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Letters to the Editor| Volume 29, ISSUE 11, P1771, November 2022

Diffuse Involvement of Pancreas Should Raise Suspicion of Mahvash Disease, an Autosomal Recessive Pancreatic Neuroendocrine Tumor Syndrome

Published:September 05, 2022DOI:https://doi.org/10.1016/j.acra.2022.08.003
      I read with great interest the excellent article by He et al., which reported pancreatic disease entities other than autoimmune pancreatitis in patients with diffuse involvement of pancreas shown on CT (
      • He M
      • Wang X
      • Xu J
      • et al.
      Diffuse involvement of pancreas is not always autoimmune pancreatitis.
      ). In particular, He et al. reported that 4.5% of cases of diffuse involvement of pancreas (11/245) are due to pancreatic neuroendocrine tumor. I wish to complement the article by pointing out that Mahvash disease, an autosomal recessive pancreatic neuroendocrine tumor syndrome, clearly exhibits diffuse involvement of pancreas on imaging (
      • Zhou C
      • Dhall D
      • Nissen NN
      • et al.
      Homozygous P86S mutation of the human glucagon receptor is associated with hyperglucagonemia, alpha cell hyperplasia, and islet cell tumor.
      ,
      • Yu R.
      Mahvash Disease: 10 years after discovery.
      ) and could be the underlying specific diagnosis of at least a few of the 11 cases of pancreatic neuroendocrine tumor with diffuse involvement of pancreas reported by He et al. (
      • He M
      • Wang X
      • Xu J
      • et al.
      Diffuse involvement of pancreas is not always autoimmune pancreatitis.
      ).
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      REFERENCES

        • He M
        • Wang X
        • Xu J
        • et al.
        Diffuse involvement of pancreas is not always autoimmune pancreatitis.
        Acad Radiol. 2022; 9 (MarS1076-6332(22)00063-0. Epub ahead of print)
        • Zhou C
        • Dhall D
        • Nissen NN
        • et al.
        Homozygous P86S mutation of the human glucagon receptor is associated with hyperglucagonemia, alpha cell hyperplasia, and islet cell tumor.
        Pancreas. 2009; 38: 941-946
        • Yu R.
        Mahvash Disease: 10 years after discovery.
        Pancreas. 2018; 47: 511-515
        • Gild ML
        • Tsang V
        • Samra J
        • et al.
        Hypercalcemia in glucagon cell hyperplasia and neoplasia (Mahvash Syndrome): a new association.
        J Clin Endocrinol Metab. 2018; 103: 3119-3123
        • Singh R
        • Calhoun S
        • Shin M
        • et al.
        Pancreatic neuroendocrine tumor with atypical radiologic presentation.
        Radiol Case Rep. 2015; 3: 162
        • Naringrekar H
        • Vogel A
        • Prestipino A
        • et al.
        Imitators of chronic pancreatitis: diffuse neuroendocrine tumour of the pancreas.
        BJR Case Rep. 2017; 320170015
        • Bhargava P
        • Haque K
        • Yang Z
        • et al.
        Diffuse pancreatic neuroendocrine tumor: a rare presentation.
        Indian J Nucl Med. 2018; 33: 364-365
        • Salahshour F
        • Taslimi R
        • moosavi N
        • et al.
        Pancreatic neuroendocrine tumor presenting as a diffuse pancreatic enlargement, case report and review of literature.
        Radiology Case. 2021; 15: 11-20