PMC4899862_01
75-year-old male with amyloidosis. Axial, contrast-enhanced CT section of the abdomen in the arterial phase shows a large, heterogenous, peritoneal lesion displacing the bowel loops centrally.
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The subsequent frontal chest radiograph demonstrated a wide mediastinum with clear lung fields (Fig. 1).
75-year-old male with amyloidosis. Contrast-enhanced CT of the thorax in soft-tissue window. A: Axial image with large and bulky lymphadenopathy in the right hilar and subcarinal regions. The adenopathy is homogeneously low in attenuation, with smooth contours and speckled internal calcifications. B: Coronal Maximum Intensity Projection (MIP) image demonstrates large adenopathy with speckled calcifications in the right hilar, subcarinal, and right paraesophageal regions.
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A contrast-enhanced CT of the chest showed a normal-sized heart without effusion and no evidence of acute aortic syndrome (Fig. 2).
75-year-old male with amyloidosis. A: Contrast-enhanced CT of the thorax in soft-tissue window obtained 10 years before Fig. 2. Axial image depicts subtle subcarinal lymphadenopathy without calcification. B: Contrast-enhanced. axial CT image of the thorax in soft-tissue window obtained 5 years before Fig. 2 shows the subcarinal mass to be enlarged with faint punctate calcifications. There is also new right hilar lymphadenopathy.
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On further review of prior CT scans at our institution, dating back approximately 10 years, the mediastinal masses were seen to be present but slowly enlarging and growing in number (Fig. 3).
75-year-old male with amyloidosis. Core biopsy of the subcarinal mass reveals the presence of amyloid. A: Hematoxylin and eosin stain shows the amorphous eosinophilic acellular material (arrows), x100. B: Apple-green birefringence under polarized light after Congo red stain (arrows), x100; consistent with amyloid.
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At this time, a mediastinal biopsy was performed of the subcarinal mass to exclude metastatic disease (Fig. 4).
75-year-old male with amyloidosis. CT of the thorax without contrast taken 5 months after Fig. 2. Axial (A) and coronal (B) soft-tissue window images show the extensive amyloidomas in the subcarinal, right hilar, and right paraesophageal regions. There is a slight increase in speckled calcifications within the amyloidomas. C: Axial image in lung window setting shows the mediastinal amyloidomas with normal pleura and pulmonary parenchyma, confirming the rare imaging manifestations of this disease.
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A CT scan of the thorax without contrast again showed the extensive mediastinal amyloidomas, with slightly progressive internal punctuate calcifications (Fig. 5).
75-year-old male with amyloidosis. Bone-marrow biopsy. A: Hematoxylin and eosin stain reveals a hypercellular marrow infiltrated with scattered plasma cells (arrows) that account for approximately 10-15% of the total cellularity, x400. B: CD 138 immunohistochemical stain shows increased plasma cells (arrow), x200. C: In situ hybridization is positive for lambda light chain (arrow), x200. D: The neoplastic plasma cells are negative for kappa light chain, x200.
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The hematoxylin and eosin-stained sections revealed a hypercellular marrow infiltrated with scattered plasma cells, which accounted for approximately 10-15% of the total cellularity (Fig. 6A). This was confirmed by CD 138 immunostain for plasma cells (Fig. 6B). The in-situ hybridization for kappa and lambda demonstrated that the plasma cells were lambda-restricted (Figs. 6C, D).