Korean J Radiol.  2012 Feb;13(1):94-97. 10.3348/kjr.2012.13.1.94.

Pancreatic Islet Cell Amyloidosis Manifesting as a Large Pancreas

• Onur MR
• Yalniz M
• Poyraz AK
• Ozercan IH
• Ozkan Y

• KMID: 1058799
• DOI: http://doi.org/10.3348/kjr.2012.13.1.94

Abstract

A 39-year-old female patient presented to our hospital with epigastric pain lasting for two months. Laboratory results showed impaired glucose tolerance. Ultrasonography of the patient showed a hypoechoic, diffusely enlarged pancreas. CT revealed a large pancreas, with multiple calcifications. On MRI, a diffusely enlarged pancreas was seen hypointense on both T1- and T2-weighted images with heterogeneous enhancement after gadolinium administration. A biopsy of the pancreas revealed primary amyloidosis of islet cells. Decreased signal on T1-weighted images without inflammation findings on CT and MRI were clues for the diagnosis.

Keyword

Pancreas; Amyloidosis; Ultrasonography; Computed tomography; Magnetic resonance imaging

MeSH Terms

Adult
Amyloidosis/*diagnosis
Contrast Media/diagnostic use
Diagnosis, Differential
*Diagnostic Imaging
Female
Glucose Tolerance Test
Humans
Islets of Langerhans/*pathology
Pancreatic Diseases/*diagnosis

Figure

• 

  Fig. 1 Imaging findings and histopathology of pancreatic amyloidosis. A. Ultrasonography shows diffuse hypoechoic, enlarged pancreas including multiple millimetric calcifications with posterior acoustic enhancement (arrow). There is no pancreatic duct dilatation. B. Axial CT image shows diffuse prominent enlargement of pancreas (arrow), with multiple hyperdense calcifications (thin arrow). C. Axial CT after contrast enhancement on venous phase shows diffuse contrast enhancement of pancreas parenchyma with absence of inflammatory changes in peripancreratic tissues. D. Normal high signal intensity of pancreas is decreased on axial T1-weighted image. Pancreas is seen diffusely hypointense (arrow). E. Axial T2-weighted image of abdomen shows diffusely enlarged pancreas (arrow), with hyperintense cystic components (thin arrow). Pancreas tail is seen more hyperintense than body with no inflammation. Also, pancreatic duct is not dilated. F. Histopathologic examination reveals amyloid deposition stained with congo red islet cells of Langerhans (arrows).

Reference

1. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997. 337:898–909.

2. Georgiades CS, Neyman EG, Barish MA, Fishman EK. Amyloidosis: review and CT manifestations. Radiographics. 2004. 24:405–416.

3. Urban BA, Fishman EK, Goldman SM, Scott WW Jr, Jones B, Humphrey RL, et al. CT evaluation of amyloidosis: spectrum of disease. Radiographics. 1993. 13:1295–1308.

4. Ozdemir D, Dagdelen S, Erbas T. Endocrine involvement in systemic amyloidosis. Endocr Pract. 2010. 16:1056–1063.

5. Garcia RL. Verrucose areolar hyperpigmentation of pregnancy. Arch Dermatol. 1973. 107:774.

6. Kennan NM, Evans C. Case report: hepatic and splenic calcification due to amyloid. Clin Radiol. 1991. 44:60–61.

7. Sainani N, Catalano O, Sahani D. Haaga JR, Dogra VS, Forsting M, Gilkeson RC, Ha HK, Sundaram M, editors. Pancreas. CT and MRI of the whole body. 2009. . Philadelphia: Mosby Elsevier;1599–1675.

8. Rafal RB, Jennis R, Kosovsky PA, Markisz JA. MRI of primary amyloidosis. Gastrointest Radiol. 1990. 15:199–201.

9. De Backer AI, Mortele KJ, Bomans P, De Keulenaer BL, Vanschoubroeck IJ, Kockx MM. Tuberculosis of the pancreas: MRI features. AJR Am J Roentgenol. 2005. 184:50–54.

10. Hoppener JW, Nieuwenhuis MG, Vroom TM, Ahren B, Lips CJ. Role of islet amyloid in type 2 diabetes mellitus: consequence or cause? Mol Cell Endocrinol. 2002. 197:205–212.