TY - JOUR
T1 - Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism
AU - Peranteau, William H.
AU - Mehdi Bathaii, Seyed
AU - Pawel, Bruce
AU - Hardy, Olga
AU - Alavi, Abass
AU - Stanley, Charles A.
AU - Adzick, N. Scott
PY - 2007/1/1
Y1 - 2007/1/1
N2 - Congenital hyperinsulinism (HI) exists in 2 histologic forms, focal and diffuse, and rarely has been attributed to lesions in ectopic pancreatic tissue. The ability to distinguish focal from diffuse HI and locate focal lesions has been difficult, thus limiting the optimal management of HI. We present a case of HI resulting from focal pancreatic and ectopic pancreatic lesions. After a near-total pancreatectomy failed to improve the patient's condition, a positron emission tomography (PET) scan performed with 18F-fluoro-l-dihydroxyphenylalanine demonstrated a focal lesion remaining in the head of the pancreas as well as 4 hot spots inferior to the remaining pancreas. Surgical exploration found pancreatic rests in the jejunum responsible for the hot spots seen on PET. Resection of the remainder of the pancreas as well as the small intestinal lesions resulted in correction of the patient's HI. Pathology confirmed the presence of focal HI lesions in the pancreatic head and small intestinal specimens. This case supports the ability of ectopic pancreatic tissue to contribute to the pathology of HI. It highlights the ability of PET to successfully identify focal lesions, including ectopic tissue, responsible for hyperinsulinemic hypoglycemia.
AB - Congenital hyperinsulinism (HI) exists in 2 histologic forms, focal and diffuse, and rarely has been attributed to lesions in ectopic pancreatic tissue. The ability to distinguish focal from diffuse HI and locate focal lesions has been difficult, thus limiting the optimal management of HI. We present a case of HI resulting from focal pancreatic and ectopic pancreatic lesions. After a near-total pancreatectomy failed to improve the patient's condition, a positron emission tomography (PET) scan performed with 18F-fluoro-l-dihydroxyphenylalanine demonstrated a focal lesion remaining in the head of the pancreas as well as 4 hot spots inferior to the remaining pancreas. Surgical exploration found pancreatic rests in the jejunum responsible for the hot spots seen on PET. Resection of the remainder of the pancreas as well as the small intestinal lesions resulted in correction of the patient's HI. Pathology confirmed the presence of focal HI lesions in the pancreatic head and small intestinal specimens. This case supports the ability of ectopic pancreatic tissue to contribute to the pathology of HI. It highlights the ability of PET to successfully identify focal lesions, including ectopic tissue, responsible for hyperinsulinemic hypoglycemia.
KW - Congenital hyperinsulinism
KW - Ectopic pancreatic tissue
KW - F-fluoro-l-dihydroxyphenylalanine
KW - Focal hyperinsulinism
KW - Nesidioblastosis
KW - PET scan
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U2 - 10.1016/j.jpedsurg.2006.09.046
DO - 10.1016/j.jpedsurg.2006.09.046
M3 - Article
C2 - 17208563
AN - SCOPUS:33845930865
SN - 0022-3468
VL - 42
SP - 188
EP - 192
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 1
ER -