CONTEXT: Congenital hyperinsulinism (CHI) is a rare disease characterized by severe hypoglycaemic episodes due to pathologically increased insulin secretion from the pancreatic beta cells. When untreated, CHI might result in irreversible brain damage and death. Currently, two major subtypes of CHI are known: a focal form, associated with local distribution of affected beta cells and a nonfocal form, affecting every single beta cell. The identification of focal forms is important, as the patients can be cured by limited surgery. (18) F DOPA-PET/CT is an established non-invasive approach to differentiate focal from nonfocal CHI. OBJECTIVE: The purpose of this study was to identify possible limitations of (18) F DOPA-PET/CT scan in patients with focal forms nonfocal CHI. DESIGN: A retrospective chart review of 32 patients (from 2008 through 2013) who underwent (18) F DOPA-PET/CT and partial pancreatectomy for focal CHI at the reference centres in Berlin, Germany and London, UK. RESULTS: In most cases (n = 29, 90·7%), (18) F DOPA-PET/CT was sufficient to localize the complete focal lesion. However, in some patients (n = 3, 9·3%), (18) F DOPA-PET/CT wrongly visualized only a small portion of the focal lesion. In this group of patients, a so-called 'giant focus' was detected in histopathological analysis during the surgery. CONCLUSIONS: Our data show that in most patients with focal CHI (18) F DOPA-PET/CT correctly predicts the size and anatomical localisation of the lesion. However, in those patients with a 'giant focal' lesion (18) F DOPA-PET/CT is unreliable for correct identification of 'giant focus' cases.
CONTEXT: Congenital hyperinsulinism (CHI) is a rare disease characterized by severe hypoglycaemic episodes due to pathologically increased insulin secretion from the pancreatic beta cells. When untreated, CHI might result in irreversible brain damage and death. Currently, two major subtypes of CHI are known: a focal form, associated with local distribution of affected beta cells and a nonfocal form, affecting every single beta cell. The identification of focal forms is important, as the patients can be cured by limited surgery. (18) F DOPA-PET/CT is an established non-invasive approach to differentiate focal from nonfocal CHI. OBJECTIVE: The purpose of this study was to identify possible limitations of (18) F DOPA-PET/CT scan in patients with focal forms nonfocal CHI. DESIGN: A retrospective chart review of 32 patients (from 2008 through 2013) who underwent (18) F DOPA-PET/CT and partial pancreatectomy for focal CHI at the reference centres in Berlin, Germany and London, UK. RESULTS: In most cases (n = 29, 90·7%), (18) F DOPA-PET/CT was sufficient to localize the complete focal lesion. However, in some patients (n = 3, 9·3%), (18) F DOPA-PET/CT wrongly visualized only a small portion of the focal lesion. In this group of patients, a so-called 'giant focus' was detected in histopathological analysis during the surgery. CONCLUSIONS: Our data show that in most patients with focal CHI (18) F DOPA-PET/CT correctly predicts the size and anatomical localisation of the lesion. However, in those patients with a 'giant focal' lesion (18) F DOPA-PET/CT is unreliable for correct identification of 'giant focus' cases.
Authors: Milena Pizzoferro; Gabriele Masselli; Arianna Maiorana; Emanuele Casciani; Saadi Sollaku; Carlo Dionisi-Vici; Marco Spada; Claudio Altini; Maria Felicia Villani; Vittoria Rufini; Gianfranco Gualdi; Maria Carmen Garganese Journal: Am J Nucl Med Mol Imaging Date: 2022-04-15
Authors: Bing Han; Melanie Newbould; Gauri Batra; Edmund Cheesman; Ross J Craigie; Zainab Mohamed; Lindsey Rigby; Raja Padidela; Mars Skae; Aleksandr Mironov; Tobias Starborg; Karl E Kadler; Karen E Cosgrove; Indraneel Banerjee; Mark J Dunne Journal: Am J Clin Pathol Date: 2016-06 Impact factor: 2.493
Authors: Ross J Craigie; Maria Salomon-Estebanez; Daphne Yau; Bing Han; Walaa Mal; Melanie Newbould; Edmund Cheesman; Stefania Bitetti; Zainab Mohamed; Rakesh Sajjan; Raja Padidela; Mars Skae; Sarah Flanagan; Sian Ellard; Karen E Cosgrove; Indraneel Banerjee; Mark J Dunne Journal: Front Endocrinol (Lausanne) Date: 2018-10-17 Impact factor: 5.555
Authors: Maria Gϋemes; Sofia Asim Rahman; Ritika R Kapoor; Sarah Flanagan; Jayne A L Houghton; Shivani Misra; Nick Oliver; Mehul Tulsidas Dattani; Pratik Shah Journal: Rev Endocr Metab Disord Date: 2020-12 Impact factor: 6.514