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 as 18F-FDG-PET/CT, 123I-MIBG
SPECT/CT, and 68Ga-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 of 68Ga-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.
Somatostatin receptor expression in lymphomas: a source of false diagnosis of neuroendocrine tumor at 68Ga-DOTANOC PET/CT imaging
