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Amyloidosis: Review and CT Manifestations¹

¹ From the Department of Radiology and Radiological Sciences, Johns Hopkins Hospital, 601 N Caroline St, Baltimore, MD 21287 (C.S.G., E.G.N., E.K.F.); and the Department of Radiology, Brigham and Women’s Hospital, Boston, Mass (M.A.B.). Presented as an education exhibit at the 2002 RSNA scientific assembly. Received April 23, 2003; revision requested May 19 and received July 8; accepted July 11. All authors have no financial relationships to disclose. Address correspondence to E.K.F. (e-mail: efishman@jhmi.edu).

View larger version (152K): [in a new window]   Figure 1a. Biopsy specimens from the tongue of a patient with known primary systemic amyloidosis and macroglossia. (a) Photomicrograph (hematoxylin-eosin stain) shows that the intrinsic musculature of the tongue is infiltrated by collagen-type extracellular material that stains pink. (b) Photomicrograph (Congo red stain) shows that the same areas as in a have strong affinity for the stain. (c) Photomicrograph obtained with polarized light microscopy of the same specimen as in b shows apple-green birefringence (arrow), which is diagnostic of amyloid.

View larger version (153K): [in a new window]   Figure 1b. Biopsy specimens from the tongue of a patient with known primary systemic amyloidosis and macroglossia. (a) Photomicrograph (hematoxylin-eosin stain) shows that the intrinsic musculature of the tongue is infiltrated by collagen-type extracellular material that stains pink. (b) Photomicrograph (Congo red stain) shows that the same areas as in a have strong affinity for the stain. (c) Photomicrograph obtained with polarized light microscopy of the same specimen as in b shows apple-green birefringence (arrow), which is diagnostic of amyloid.

View larger version (143K): [in a new window]   Figure 1c. Biopsy specimens from the tongue of a patient with known primary systemic amyloidosis and macroglossia. (a) Photomicrograph (hematoxylin-eosin stain) shows that the intrinsic musculature of the tongue is infiltrated by collagen-type extracellular material that stains pink. (b) Photomicrograph (Congo red stain) shows that the same areas as in a have strong affinity for the stain. (c) Photomicrograph obtained with polarized light microscopy of the same specimen as in b shows apple-green birefringence (arrow), which is diagnostic of amyloid.

View larger version (105K): [in a new window]   Figure 2. Primary amyloidosis in a 75-year-old man with end-stage renal disease and autonomic neuropathy who presented with hematemesis and esophagitis. Axial computed tomographic (CT) image obtained after oral administration of contrast material shows circumferential thickening of the esophageal wall (arrow). Endoscopic biopsy showed diffuse involvement by amyloid.

View larger version (107K): [in a new window]   Figure 3. Primary systemic amyloidosis in a 65-year-old woman with new-onset explosive diarrhea in addition to chronic low-grade abdominal pain. Axial CT image enhanced with intravenously administered contrast material shows diffusely thickened loops of small intestine. The differential diagnosis included infectious enteritis, ischemia, and an infiltrating process. Endoscopic biopsy demonstrated amyloid deposition.

View larger version (121K): [in a new window]   Figure 4. Amyloidosis in a 56-year-old woman with acute onset of peritoneal signs. Axial CT image shows dilated loops of small intestine with a fluid-fluid level (arrow), which is compatible with hemorrhage. Biopsy showed amyloid infiltration of the small intestine and colonic wall.

View larger version (111K): [in a new window]   Figure 5. Amyloidosis in a 58-year-old man with nonspecific plasma cell dyscrasia who presented with acute-onset abdominal pain, peritoneal signs, and unstable vital signs. Axial CT image shows heterogeneous attenuation in an enlarged spleen and perihepatic fluid. The patient was eventually taken to the operating room for treatment of a ruptured spleen but died soon afterward. The patient’s condition was complicated by colonic ischemia. Pathologic analysis revealed diffuse amyloid infiltration of the spleen, colon, pancreas, adrenal glands, testicles, and vessel walls.

