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©2013 MFMER | slide-1
Case of the Day – Chest Darin White MDCM FRCPC Department of Radiology, Mayo Clinic 76th Annual Scientific Meeting Canadian Association of Radiologists Montreal, QC April 26, 2013
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Disclosures • Relevant Financial Disclosures • None
• Off Label Use Disclosures • None
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History • 46 year-old male • Progressive dyspnea on exertion • New small amounts of hemoptysis for 2 months
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Differential Diagnosis – Unilateral Pulmonary Interstitial Pattern • ACUTE
• Infection • Edema • Aspiration • Resolving hemorrhage
• Airspace opacity superimposed on already abnormal lung (i.e. emphysema, fibrosis, etc.)
• CHRONIC • Recurrent aspiration/
infection • Radiation fibrosis • Lymphangitic
carcinomatosis • Pulmonary venous
obstruction • Unilateral lung
transplant • Other rare
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Differential Diagnosis • NEOPLASTIC
• Bronchogenic carcinoma
• Metastatic disease • Lymphoma
• NON-NEOPLASTIC • Fibrosing mediastinitis • IgG4-related disease
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Subsequent Evaluation • 18F-FDG PET/CT
• Mildly increased metabolic activity in subcarinal and para-aortic lymph nodes (SUVmax = 3.6)
• Low level 18F-FDG activity in region of left hilar soft tissue thickening
• EBUS-guided Biopsy • Negative for malignancy
• Serology • Histoplasma capsulatum
antibody positive
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Answer
Fibrosing Mediastinitis
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Fibrosing Mediastinitis • Also referred to as mediastinal fibrosis or
sclerosing mediastinitis
• Rare benign proliferation of fibrous tissue within the mediastinum • Pathologically, a spectrum from loose fibromyxoid
tissue to dense collagen with a variable inflammatory cellular infiltrate
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Fibrosing Mediastinitis – Etiology • Granulomatous infection
• Histoplasma capsulatum • Tuberculosis, actinomycosis,
blastomycosis, aspergillosis…
• Non-infectious granulomatous disease • Sarcoidosis, silicosis, Behcet’s
• Immunologic/Autoimmune • Collagen vascular disease, IgG4-
related disease, multisystem fibrosis
• Malignancy
• Iatrogenic • methysergide, radiation
• Trauma
• Idiopathic
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Fibrosing Mediastinitis – Presentation • Variable age at presentation • ♂=♀ • Signs and symptoms related to compression or
obstruction of mediastinal or hilar structures • Cough, dyspnea, recurrent infection, chest pain, hemoptysis,
superior vena cava syndrome, dysphagia, hoarseness, diaphragmatic paralysis, Horner syndrome, chylothorax…
• Constitutional symptoms • Fever, weight loss, malaise
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Fibrosing Mediastinitis – Imaging • Chest Radiographs
• mediastinal widening • distortion of mediastinal lines
and stripes • hilar enlargement • +/- calcification • Sequelae:
• airway narrowing, post-obstructive atelectasis/consolidation
• pulmonary venous hypertension, pulmonary edema
• vascular asymmetry, pulmonary infarcts
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Fibrosing Mediastinitis – Imaging • CT
• infiltrative mediastinal/hilar soft tissue thickening or mass
• focal (82%) or diffuse (18%) • calcification (60-90%) • variable enhancement • Sequelae
• compression/obstruction of central airways, central vessels, esophagus
• atelectasis, consolidation, edema, infarcts
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Fibrosing Mediastinitis – Imaging • MRI
• typically intermediate T1 and heterogeneous T2 signal intensity
• often regions of T2 hypointensity
• inferior to CT for detection of calcification
• heterogeneous enhancement post-gadolinium
• Sequelae • as demonstrated on CT
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Fibrosing Mediastinitis – Imaging • Nuclear Medicine • 18-FDG PET
• variable metabolic activity within and between lesions
• Ventilation/Perfusion • perfusion defects reflecting
pulmonary arterial or venous obstruction
• lobar/segmental ventilation defects or air trapping reflecting airway involvement
Ventilation Perfusion
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Summary • Fibrosing mediastinitis should be considered in
the setting of localized mediastinal soft tissue thickening associated with vascular and/or airway compromise. • Accompanying calcification further suggests the
diagnosis. • In the correct clinical setting, such as positive
Histoplasma capsulatum serology and no known malignancy, CT findings may obviate the need for biopsy.
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Questions?
white.darin@mayo.edu
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Review • At the conclusion of this session, participants should
be able to: 1. Provide a differential diagnosis for a unilateral pulmonary
interstitial pattern. (CanMEDS Roles: Medial Expert) 2. Describe the typical imaging features and differential
diagnosis of fibrosing mediastinitis. (CanMEDS Roles: Medical Expert)
3. List at least four causes of fibrosing mediastinitis. (CanMEDS Roles: Medical Expert)
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References • McNeeley MF, Chung JH, Bhalla S, Godwin JD. Imaging of
granulomatous fibrosing mediastinitis. AJR. 2012; 199(2):319-327.
• Rossi SE, McAdams HP,Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis. Radiographics. 2001; 21(3):737-757.
• Franquet T. Mediastinitis. In: Muller NL, Silva CIS, eds. Imaging of the Chest. Philadelphia, PA: Saunders Elsevier; 2008: 1457-1472.
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