyses were applied to identify independent risk factors for OMAS with neuroblastoma and to develop the clinical model. Finally, independent risk factors and PET/CT were fitted to build the combined model for the diagnosis of OMAS with neuroblastoma and presented as a nomogram. Receiver operating characteristic curve, decision curve, and calibration curve analyses were conducted to evaluate the performance of the models.

Results

Among 45 patients, 27 were PET/CT-positive, 23/27 lesions were neuroblastoma, and four were false positives. One of the false positive patients was confirmed to be adrenal reactive hyperplasia by postoperative pathology, and the symptoms of OMAS disappeared in the remaining three cases during clinical follow-up. The average maximal standardized uptake value of PET/CT-positive lesions was 2.6. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of PET/CT were 100%, 81.8%, 85.2%, 100%, and 91.1%, respectively. Age at diagnosis, lactate dehydrogenase, and neuron-specific enolase showed statistically significant differences between OMAS with and without neuroblastoma. Lactate dehydrogenase was identified as the independent risk factor to develop the clinical model, and the clinical model demonstrated an area under the curve (AUC) of 0.82 for the diagnosis of OMAS with neuroblastoma, with an AUC as high as 0.91 when combined with PET/CT. The decision curve analysis and calibration curve demonstrated that the nomogram had good consistency and clinical usefulness.

Conclusion

In patients with OMAS, 18F-FDG PET/CT has a high diagnostic accuracy in detecting tumors of the neuroblastoma, especially when combined with the independent risk factor serum lactate dehydrogenase.

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Data availability

The datasets analyzed during the current study are available from the corresponding authors on reasonable request.

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Funding

This work was supported by the National Natural Science Foundation of China (No. 82272034).

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Author notes

  1. Lijuan Feng and Shen Yang contributed equally to this work.

Authors and Affiliations

  1. Department of Nuclear Medicine, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, China

    Lijuan Feng & Jigang Yang

  2. Department of Surgical Oncology, Beijing Children’s Hospital, Beijing, China

    Shen Yang, Yu Lin & Huanmin Wang

  3. Department of Neurology, Beijing Children’s Hospital, Beijing, China

    Jiuwei Li

  4. Department of Thoracic Surgery and Surgical Oncology, Children’s Hospital, Capital Institute of Pediatrics, Beijing, China

    Zhenhua Cao & Qipeng Zheng

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  1. Lijuan Feng
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Contributions

Conceptualization: L.F., S.Y., H.W., and J.Y.; data curation: L.F., S.Y., and Y.L.; formal analysis: J.L., Z.C., and Q.Z.; funding acquisition: J.Y.; investigation: S.Y. and Y.L.; methodology: L.F., S.Y., and Y.L.; project administration: H.W. and J.Y.; resources: H.W. and J.Y.; software: L.F., S.Y., and J.L.; supervision: H.W. and J.Y.; validation: L.F., S.Y., J.L., Z.C., and Q.Z.; visualization: Y.L., J.L., Z.C., Q.Z., and Q.Z.; writing — original draft preparation: L.F., S.Y., Y.L., J.L., Z.C., Q.Z., H.W., and J.Y.; writing  — review and editing: L.F., S.Y., Y.L., J.L., Z.C., Q.Z., H.W., and J.Y. All authors reviewed and approved the final manuscript.

Corresponding author

Correspondence to Jigang Yang.

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Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Institutional Review Board of Beijing Friendship Hospital, Capital Medical University.

Informed consent

The requirement to obtain informed consent was waived by the Institutional Review Board due to the use of retrospective anonymized data.

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Feng, L., Yang, S., Lin, Y. et al. Diagnostic value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography imaging in pediatric opsoclonus myoclonus ataxia syndrome presenting with neuroblastoma. Pediatr Radiol (2024). https://doi.org/10.1007/s00247-024-05921-9

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