Pancreas appears identical to lipomatous pseudohypertrophy, but with very different clinical presentation
Clinical presentation is very different, starting in childhood
• Pancreatic senescent changes
Pancreatic atrophy, but rarely clinically significant
Usually more uneven fatty replacement of pancreas
Also associated with obesity, diabetes, and steroid use
• Pancreatic focal fatty infiltration
Usually limited to head-uncinate or body-tail segments
• Shwachman-Diamond syndrome
Rare congenital disorder characterized by pancreatic insufficiency, bone marrow dysfunction, and short stature
• Retroperitoneal liposarcoma
Often confused with lipomatous pseudohypertrophy due to massive fatty hypertrophy in retroperitoneum
PATHOLOGY
• Exact etiology is unknown
• Possibly congenital, but may require inciting factor such as cirrhosis, viral infection, or abnormal metabolism
CLINICAL ISSUES
• Usually incidental finding on CT or in autopsy
• Rarely, patients require enzyme therapy due to exocrine deficiency
(Left) Axial NECT in a woman with alcoholic cirrhosis shows typical cirrhotic morphology of the liver (nodular with widened fissures) and ascites.
(Right) Axial NECT in the same patient shows diffuse enlargement and fatty replacement of the pancreas , which mimics findings seen in cystic fibrosis.
(Left) Axial T1WI C+ FS MR in the same patient shows the lipomatous pseudohypertrophy with diffuse dropout of signal throughout the body of the pancreas due to fat suppression.
(Right) Axial opposed-phase T1WI in the same patient shows high signal throughout the pseudohypertrophied pancreas .
TERMINOLOGY
Definitions
• Enlargement of pancreas due to replacement by adipose tissue with atrophy of normal exocrine parenchyma
IMAGING
General Features
• Best diagnostic clue
Diffuse enlargement and fatty replacement of pancreas
No imaging or clinical signs of cystic fibrosis
• Location
Focal (tail, body, head) or diffuse involvement (more common)
• Size
Usually 2-4x size of normal pancreas
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, which mimics findings seen in cystic fibrosis.
due to fat suppression.
.
