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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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