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淀粉样变性影像学表现 Douglas Krakower Gillian Lieberman, MD Amyloidosis: Imaging a Zebra Douglas Krakower Harvard Medical School Year III Gillian Lieberman, MD Douglas Krakower Gillian Lieberman, MD July 2002 2 Douglas Krakower Gillian Lieberman, MD Goals of this Presen...

淀粉样变性影像学表现
Douglas Krakower Gillian Lieberman, MD Amyloidosis: Imaging a Zebra Douglas Krakower Harvard Medical School Year III Gillian Lieberman, MD Douglas Krakower Gillian Lieberman, MD July 2002 2 Douglas Krakower Gillian Lieberman, MD Goals of this Presentation • Outline a patient work-up that leads to a diagnosis of amyloidosis • Define amyloidosis • Outline basic facts about the epidemiology, classification, etiology, and prognosis of amyloidosis • Illustrate briefly two other presentations of amyloidosis 3 Douglas Krakower Gillian Lieberman, MD Initial Presentation of Our Patient: “Mr. M” • Mr. M → 52-year-old man • History → HTN, hypercholesterolemia, s/p cholecystectomy • Presentation → abdominal pain x 4 hours radiating to his back • DDx: – Pancreatitis – Abdominal aortic aneurysm – Perforation 4 Douglas Krakower Gillian Lieberman, MD • The work-up of Mr. M’s presentation… 5 Douglas Krakower Gillian Lieberman, MD Step 1 in Work Up: Radiographs and Labs • Abdominal supine and erect radiographs → within normal limits • Labs → within normal limits, incl. normal amylase/lipase • Step 1 tests are inconclusive ∴ Step 2 → Abdominal CT 6 Douglas Krakower Gillian Lieberman, MD Step 2 in Work Up: Abdominal CT …Reveals Finding #1 Finding #1 •3.5 x 4.9 cm rounded soft tissue mass located in the inferior portion of the porta hepatis •Extends into caudate lobe of liver BIDMC-PACS 7 Douglas Krakower Gillian Lieberman, MD Abdominal CT …Slice at Level of Paragastric and Paracaval Lymph Nodes Reveals Finding #2 Finding #2 •Paragastric lymphadenopathy •Paracaval lymphadenopathy …other slices reveal lymphadenopathy in the celiac nodes as well BIDMC-PACS 8 Douglas Krakower Gillian Lieberman, MD Abdominal CT …Liver Window Reveals Finding #3 Finding #3 •Diffuse, heterogeneous, low attenuation of liver BIDMC-PACS 9 Douglas Krakower Gillian Lieberman, MD • An anatomy review will help to place Mr. M’s findings in context… 10 Douglas Krakower Gillian Lieberman, MD Anatomy of Lymph Nodes in the Region of the Porta Hepatis and the Pancreas •From Netter, FH. The Ciba Collection of Medical Illustrations/prepared by Frank H. Netter. West Caldwell, NJ: Ciba, 1983. Finding #1 Soft tissue mass near porta hepatis/ head of pancreas Finding #3: Diffuse, heterogeneous low attenuation of liver Finding #2 Lymphadeno- pathy of paragastric, paracaval, and celiac nodes 11 Douglas Krakower Gillian Lieberman, MD • What are the differential diagnoses for Mr. M’s findings? 12 Douglas Krakower Gillian Lieberman, MD Differential Diagnoses for Mr. M’s Findings DDx for Mass in the Region of the Pancreas on CT • Abcess • Carcinoma of liver, bile duct, pancreas • Metastasis • Lymphadenopathy DDx for Generalized Low Attenuation Lesions on Non- contrast CT • Diffuse malignancy • Fatty infiltration • Amyloidosis (uncommon) …No clear diagnosis → CT-guided biopsies 13 Douglas Krakower Gillian Lieberman, MD • As imaging studies yield no clear diagnosis for Mr. M, the work-up requires a biopsy of Mr. M’s lesions. • The biopsy is performed under CT