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Diagnostic values of 68Ga-labelled DOTANOC PET/CT imaging in pediatric patients presenting with paraneoplastic opsoclonus myoclonus ataxia syndrome

  • Nuclear Medicine
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Objectives

Opsoclonus myoclonus ataxia (OMA) syndrome, also known as “Kinsbourne syndrome” or “dancing eye syndrome,” is a rare, paraneoplastic entity which may be associated with pediatric neuroblastic tumors and carry a grave prognosis. We aimed to evaluate the role of 68Ga DOTANOC PET/CT for detecting neuroblastic tumors in patients with OMA syndrome.

Methods

We retrospectively evaluated the 68Ga-DOTANOC PET/CT data of pediatric patients presenting with OMA syndrome from March 2012 to November 2018. A somatostatin receptor (SSTR)-expressing lesion with corresponding morphological change on CT image was considered PET-positive, while no abnormal SSTR expression or lesion was noticed in PET-negative patients. Histopathology and/or clinical/imaging follow-up (minimum one year) was considered a reference standard for comparing the PET/CT findings. The results of 68Ga-DOTANOC PET/CT were also compared with 131I MIBG whole-body scintigraphy, which was available in five patients.

Results

Of 38 patients (13 males, 25 females, aged 3–96 months), 18 (47.3%) had SSTR-expressing lesions (PET-positive), and histopathology revealed neuroblastic tumors in 17/18 lesions (neuroblastoma 14, ganglioneuroblastoma 2, and ganglioneuroma 1) and reactive hyperplasia in 1/18. The remaining 20/38 (52.6%) patients did not demonstrate SSTR-expressing lesions (PET-negative) and had an uneventful follow-up. The average SUVmax of the PET-positive lesions was 10.3 (range 2.8–34.5). The PET/CT results revealed 17 true-positive, one false-positive, 20 true-negative, and zero false-negative. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 100%, 95.2%, 94.4%, 100%, and 97.3% respectively.

Conclusions

68Ga-DOTANOC PET/CT identified neuroblastic tumors with a high diagnostic accuracy in our cohort compared to histology and follow-up.

Key Points

• Opsoclonus myoclonus ataxia (OMA) syndrome or “dancing eye syndrome” is a rare paraneoplastic entity which may be associated with pediatric neuroblastic tumors with a grave prognosis.

• 123I/131I MIBG imaging has a proven role for functional imaging in neuroblastoma or patients with OMA, but the role of 68Ga-DOTANOC PET/CT is not yet studied.

• 68Ga-labelled DOTANOC PET/CT (SSTR) imaging, in our cohort, was able to positively identify neuroblastic tumors with high diagnostic accuracy when compared with histology.

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Abbreviations

CT:

Computed tomography

KeV:

Kilo electron volt

mA:

Milli ampere

MBq:

Megabecquerel

mCi:

Millicurie

MIBG:

Metaiodobenzyl guanidine

MRI:

Magnetic resonance imaging

OMA syndrome:

Opsoclonus myoclonus ataxia syndrome

OSEM:

Ordered subset expectation maximization

PET/CT:

Positron emission tomography computed tomography

SPECT/CT:

Single-photon emission tomography/computed tomography

SSTR:

Somatostatin receptor

SUVmax:

Standardized uptake value

USG:

Ultrasonography

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The authors state that this work has not received any funding.

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Author notes

  1. Rajender Kumar and Kousik Vankadari contributed equally as first authors.

Authors and Affiliations

  1. Department of Nuclear Medicine and PET/CT, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India

    Rajender Kumar, Kousik Vankadari & Bhagwant Rai Mittal

  2. Department of Pediatric Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

    Deepak Bansal & Amita Trehan

  3. Department of Pediatric Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

    Jitendra K. Sahu & Naveen Sankhyan

Authors
  1. Rajender Kumar
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  2. Kousik Vankadari
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Correspondence to Bhagwant Rai Mittal.

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The scientific guarantor of this publication is Prof. Bhagwant Rai Mittal

Conflict of interest

The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

No complex statistical methods were necessary for this paper.

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Written informed consent was waived by the Institute ethics committee due to retrospective and observational nature of the study.

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Institute ethics committee approved the study.

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Kumar, R., Vankadari, K., Mittal, B.R. et al. Diagnostic values of 68Ga-labelled DOTANOC PET/CT imaging in pediatric patients presenting with paraneoplastic opsoclonus myoclonus ataxia syndrome. Eur Radiol 31, 4587–4594 (2021). https://doi.org/10.1007/s00330-020-07587-x

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  • DOI: https://doi.org/10.1007/s00330-020-07587-x

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