68Ga-DOTATATE PET in Pediatric Paraganglioma / Pheochromocytoma: A Case-series Highlighting the Role of Functional Imaging
Aleksandra Augustynowicz1, Neha Kwatra2, Laura Drubach2, Christopher B. Weldon3,4,5, Katherine A. Janeway4, Steven G. DuBois4, Junne Kamihara4, and Stephan D. Voss2,4
Author Affiliations:
1Department of Radiology, Mount Auburn Hospital
2Department of Radiology, Boston Children’s Hospital
3Department of Surgery, Boston Children’s Hospital
4Dana-Farber/Boston Children’s Cancer and Blood Disorders Center
5Department of Anesthesiology, Critical Care & Pain Medicine. Boston Children’s Hospital
Keywords: Paraganglioma, Pheochromocytoma,68Ga-DOTATATE PET/CT
Title character count: 108
Number of references: 36
Number of figures: 4
Abstract word count: 198
Word count Text: 2919
Corresponding Author:
Stephan D. Voss, MD, PhD Department of Radiology, Boston Children’s Hospital Boston Children’s Hospital, 300 Longwood Avenue Boston, MA 02115email: stephan.voss@childrens.harvard.edu
Conflicts of Interest/funding:
SGD has received consulting fees from Bayer and travel expenses from Loxo, Roche, and Salarius. The other authors declare no conflicts of interest.
Abbreviations: Cancer predisposition syndromes – CPS; Pheochromocytoma Paraganglioma – PPGL; Inflammatory myofibroblastic tumor – IMT; Whole body MRI - wbMRI
Running Title: 68Ga-DOTATATE PET in Pediatric Paragangloma Pheochromocytoma
ABSTRACT
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors in childhood. Cancer predisposition syndromes (CPS) are increasingly recognized as the underlying cause for a number of pediatric malignancies and up to 40% of PPGL are currently thought to be associated with a hereditary predisposition1,2. With the increasingly widespread availability of functional molecular imaging techniques, nuclear medicine imaging modalities such as18F-FDG-PET/CT,123I-MIBG SPECT/CT, and68Ga-DOTATATE PET/CT now play an essential role in the staging, response assessment and determination of suitability for targeted radiotherapy in patients with PPGL. Each of these imaging modalities targets a different cellular characteristic, such as glucose metabolism (FDG), norepinephrine transporter expression (MIBG), or somatostatin receptor expression (DOTATATE), and therefore can be complementary to anatomic imaging and to each other. Given the recent FDA approval3 and increasing use of68Ga-DOTATATE for imaging in children4, the purpose of this article is to use a case-based approach to highlight both the advantages and limitations of DOTATATE imaging as it compares to current radiologic imaging techniques in the staging and response assessment of pediatric PPGL, and to offer a decision algorithm for the use of functional imaging that can be applied to PPGL, as well as other neuroendocrine malignancies.