Indian Journal of Nuclear Medicine
Home | About IJNM | Search | Current Issue | Past Issues | Instructions | Ahead of Print | Online submissionLogin 
Indian Journal of Nuclear Medicine
  Editorial Board | Subscribe | Advertise | Contact
Users Online: 586 Print this page  Email this page Small font size Default font size Increase font size


 
 Table of Contents     
CASE REPORT
Year : 2013  |  Volume : 28  |  Issue : 3  |  Page : 163-164  

Diffuse nesidioblastosis diagnosed on a Ga-68 DOTATATE positron emission tomography/computerized tomography


1 Department of Nuclear Medicine and PET, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Paediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication9-Oct-2013

Correspondence Address:
Bhagwant Rai Mittal
Department of Nuclear Medicine and PET, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-3919.119547

Rights and Permissions
   Abstract 

The authors describe a 50 days old pre-term infant with persistent hyperinsulinemic hypoglycemia of infancy in whom Ga-68 DOTATATE positron emission tomography/computerized tomography scan showed diffusely increased tracer uptake in the entire pancreas with no abnormal tracer uptake anywhere else in the body, suggestive of a diffuse variant of nesidioblastosis.

Keywords: Ga-68 DOTATATE, hyperinsulinemic hypoglycaemia, infant, nesidioblastosis, positron emission tomography/computerized tomography


How to cite this article:
Arun S, Mittal BR, Shukla J, Bhattacharya A, Kumar P. Diffuse nesidioblastosis diagnosed on a Ga-68 DOTATATE positron emission tomography/computerized tomography. Indian J Nucl Med 2013;28:163-4

How to cite this URL:
Arun S, Mittal BR, Shukla J, Bhattacharya A, Kumar P. Diffuse nesidioblastosis diagnosed on a Ga-68 DOTATATE positron emission tomography/computerized tomography. Indian J Nucl Med [serial online] 2013 [cited 2019 Nov 14];28:163-4. Available from: http://www.ijnm.in/text.asp?2013/28/3/163/119547


   Introduction Top


Nesidioblastosis is a pathological condition caused by neo-differentiation of  Islets of Langerhans More Details from pancreatic ductal epithelium. This condition later renamed as persistent hyperinsulinemic hypoglycaemia of infancy (PHHI) exists in two forms. One corresponds to a focal pancreatic adenomatous hyperplasia (focal PHHI) and the other is characterized by a diffuse cell abnormality (diffuse PHHI). The underlying pathology and treatment strategies are different for both the types however differentiation by clinical and biochemical parameters is not feasible. No definite role of imaging to differentiate diffuse versus focal condition has been reported in the literature.


   Case Report Top


A 50-day-old pre-term female infant presented with persistent hyperinsulinemic hypoglycemia. The child was born at 35 weeks of gestation, with breathing difficulty since birth. She developed cyanosis on day 2 of life with seizures thereafter. On investigation the child was found to be hypoglycemia (blood glucose level = 16.8 mg/dl) and hyperinsulinemia with serum insulin levels of 149.70 IU/ml (n = 2.0 IU/ml). Screening for sepsis was negative. The child required intravenous glucose infusion up to 10 mg/kg/min and oral feeds fortified with glucose to maintain euglycemic state. Ga-68 DOTATATE positron emission tomography/computerized tomography (PET/CT) scan showed diffusely increased tracer uptake in the entire pancreas with no abnormal tracer uptake anywhere else in the body, suggestive of a diffuse variant of nesidioblastosis [Figure 1]. The child is currently on injection octreotide but not fit for definitive surgical management (near total pancreatectomy).
Figure 1: Ga-68 DOTATATE positron emission tomography/computed tomography (CT) (a) Maximum intensity projection (b) transaxial CT (c) transaxial fused images showing diffusely increased tracer uptake in the entire pancreas (arrow) with no abnormal tracer uptake elsewhere in the body, suggestive of a diffuse variant of nesidioblastosis

