PMC8322518_01
A focal lesion within the pancreatic head/uncinate region (+) is visualized on 3D-MIP image (A) and fused PET/CT image (B) in patient 1. Two focal lesions are identified in patient 2 (C-F). 3D-MIP image at 50 min (C) shows one lesion in the pancreatic head (+) and the other in the proximal pancreatic tail. Fused PET/CT image (D) shows the focal lesion in the pancreatic head (+). Rotated 3D-MIP image (E) permits better visualization of the proximal tail lesion (+), which is shown on fused PET/CT image (F) to be exophytic along posterior margin of pancreas. High uptake in the kidneys and bladder is visualized in all images, as expected, due to renal excretion of the radioisotope. Images were originally published in JNM. States LJ, Davis JC, Hamel SM, Becker SA, Zhuang H. Imaging of congenital hyperinsulinism. J Nucl Med.   SNMMI.
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PMC8322518_02_fped-09-699129-g0001.jpg
F1
In addition, a single base change in intron 16 (c.2222+15 C>A) of the ABCC8 gene was also identified in peripheral blood DNA; parent of origin was not determined. 18F-DOPA PET/CT revealed two separate foci of increased uptake: an exophytic lesion arising from the posterior aspect of the pancreatic body and a lesion on the medial aspect of the pancreatic head (Figures 1C-F).
Patient 1. Focal lesions from the pancreatic head [(A) hematoxylin and eosin [H&E], (B) chromogranin immunohistochemical stain [IHC], both at x20 original magnification] and body [(E) H&E, x20 original magnification] composed of localized increases in endocrine tissue with occasional ducts (arrowhead) [(C,F) H&E, x200 original magnification]. Endocrine cells demonstrate loss of nuclear immunoreactivity for p57 in both lesions with non-specific reactivity within the cytoplasm [(D,G) p57 IHC, x200 original magnification].
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F2
Histopathology showed that both resected regions demonstrated localized islet cell hyperplasia with occasional nucleomegaly surrounded by normal pancreas, consistent with focal HI (Figure 2).
PMC8322518_02
A focal lesion within the pancreatic head/uncinate region (+) is visualized on 3D-MIP image (A) and fused PET/CT image (B) in patient 1. Two focal lesions are identified in patient 2 (C-F). 3D-MIP image at 50 min (C) shows one lesion in the pancreatic head (+) and the other in the proximal pancreatic tail. Fused PET/CT image (D) shows the focal lesion in the pancreatic head (+). Rotated 3D-MIP image (E) permits better visualization of the proximal tail lesion (+), which is shown on fused PET/CT image (F) to be exophytic along posterior margin of pancreas. High uptake in the kidneys and bladder is visualized in all images, as expected, due to renal excretion of the radioisotope. Images were originally published in JNM. States LJ, Davis JC, Hamel SM, Becker SA, Zhuang H. Imaging of congenital hyperinsulinism. J Nucl Med.   SNMMI.
fped-09-699129-g0001.jpg
PMC8322518_02_fped-09-699129-g0001.jpg
F1
In addition, a single base change in intron 16 (c.2222+15 C>A) of the ABCC8 gene was also identified in peripheral blood DNA; parent of origin was not determined. 18F-DOPA PET/CT revealed two separate foci of increased uptake: an exophytic lesion arising from the posterior aspect of the pancreatic body and a lesion on the medial aspect of the pancreatic head (Figures 1C-F).
Patient 2. Two distinct focal lesions arising in the pancreatic head (A,B) and body (C,D) each demonstrating localized increases in endocrine tissue which are highlighted on chromogranin immunohistochemical (IHC) stain [(A,C) hematoxylin and eosin [H&E], (B,D) chromogranin IHC, all at x20 original magnification]. Higher-power magnification of head lesion demonstrating increased endocrine cells with rare intermixed acinar cells (arrow) and small ducts (arrowhead) [(E) H&E, x200 original magnification] with loss of nuclear p57 immunoreactivity within lesional cells [(F) p57 IHC, x200 original magnification]. Compared to lesional areas, normal pancreatic tissue shows relatively small islets (arrow) composed of endocrine cells which are positive for chromogranin but with retained nuclear p57 immunoreactivity; non-specific p57 immunoreactivity is observed within the cytoplasm [(G) H&E, (H) chromogranin IHC, (I) p57 IHC, all at x200 original magnification].
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F3
Final histopathology was consistent with focal islet cell hyperplasia (Figure 3).