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.