Multimodality imaging in amyloidosis

Abstract

Amyloidosis comprises a rare spectrum of protein deposition diseases that diffusely or focally affect any organ. Amyloid's variable clinical presentation and nonspecific disease course often cause it to evade early diagnosis. This pictorial essay aims to familiarize radiologists with the pathophysiology of amyloidosis, to describe the basic classifications of amyloidosis, and to use multimodality imaging to illustrate its varied appearance throughout the body. This review highlights the diagnostic challenge of interpreting radiographic studies in patients with hematologic malignancies and concurrent amyloidosis. Radiologists should consider amyloid in chronically ill patients or patients with hematologic malignancies who have unusual/unexpected imaging findings.

Figure 1

A 75-year-old man presenting with confusion. (A) Non-contrast axial CT image at the level of the lateral ventricles reveals large subcortical hyperdense lesions in the frontal and parietal lobes; the hyperdensity suggests hemorrhage. Hemorrhage layers in the posterior horn of the left lateral ventricle (arrow). (B) Axial T1-weighted gradient echo MR sequence confirms intralesional and intraventricular hemorrhage.

Figure 2

A 62-year-old with a history of lymphoplasmacytic lymphoma. (A) Axial T1-weighted postcontrast image reveals a large, avidly enhancing mass in the right parapharyngeal space involving the medial and lateral ptygeroid muscles and partially encasing the right internal carotid artery. (B) T2-weighted image at the same level reveals a mildly heterogeneous, predominantly hyperintense mass. (C) Axial unenhanced CT image obtained just inferiorly reveals extension of this mass across the midline. (D) Fused axial PET/CT image reveals an intensely FDG avid mass involving the right parapharyngeal space and extending across the midline (standardized uptake value (SUV)_max 20.0). Complete surgical resection of this mass revealed diffuse amyloidosis, with no lymphomatous involvement.

Figure 3

A 60-year-old with a history of lymphplasmacytic lymphoma and new onset double vision. Axial pre- (A) and post-contrast (B) T1-weighted MR images show hypointense enhancing masses (arrows), involving bilateral lacrimal glands. (C) Coronal short tau inversion recovery (STIR) image shows hyperintense lacrimal masses bilaterally. (D) Coronal fused image from an FDG-PET/CT shows intense FDG avidity in bilateral masses (SUV_max 32.3 left side, 17.5 right side). Biopsy of bilateral lacrimal glands confirmed lymphomatous involvement, intermixed with amyloid deposition.

Figure 4

A 60-year-old woman with a history of Waldenström macroglobulinemia/lymphoplasmacytic lymphoma presenting with neck swelling. (A) Chest radiograph reveals extensive calcified cervical, supraclavicular, axillary, and mediastinal lymphadenopathy. (B) Fused coronal images from a PET/CT shows little FDG avidity in the calcified lymphadenopathy (right axilla SUV_max 1.2). Biopsy of the right axilla confirmed primary amyloid infiltration, with minimal evidence of lymphoma.

Figure 5

An 80-year-old woman with no significant past medical history presenting with recurrent pneumonias/hemoptysis. Coronal (A) and high-resolution axial (B) CT images performed without intravenous contrast reveal a consolidative opacity in the basilar segments of the left lower lobe superimposed on a background of ground glass opacity and smooth interlobular septal thickening, consistent with pneumonia superimposed on diffuse alveolar septal amyloid. Left lower lobe transbronchial biopsy revealed pulmonary amyloidosis.

Figure 6

A 68-year-old man with no significant past medical history presenting with a cough. Fused axial images from a PET/CT confirm multiple pulmonary nodules with little to no FDG avidity above background (SUV_max in nodules ranged from 1.5 to 2.7). Surgical resection of a left upper lobe nodule revealed amyloid, with no evidence of malignancy.

Figure 7

A 70-year-old man with a history of arrhythmia. T1-weighted gadolinium enhanced delayed imaging of the heart in 4 chamber (A), and short-axis (B) views reveal mild left and moderate right atrial enlargement, with severe diffuse thickening of the left ventricular myocardium and diffuse subendocardial late gadolinium enhancement involving all 4 chambers, consistent with biopsy-proven cardiac amyloidosis.

Figure 8

A 73-year-old with a history of multiple myeloma and cardiac amyloid and multiple hospital admissions for heart failure. Four-chamber (A) and short-axis (B) grayscale sonographic images reveal a small left ventricle with severe concentric left ventricular hypertrophy. Atria are borderline enlarged. Ejection fraction was preserved at 70%.

Figure 9

A 59-year-old woman presenting with abdominal pain. Fluoroscopic images obtained during a small bowel follow-through reveal diffuse, somewhat nodular fold thickening involving predominately the jejunum; biopsy yielded amyloidosis.

Figure 10

A 54-year-old with a history of B-cell lymphoproliferative disorder and amyloid. Non-contrast coronal CT reveals bilateral nephromegaly, with the right kidney measuring 17.8 cm and the left kidney measuring 17.4 cm.

Figure 11

A 91-year-old man with urinary urgency and hematuria. Delayed images from a contrast-enhanced axial CT scan reveal diffuse thickening of the posteriolateral bladder wall. Subsequent cystoscopically guided biopsy of the posterior bladder wall revealed diffuse amyloid infiltration.

Figure 12

A 56-year-old woman with a history of lymphoplasmacytic lymphoma who presented with left buttock pain. Axial contrast-enhanced CT (A) revealed a non-enhancing, somewhat infiltrative soft tissue mass involving the left gluteal musculature (curved white arrow) extending through the sciatic foramen, with associated calcifications. Additionally, a non-enhancing lobular soft tissue mass (straight white arrow) was noted in the right adnexa. Black arrow points to a portion of a capacious bladder. Biopsy of the gluteal lesion revealed diffuse amyloid infiltration, with no evidence of lymphoma. Surgical resection of the right adnexa revealed both lymphplasmacytic lymphoma and amyloid. Axial proton density images from a subsequent MRI (B) revealed the gluteal mass to be hypointense (the adnexal lesion had been resected). Coronal T1-weighted fat-saturated image obtained after gadolinium administration (C) revealed no enhancement in this infiltrative lesion.

Figure 13

A 68 year-old man with a recent diagnosis of prostate cancer, found to have an incidental lesion detected on MRI. (A) T2-weighted axial MR image at the level of the pubic symphysis (with rectal coil in place posteriorly) reveals a heterogeneous, well-circumscribed extraprostatic lesion with dark rim extending from the pubic symphysis posteriorly into the pre-prostatic fat, and causing a mild mass effect on the anterior prostate. Coronal T1-weighted pre- (B), and fat-suppressed post-gadolinium images (C), reveal a hypointense peripherally enhancing lesion. Pathology at surgical resection revealed a focal amyloidoma.

Figure 14

A 72-year-old woman with a history of multiple myeloma presenting with a right thigh mass. Coronal contrast-enhanced CT image reveals a non-enhancing heterogeneous mass in the musculature of the right hip containing chunky internal calcifications. Biopsy revealed extensive amyloid deposition.