Click here to view



   Discussion Top


Laidlaw in 1938 first identified the disease and coined the term nesidioblastosis to describe the neodifferentiation of islets of Langerhans from pancreatic ductal epithelium. [1] This condition later renamed as PHHI of infancy exists in two forms. One corresponds to a focal pancreatic adenomatous hyperplasia (focal PHHI) and the other is characterized by a diffuse cell abnormality (diffuse PHHI). [2],[3],[4] These two forms could not be differentiated by clinical or biochemical data, although their underlying pathological mechanisms and the treatment remains totally different. [5] F18-fluoro-dihydroxyphenylalanine (F-DOPA) PET scan has been used to detect the hyperfunctional pancreatic islet tissue and to differentiate between focal and diffuse PHHI with a reported accuracy of 96% in diagnosing focal or diffuse disease and 100% in localizing the focal lesion. [6],[7] The principle behind the use of F-DOPA PET in PHHI is that neuroendocrine pancreatic cells have an affinity for taking up F-DOPA and decarboxylate it into dopamine through aromatic amino acid decarboxylase. [8] Similarly neuroendocrine cells of pancreas also express high affinity somatostatin receptors (SSTR). 68-Ga DOTATATE specifically binds to the SSTR type II, which are highly concentrated in the pancreatic islet cells. [9] Previously only one case report was published where Ga-68 DOTATOC PET scan was used to differentiate focal versus diffuse nesidioblastosis with limited success. [10] This is the first case where Ga-68 DOTATATE PET scan had been used to successfully differentiate focal versus diffuse nesidioblastosis and thus help in tailoring the management in the infant.

 
   References Top

1.Laidlaw GF. Nesidioblastoma, the islet tumor of the pancreas. Am J Pathol 1938;14:125-134.5.  Back to cited text no. 1
    
2.Rahier J, Sempoux C, Fournet JC, Poggi F, Brunelle F, Nihoul-Fekete C, et al. Partial or near-total pancreatectomy for persistent neonatal hyperinsulinaemic hypoglycaemia: The pathologist's role. Histopathology 1998;32:15-9.  Back to cited text no. 2
    
3.Sempoux C, Guiot Y, Lefevre A, Nihoul-Fékété C, Jaubert F, Saudubray JM, et al. Neonatal hyperinsulinemic hypoglycemia: Heterogeneity of the syndrome and keys for differential diagnosis. J Clin Endocrinol Metab 1998;83:1455-61.  Back to cited text no. 3
    
4.de Lonlay-Debeney P, Poggi-Travert F, Fournet JC, Sempoux C, Vici CD, Brunelle F, et al. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med 1999;340:1169-75.  Back to cited text no. 4
    
5.Rahier J, Guiot Y, Sempoux C. Persistent hyperinsulinaemic hypoglycaemia of infancy: A heterogeneous syndrome unrelated to nesidioblastosis. Arch Dis Child Fetal Neonatal Ed 2000;82:F108-12.  Back to cited text no. 5
    
6.Ribeiro MJ, De Lonlay P, Delzescaux T, Boddaert N, Jaubert F, Bourgeois S, et al. Characterization of hyperinsulinism in infancy assessed with PET and 18F-fluoro-L-DOPA. J Nucl Med 2005;46:560-6.  Back to cited text no. 6
    
7.Hardy OT, Hernandez-Pampaloni M, Saffer JR, Suchi M, Ruchelli E, Zhuang H, et al. Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan. J Pediatr 2007;150:140-5.  Back to cited text no. 7
    
8.Rindi G, Capella C, Solcia E. Cell biology, clinicopathological profile, and classification of gastro-enteropancreatic endocrine tumors. J Mol Med (Berl) 1998;76:413-20.  Back to cited text no. 8
    
9.Sundin A, Garske U, Orlefors H. Nuclear imaging of neuroendocrine tumors. Best Pract Res Clin Endocrinol Metab 2007;21:69-85.  Back to cited text no. 9
    
10.Dutta S, Venkataseshan S, Bal C, Rao KL, Gupta K, Bhattacharya A, et al. Novel use of somatostatin receptor scintigraphy in localization of focal congenital hyperinsulinism: Promising but fallible. J Pediatr Endocrinol Metab 2009;22:965-9.  Back to cited text no. 10
    


    Figures

  [Figure 1]



 

Top
  
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1154    
    Printed29    
    Emailed0    
    PDF Downloaded106    
    Comments [Add]    

Recommend this journal