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Diagnostic values of 68Ga-labelled DOTANOC PET/CT imaging in pediatric patients presenting with paraneoplastic opsoclonus myoclonus ataxia syndrome
- Source :
- European Radiology. 31:4587-4594
- Publication Year :
- 2021
- Publisher :
- Springer Science and Business Media LLC, 2021.
-
Abstract
- 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. 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. 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. 68Ga-DOTANOC PET/CT identified neuroblastic tumors with a high diagnostic accuracy in our cohort compared to histology and follow-up. • 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.
- Subjects :
- medicine.medical_specialty
medicine.diagnostic_test
business.industry
General Medicine
medicine.disease
Scintigraphy
Neuroblastic Tumor
030218 nuclear medicine & medical imaging
03 medical and health sciences
0302 clinical medicine
030220 oncology & carcinogenesis
Neuroblastoma
Opsoclonus myoclonus syndrome
medicine
Radiology, Nuclear Medicine and imaging
Histopathology
Ganglioneuroma
Radiology
business
Neuroradiology
Ganglioneuroblastoma
Subjects
Details
- ISSN :
- 14321084 and 09387994
- Volume :
- 31
- Database :
- OpenAIRE
- Journal :
- European Radiology
- Accession number :
- edsair.doi...........06fb1d9d1c947a0230b3ff6013fe21f7