guidance… 14 Douglas Krakower Gillian Lieberman, MD Step 3 in Work Up: Biopsy of Lesions • CT-guided biopsies (1) mass near porta hepatis (2) liver tissue in area of low attenuation • Congo red stain → Green bi-refringence ∴ Dx → AMYLOIDOSIS 15 Douglas Krakower Gillian Lieberman, MD • The pathological diagnosis is amyloidosis. • Here is an example of the histology of amyloidosis… 16 Douglas Krakower Gillian Lieberman, MD Step 3 in Work Up: Biopsy of Lesions • Histology of amyloidosis in liver tissue • Note amyloid deposits in blood vessel walls Liver tissue:H&E stain Congo Red stain From Robbins Pathologic Basis of Disease, 6th ed, by Ramzi S Cotran, Vinay Kumar, and Tucker Collins, eds, 1425 pp. Philadelphia, Pa: WB Saunders & Co, 1998. 17 Douglas Krakower Gillian Lieberman, MD • Are there any other findings in Mr. M that support the diagnosis of amyloidosis? • Thoracic CT reveals supportive findings… 18 Douglas Krakower Gillian Lieberman, MD Support of Diagnosis in Mr. M: CT and Pathology Findings in Thorax •Mediastinal and hilar lymphadenopathy •Ultrasound-guided biopsy of lymph nodes → amyloid deposits BIDMC-PACS 19 Douglas Krakower Gillian Lieberman, MD • Mr. M has amyloidosis. • What is amyloidosis? 20 Douglas Krakower Gillian Lieberman, MD Amyloidosis: Definition • Not a single disease entity • Term for diseases that lead to extracellular deposition of insoluble fibrillar proteins in tissues • Fibrills are all beta-pleated sheets 21 Douglas Krakower Gillian Lieberman, MD Amyloidosis: Most Common Presentations • Heart → CHF • Kidneys → Nephrotic syndrome • Liver → Hepatomegaly 22 Douglas Krakower Gillian Lieberman, MD Amyloidosis: Incidence and Classification • Incidence: 1275-3200 patients/year in USA Subtype Underlying Disease Process Prognosis (Median survival after diagnosis) Primary amyloidosis Plasma cell dyscrasias 1-2 yrs Secondary amyloidosis Chronic inflammation Variable Familial amyloidosis Mutated proteins Up to 15 yrs Adapted from Falk RH, Comenzo, RL, Skinner, M. The Sustemic Amyloidoses. N Engl J Med 1997; 337: 898-909. 23 Douglas Krakower Gillian Lieberman, MD • Let’s look at another patient’s presentation of amyloidosis… 24 Douglas Krakower Gillian Lieberman, MD Another Presentation of Amyloidosis: Patient #2 • Patient #2 → 19-year-old man with a 9 year history of progressive wheezing • Chest radiographs were obtained 25 Douglas Krakower Gillian Lieberman, MD Patient #2 → PA Chest Radiograph • Region of increased radio-opacity in tracheal lumen suggestive of a mass •Left tracheal deviation Note increased radio- opacity in tracheal lumen Courtesy of Dr. Phillip Boiselle, BIDMC 26 Douglas Krakower Gillian Lieberman, MD • What is the differential diagnosis for an intratracheal mass as seen in Patient #2? 27 Douglas Krakower Gillian Lieberman, MD Patient #2 → Differential Diagnosis • DDx of Intratracheal Mass – Adenoma – Neoplasm, primary or secondary – Amyloidosis – Hamartochondroma – Lymphoma – Many more… • Next step in work up →MRI 28 Douglas Krakower Gillian Lieberman, MD • An MRI is needed to characterize further the intratracheal mass… 29 Douglas Krakower Gillian Lieberman, MD MRI of Tracheobronchial Amyloidosis •Coronal view of trachea •Exophytic mass •Biopsy → amyloid Courtesy of Dr. Phillip Boiselle, BIDMC 30 Douglas Krakower Gillian Lieberman, MD • Biopsy of the intratracheal mass