Hemodialysis‐associated soft tissue amyloidomas of the chest and abdominal wall

Abstract

Amyloidoma is a highly unusual presentation of amyloidosis in tumoral or nodular form. Isolated soft tissue amyloidomas in individuals with end-stage renal disease on chronic hemodialysis is exceedingly rare, particularly in the era of advanced dialysis technologies. We report the case of a 55-year-old male with end-stage renal disease due to autosomal-dominant polycystic kidney disease, on HD for over 30 years, who was found to have soft-tissue, dialysis-related (β 2 -microglobulin) amyloidomas (DRA). He presented with painful, palpable masses within the thoracic and abdominal walls. Serum β 2 -microglobulin level was only mildly elevated at 24.9 mg/L. Biopsy confirmed amyloidosis with positivity for Congo Red staining and apple-green birefringence under polarized light. Amyloid subtyping with immunohistochemistry showed positive β 2 -microglobulin staining within the deposits. Conservative therapy involving pain management and close monitoring resulted in eventual improvement in symptoms and thus proved to be a viable option for treatment.A 55-year-old African American male presented to the hospital with complaints of left-sided lower abdominal pain and fullness for 1 week. The patient had a history of

Amyloid Arthropathy: A Review

“…The most common findings, occurring in 71% of patients, include progressive joint space widening related to synovial membrane thickening, and poorly defined erosions. Plain radiographs of affected joints are useful in demonstrating complications such as subarticular cysts, osteoporosis, and pathologic fractures in relation to scalloped erosions 89,90. Computed tomography and magnetic resonance imaging are helpful in determining the extent and location of articular amyloid deposits.…”

“…Computed tomography and magnetic resonance imaging are helpful in determining the extent and location of articular amyloid deposits. Amyloid synovium usually has low signal intensity on T1 and increased signal intensity on T2 weighted images, and variably enhance with gadolinium 24,90,91. Amyloid has high affinity for calcium and is seen as hyperactivity of the synovium on technetium 99m and fluorodeoxyglucose-positron emission tomography/computed tomography scans 92.…”

“…Plain radiographs of affected joints are useful in demonstrating complications such as subarticular cysts, osteoporosis, and pathologic fractures in relation to scalloped erosions. 89,90 Computed tomography and magnetic resonance imaging are helpful in determining the extent and location of articular amyloid deposits. Amyloid synovium usually has low signal intensity on T1 and increased signal intensity on T2 weighted images, and variably enhance with gadolinium.…”

“…Amyloid synovium usually has low signal intensity on T1 and increased signal intensity on T2 weighted images, and variably enhance with gadolinium. 24,90,91 Amyloid has high affinity for calcium and is seen as hyperactivity of the synovium on technetium 99m and fluorodeoxyglucose-positron emission tomography/computed tomography scans. 92 The differential diagnoses on imaging includes gout, giant cell tenosynovial tumors, and hemophilia.…”