Islets of hypoglycaemia: a rare case of adult onset nesidioblastosis

A case of diffuse nesidioblastosis in an adult patient is reported in this study. A 24-year-old female with no known comorbidities presented with multiple episodes of documented recurrent hypoglycaemia and Hypoglycaemia induced seizures both in fasting and postprandial state. Her blood investigations revealed low plasma glucose levels, high insulin and C-peptide levels with positive 72-hour fast test. Her transabdominal USG and CECT abdomen did not reveal any abnormality, 68Ga DOTANOC PET CT done showed ill-defined diffuse somatostatin receptor expression in the pancreatic head and tail suggestive of nesidioblastosis. As patient was not willing for surgical treatment, hence started on medical treatment with oral nifedipine. Nesidioblastosis is very rare in adults. It is an important differential diagnosis in adults with hyperinsulinemic hypoglycemia although the incidence is very rare in adults. PET SCAN was used to non-invasively diagnose nesidioblastosis in this case. Surgery being the preferred choice of treatment in nesidioblastosis, there is limited data on medical line of management in nesidioblastosis.

Orbital IgG4 Disease: Imaging Findings on 68Ga-DOTANOC PET/CT / 대한핵의학회잡지

Immunoglobulin G4 (IgG4)–related diseases are a spectrum of systemic inflammatory conditions of unknown etiology, which are characterized by infiltration of tissues by IgG4 plasma cells and sclerosing inflammation (Cheuk and Chan Adv Anat Pathol 17:303-32, 2010). Although this condition was initially described in relation to autoimmune pancreatitis, now it has been reported in almost every organ system of body (Zen and Nakanuma Am J Surg Pathol 34:1812-9, 2010, Masaki et al. Ann Rheuma Dis 68:1310-5, 2009). Orbital involvement by IgG4 disease can involve extraocular muscles (EOM), lacrimal glands, conjunctiva, eyelids, infraorbital nerve, orbital fat, and nasolacrimal system (McNab and McKelvie. Ophthal Plast Reconstr Surg 31:167-78, 2015, Katsura et al. Neuroradiology 54:873-82, 2012). The basis of using ⁶⁸Ga-DOTANOC PET/CT in IgG4 orbital disease is the known expression of somatostatin receptors in chronic inflammatory cells (Cuccurullo et al. Indian J Radiol Imaging 27:509-16, 2017) and also avidity shown previously in other IgG4-related diseases (Cheng et al. Clin Nucl Med 43:773-6, 2018).

Von Hippel-Lindau Syndrome: Demonstration of Entire Disease Spectrum with 68Ga-DOTANOC PET-CT

Von Hippel-Lindau (VHL) syndrome is a rare neoplastic disorder characterized by central nervous system (CNS) and visceral tumors. We here present 68Ga-labelled [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-Nal3-Octreotide positron emission tomography computed tomography findings in a 52 year old female with VHL syndrome, demonstrating both CNS and visceral tumors.