View larger version (158K): [in a new window]   Figure 6. Amyloidosis in a 61-year-old woman with an enlarged liver at physical examination. Axial contrast material-enhanced CT image shows a diffusely hypoattenuating and enlarged liver. Biopsy showed diffuse involvement by amyloid.

View larger version (120K): [in a new window]   Figure 7. Amyloidosis in a 65-year-old man with nonspecific pain in the right upper abdomen. Sagittal ultrasonographic (US) image shows multiple foci of increased echogenicity (arrow). Biopsy showed amyloid infiltration of the peribiliary regions of the liver.

View larger version (106K): [in a new window]   Figure 8. Amyloidosis in a 65-year-old man with worsening chronic pain in the right upper abdomen but no peritoneal signs. Sagittal US image of the gallbladder shows diffuse wall thickening. Pathologic examination after resection showed diffuse amyloid deposition.

View larger version (116K): [in a new window]   Figure 9. Known primary amyloidosis in a 51-year-old man who presented with slowly progressive dysphagia. Sagittal T1-weighted magnetic resonance (MR) image shows an enlarged, fleshy tongue. Biopsy showed diffuse amyloid infiltration.

View larger version (149K): [in a new window]   Figure 10. Amyloidosis in a 65-year-old woman with congestive heart failure. Axial contrast-enhanced CT image shows diffuse thickening of the left ventricular wall. Despite this finding, the patient had a decreased ejection fraction. Cardiac biopsy demonstrated amyloid infiltration.

View larger version (130K): [in a new window]   Figure 11. Amyloidosis in a 68-year-old woman with shortness of breath. Axial CT image of the chest (pulmonary window) shows a budding tree appearance, which is most notable in the right middle lobe (open arrow), and a focal ground-glass infiltrate in the peripheral left lower lobe (solid arrow). Biopsy demonstrated amyloid infiltration.

View larger version (127K): [in a new window]   Figure 12. Amyloidosis in a 55-year-old man with worsening chronic shortness of breath. Axial CT image (pulmonary window) shows bilateral ground-glass infiltrates and pleural effusions. Biopsy showed interstitial thickening due to amyloid infiltration.

View larger version (136K): [in a new window]   Figure 13. Amyloidosis in a 61-year-old man with recurrent episodes of pneumonia despite antibiotic treatment. Axial CT image (pulmonary window) shows a pleura-based mass lesion in the left lower lobe (open arrow) and multiple nodules in the right lower lobe (solid arrow).

View larger version (158K): [in a new window]   Figure 14. Amyloidosis in a 62-year-old woman with mild chronic shortness of breath. Midsagittal reformatted CT image shows a diffusely calcified tracheal wall. Endoscopic biopsy demonstrated amyloid infiltration.

View larger version (108K): [in a new window]   Figure 15a. Primary systemic amyloidosis in a 59-year-old woman with worsening stridor. Axial (a) and midsagittal reformatted (b) CT images show a mucosal polypoid lesion in the upper trachea (arrow). An amyloidoma was diagnosed at biopsy, and the patient’s symptoms improved after removal of the lesion.

View larger version (124K): [in a new window]   Figure 15b. Primary systemic amyloidosis in a 59-year-old woman with worsening stridor. Axial (a) and midsagittal reformatted (b) CT images show a mucosal polypoid lesion in the upper trachea (arrow). An amyloidoma was diagnosed at biopsy, and the patient’s symptoms improved after removal of the lesion.

View larger version (130K): [in a new window]   Figure 16. Amyloidoma in a 58-year-old man with chest wall pain and a palpable mass. Axial CT image of the chest shows a partially calcified, lobulated mass that originates in the left chest wall and destroys the involved rib. Excision biopsy showed that the mass was an amyloidoma.

View larger version (121K): [in a new window]   Figures 17. Amyloidoma in a 72-year-old woman with primary amyloidosis who presented with chest and back pain. Axial CT image shows a posterior mediastinal mass, which is mostly on the left side of the spine. Biopsy showed that the mass was an amyloidoma.

