Congenital hyperinsulinism(CHI) is a rare but complex heterogeneous disorder caused by unregulated secre-tion of insulin from the β-cells of the pancreas leading to severe hypoglycaemia and neuroglycopaenia. Swift di...Congenital hyperinsulinism(CHI) is a rare but complex heterogeneous disorder caused by unregulated secre-tion of insulin from the β-cells of the pancreas leading to severe hypoglycaemia and neuroglycopaenia. Swift diagnosis and institution of appropriate management is crucial to prevent or minimise adverse neurodevel-opmental outcome in children with CHI. Histologically there are two major subtypes of CHI, diffuse and focal disease and the management approach will significantly differ depending on the type of the lesion. Patients with medically unresponsive diffuse disease require a near total pancreatectomy, which then leads on to the de-velopment of iatrogenic diabetes mellitus and pancre-atic exocrine insufficiency. However patients with focaldisease only require a limited pancreatectomy to re-move only the focal lesion thus providing complete cure to the patient. Hence the preoperative differentiation of the histological subtypes of CHI becomes paramount in the management of CHI. Fluorine-18L-3, 4-hydroxy-phenylalanine positron emission tomography(18F-DOPA-PET) is now the gold standard for pre-operative differentiation of focal from diffuse disease and locali-sation of the focal lesion. The aim of this review article is to give a clinical overview of CHI, then review the role of dopamine in β-cell physiology and finally discuss the role of 18F-DOPA-PET imaging in the management of CHI.展开更多
BACKGROUND Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography(F-18 FDG PET/CT),a functional imaging method,is usually performed on the entire torso,and regions of unexpected suspicious foc...BACKGROUND Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography(F-18 FDG PET/CT),a functional imaging method,is usually performed on the entire torso,and regions of unexpected suspicious focal hypermetabolism are not infrequently observed.Among the regions,colon,thyroid,and prostate were found to be the common organs in a recent umbrella review.Some studies reported that a high rate of malignancy was shown in incidentally identified focal hypermetabolic regions and suggested that further examinations should not be ignored.AIM To investigate the malignancy rate of incidental focal FDG uptake,useful PET parameters and their cutoffs in discrimination between malignant and benign lesions.METHODS Retrospectively,the final reports of 16510 F-18 FDG PET/CT scans performed at our hospital between January 2016 and March 2022 were reviewed to identify incidentally observed FDG uptake in the colon/rectum,thyroid,and prostate.The scans of patients with current or prior malignancies at each corresponding location,without the final reports of histopathology or colonoscopy(for colon and rectum)for the corresponding hypermetabolic regions,or with diffuse(not focal)hypermetabolism were excluded.Finally,88 regions of focal colorectal hypermetabolism in 85 patients(48 men and 37 women with mean age 67.0±13.4 years and 63.4±15.8 years,respectively),48 focal thyroid uptakes in 48 patients(12 men and 36 women with mean age 62.2±13.1 years and 60.8±12.4 years,respectively),and 39 focal prostate uptakes in 39 patients(mean age 71.8±7.5 years)were eligible for this study.For those unexpected focal hypermetabolic regions,rates of malignancy were calculated,PET parameters,such as standardized uptake value(SUV),capable of distinguishing between malignant and benign lesions were investigated,and the cutoffs of those PET parameters were determined by plotting receiver operating characteristic curves.RESULTS In the colon and rectum,29.5%(26/88)were malignant and 33.0%(29/88)were premalignant lesions.Both SUVmax 展开更多
文摘Congenital hyperinsulinism(CHI) is a rare but complex heterogeneous disorder caused by unregulated secre-tion of insulin from the β-cells of the pancreas leading to severe hypoglycaemia and neuroglycopaenia. Swift diagnosis and institution of appropriate management is crucial to prevent or minimise adverse neurodevel-opmental outcome in children with CHI. Histologically there are two major subtypes of CHI, diffuse and focal disease and the management approach will significantly differ depending on the type of the lesion. Patients with medically unresponsive diffuse disease require a near total pancreatectomy, which then leads on to the de-velopment of iatrogenic diabetes mellitus and pancre-atic exocrine insufficiency. However patients with focaldisease only require a limited pancreatectomy to re-move only the focal lesion thus providing complete cure to the patient. Hence the preoperative differentiation of the histological subtypes of CHI becomes paramount in the management of CHI. Fluorine-18L-3, 4-hydroxy-phenylalanine positron emission tomography(18F-DOPA-PET) is now the gold standard for pre-operative differentiation of focal from diffuse disease and locali-sation of the focal lesion. The aim of this review article is to give a clinical overview of CHI, then review the role of dopamine in β-cell physiology and finally discuss the role of 18F-DOPA-PET imaging in the management of CHI.
文摘BACKGROUND Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography(F-18 FDG PET/CT),a functional imaging method,is usually performed on the entire torso,and regions of unexpected suspicious focal hypermetabolism are not infrequently observed.Among the regions,colon,thyroid,and prostate were found to be the common organs in a recent umbrella review.Some studies reported that a high rate of malignancy was shown in incidentally identified focal hypermetabolic regions and suggested that further examinations should not be ignored.AIM To investigate the malignancy rate of incidental focal FDG uptake,useful PET parameters and their cutoffs in discrimination between malignant and benign lesions.METHODS Retrospectively,the final reports of 16510 F-18 FDG PET/CT scans performed at our hospital between January 2016 and March 2022 were reviewed to identify incidentally observed FDG uptake in the colon/rectum,thyroid,and prostate.The scans of patients with current or prior malignancies at each corresponding location,without the final reports of histopathology or colonoscopy(for colon and rectum)for the corresponding hypermetabolic regions,or with diffuse(not focal)hypermetabolism were excluded.Finally,88 regions of focal colorectal hypermetabolism in 85 patients(48 men and 37 women with mean age 67.0±13.4 years and 63.4±15.8 years,respectively),48 focal thyroid uptakes in 48 patients(12 men and 36 women with mean age 62.2±13.1 years and 60.8±12.4 years,respectively),and 39 focal prostate uptakes in 39 patients(mean age 71.8±7.5 years)were eligible for this study.For those unexpected focal hypermetabolic regions,rates of malignancy were calculated,PET parameters,such as standardized uptake value(SUV),capable of distinguishing between malignant and benign lesions were investigated,and the cutoffs of those PET parameters were determined by plotting receiver operating characteristic curves.RESULTS In the colon and rectum,29.5%(26/88)were malignant and 33.0%(29/88)were premalignant lesions.Both SUVmax
