Amyloidoma and Plasmacytoma Presented as a Solitary Lung Nodule in a Patient of Multiple Myeloma With AL-Amyloidosis: A Case Report and Review of Literature

Abstract

Nodular amyloidoma in the lungs is a rare entity, also the occurrence of extramedullary plasmacytoma (EMP) in the lungs is rare. To have concomitant EMP and amyloidoma presented as a single lung mass is even rarer. There was only one similar case reported in the abstract form previously. Our case did not respond to many novel chemotherapy agents, suggesting that this combination of amyloidoma and plasmacytoma belonged to a poor prognosis entity, requiring different treatment modalities, such as early bone marrow transplantation or CART (chimeric antigen receptors T) therapy.

Keywords: amyloidoma; lung nodule; multiple myeloma; plasmacytoma; systemic AL-amyloidosis.

Figure 1.

PA/lateral radiograph of the chest. Demonstrates an eccentrically based circumscribed mass in the periphery of the right mid- to upper-thorax demonstrating obtuse margins with tapering superiorly and inferiorly; note the absence of air bronchograms, indicating pleural origin. There is associated destruction of the angle/body of the right fourth rib. Abbreviation: PA, posterior anterior.

Figure 2.

Initial CT scan of the chest without contrast. (A) Axial plane soft tissue window: There is a corresponding eccentrically based, circumscribed, heterogeneous, pleura-based mass in the right upper lobe with the peripheral area of low intensity, without associated calcifications, measuring 11 × 11 × 9 cm. Associated small to moderate simple pleural effusion. No suspicious mediastinal, hilar, or axillary lymphadenopathy. (B) Coronal plane bone window: There is associated expansion of the third through the fifth ribs with destruction/involved of the body and angle of the fourth rib and extension beyond the rib cage into the right hemithorax soft tissues. Abbreviation: CT, computerized tomography.

Figure 3.

CT of the chest with IV contrast. (A) Axial and (B) coronal plane soft tissue window demonstrates pleura-based mass measuring 12 × 10 × 8 cm with peripheral curvilinear calcifications and minimal to no enhancement. Small simple right pleural effusion. No pulmonary embolism. Increased compressive atelectasis of the right upper lobe. Abbreviations: CT, computerized tomography; IV, intravenous.

Figure 4.

Lung core biopsy, H&E stain: 50×. Abundant homogeneous and eosinophilic pink material consistent with amyloid with scattered atypical plasma cells. Abbreviation: H&E, hematoxylin and eosin.

Figure 5.

Lung core biopsy. Amyloid A on immunohistochemical stain. 50×. Positive for deposits of amyloid.

Figure 6.

Kidney biopsy H&E stain. The mycangium shows moderate mesangial expansion with amorphous eosinophilic material. Abbreviation: H&E, hematoxylin and eosin.

Figure 7.

Congo red stain. It shows salmon-colored areas in the glomeruli.

Figure 8.

Immunofluorescence stain of kidney biopsy. There is homogeneous mesangial, capillary loops, and interstitial staining for kappa.

Figure 9.

Bone marrow biopsy, H&E stain, 50×. Plasma cell neoplasm. Monoclonal IgG kappa plasma cells by flow cytometry. Bone marrow biopsy showed predominantly atypical plasma cells, in the range of 20% to 30% with an average of 25%, suggestive of plasma cell neoplasm with patchy interstitial deposition of eosinophilic amorphous material, confirmed by Congo red stain as positive for amyloidosis which on immunohistochemistry showed positive staining for amyloid A amyloidosis. Liquid chromatography-tandem mass spectrometry (LCMS/MS) on bone marrow tested positive for AL-amyloid. Abbreviations: H&E, hematoxylin and eosin; IgG, immunoglobulin G; AL, amyloid light.

Figure 10.

Clinical graph depicting the levels of NT-proBNP (N-terminal pro-brain natriuretic peptide), troponin-I levels, 24-hour urine total protein. The patient was diagnosed in December with MM and AL-amyloidosis. CyBorD regimen was started in January. NT-proBNP, troponin-I, and 24-hour total protein progressions are depicted in this clinical graph over 6 months. Normal range of NT-proBNP = 0 to 125 pg/ml, troponin-I = 0 to 0.034 ng/ml, 24-hour total protein = 42 to 225 mg/24 h. The data were plotted as troponin-I levels as values × 10¹ ng/ml, 24-hour total protein as values × 10³ mg, and NT-proBNP as values × 10² pg/ml. Abbreviations: AL, amyloid light; MM, multiple myeloma; CyBorD, cyclophosphamide, bortezomib, and dexamethasone.