revealed amyloid deposits, solidifying a diagnosis of amyloidosis for Patient #2. • Let’s look briefly at one more way that amyloidosis can present… 31 Douglas Krakower Gillian Lieberman, MD Yet Another Presentation of Amyloidosis : Small Bowel Amyloidosis •Barium study of small bowel •Nodular, irregular thickening of bowel folds •Biopsy → amyloid Courtesy of Dr. Jonathan Kruskal, BIDMC 32 Douglas Krakower Gillian Lieberman, MD Take-Home Message • Amyloidosis has a protean presentation • Can affect almost any organ • Uncommon disease that commonly appears on DDx for imaging findings…Even though it is a “zebra” diagnosis, it should be considered! • If high clinical suspicion → biopsy to confirm 33 Douglas Krakower Gillian Lieberman, MD References • Carlson, HC, Breen, JF. Amyloidosis and Plasma Cell Dyscrasias: Gastrointestinal Involvement. Seminars in Roentgenology 1986; XXI: 128-138. • Falk RH, Comenzo, RL, Skinner, M. The Sustemic Amyloidoses. N Engl J Med 1997; 337: 898- 909. • Gross, BH, Felson, B, Birnberg, FA. The Respiratory Tract in Amyloidosis and the Plasma Cell Dyscrasias. Seminars in Roentgenology 1986; XXI: 113-127. • Netter, FH. The Ciba Collection of Medical Illustrations/prepared by Frank H. Netter. West Caldwell, NJ: Ciba, 1983. • Pear, BL. Other Organs and Other Amyloids. Seminars in Roentgenology 1986; XXI: 150-164. • Reeder, MM, Bradley, WG Jr. Reeder and Felson’s Gamuts in Radiology: Comprehensive Lists of Roentgen Differential Diagnosis, 3rd Edition. New York: Springer-Verlag, 1998. • Robbins Pathologic Basis of Disease, 6th ed, by Ramzi S Cotran, Vinay Kumar, and Tucker Collins, eds, 1425 pp. Philadelphia, Pa: WB Saunders & Co, 1998. • Scott, PP, Scott, WW Jr., Siegelman, SS. Amyloidosis: An Overview. Seminars in Roentgenology 1986; XXI: 103-112. • Yamada, CY. Radiology-Pathology Correlation Conference, Massachusetts General Hospital. Jan 2, 1996. ACR 671.68. References 34 Douglas Krakower Gillian Lieberman, MD Acknowledgements • Dr. Jonathan Kruskal • Dr. Phillip Boiselle • Dr. Dan Saurborn • Dr. Gillian Lieberman • Pamela Lepkowski • Larry Barbaras and Cara Lyn D’amour • The Members of BIDMC Radiology Core Clerkship, July 2002 Amyloidosis: Imaging a Zebra Goals of this Presentation Initial Presentation of Our Patient: “Mr. M” Slide Number 4 Step 1 in Work Up:�Radiographs and Labs Step 2 in Work Up: Abdominal CT�…Reveals Finding #1 Abdominal CT�…Slice at Level of Paragastric and Paracaval Lymph Nodes Reveals Finding #2 Abdominal CT�…Liver Window Reveals Finding #3 Slide Number 9 Anatomy of Lymph Nodes in the Region of the Porta Hepatis and the Pancreas Slide Number 11 Differential Diagnoses for Mr. M’s Findings Slide Number 13 Step 3 in Work Up:�Biopsy of Lesions Slide Number 15 Step 3 in Work Up:�Biopsy of Lesions Slide Number 17 Support of Diagnosis in Mr. M:�CT and Pathology Findings in Thorax Slide Number 19 Amyloidosis: Definition Amyloidosis:�Most Common Presentations Amyloidosis: �Incidence and Classification Slide Number 23 Another Presentation of Amyloidosis: Patient #2 Patient #2  PA Chest Radiograph Slide Number 26 Patient #2  Differential Diagnosis Slide Number 28 MRI of Tracheobronchial Amyloidosis Slide Number 30 Yet Another Presentation of Amyloidosis : Small Bowel Amyloidosis Take-Home Message References Acknowledgements
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