View larger version (104K): [in a new window]   Figure 18. Amyloidomas in a 52-year-old woman with palpable breast nodules. Axial CT image shows nodules of soft-tissue attenuation in the left breast (arrow), some of which are calcified. Biopsy results were diagnostic of amyloidomas.

View larger version (121K): [in a new window]   Figure 19. Primary amyloidosis in a 62-year-old woman with right flank pain. Axial CT image shows a small cortical mass with focal calcifications in the right kidney (arrow). Biopsy demonstrated amyloid infiltration.

View larger version (123K): [in a new window]   Figure 20. Amyloidosis in a 59-year-old woman with slowly progressive renal insufficiency. Sagittal US image shows diffuse increased echogenicity in the renal cortex with a renal size at the top limit of normal. Biopsy results were diagnostic of amyloidosis.

View larger version (83K): [in a new window]   Figure 21. Known primary amyloidosis in a 48-year-old woman with bilateral hip pain. Axial CT image (bone window) shows multiple erosions of the femoral heads. Synovial biopsy showed amyloid infiltration.

View larger version (106K): [in a new window]   Figures 22. Coronal T2-weighted MR image of the knees shows diffuse synovial thickening and increased signal intensity. Synovial biopsy showed amyloid infiltration.

View larger version (134K): [in a new window]   Figure 23. Amyloidosis in a 65-year-old woman with vague chronic abdominal pain. Axial CT image shows multiple focal calcifications in the mesentery, right retroperitoneal space, and left perinephric space.

View larger version (142K): [in a new window]   Figure 24. Axial CT image shows a large retroperitoneal mass surrounding the abdominal aorta, superior mesenteric artery, and renal arteries. Percutaneous needle biopsy demonstrated amyloid infiltration.

View larger version (147K): [in a new window]   Figure 25a. Amyloidoma in a 52-year-old man with multiple myeloma. (a) Axial CT image shows an incidentally found small nodule of soft-tissue attenuation in the left retroperitoneum (arrow). Biopsy results were diagnostic of an amyloidoma. (b) Axial CT image obtained approximately 1 year later shows that the mass is totally calcified.

View larger version (145K): [in a new window]   Figure 25b. Amyloidoma in a 52-year-old man with multiple myeloma. (a) Axial CT image shows an incidentally found small nodule of soft-tissue attenuation in the left retroperitoneum (arrow). Biopsy results were diagnostic of an amyloidoma. (b) Axial CT image obtained approximately 1 year later shows that the mass is totally calcified.

View larger version (141K): [in a new window]   Figure 26a. (a) Axial CT image shows nodular thickening of the cruciform ligament (arrow). (b) Sagittal T1-weighted MR image shows concentric thickening of the ligaments surrounding the spinal cord at the level of C1-2 (arrows).

View larger version (120K): [in a new window]   Figure 26b. (a) Axial CT image shows nodular thickening of the cruciform ligament (arrow). (b) Sagittal T1-weighted MR image shows concentric thickening of the ligaments surrounding the spinal cord at the level of C1-2 (arrows).

View larger version (143K): [in a new window]   Figure 27a. Primary amyloidosis in a 45-year-old man with enlarged, nontender parotid glands. (a) Axial contrast-enhanced CT image shows bilateral diffuse enlargement of the parotid glands. Narrowing of the nasopharynx is also seen. Biopsy showed diffuse amyloid infiltration. (b) Axial CT image obtained caudad to a shows bilateral enlargement of the submandibular and jugular lymph nodes. A second biopsy also demonstrated amyloid infiltration with no evidence of malignancy or infection.

View larger version (134K): [in a new window]   Figure 27b. Primary amyloidosis in a 45-year-old man with enlarged, nontender parotid glands. (a) Axial contrast-enhanced CT image shows bilateral diffuse enlargement of the parotid glands. Narrowing of the nasopharynx is also seen. Biopsy showed diffuse amyloid infiltration. (b) Axial CT image obtained caudad to a shows bilateral enlargement of the submandibular and jugular lymph nodes. A second biopsy also demonstrated amyloid infiltration with no evidence of malignancy or infection.