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Published on: August 26, 2025
Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis
Sherief H Garrana1, Jennifer R Buckley1, Melissa L Rosado-de-Christenson1
1From the Department of Radiology, Saint Luke's Hospital of Kansas City, University of Missouri in Kansas City, 4401 Wornall Rd, Kansas City, MO 64111 (S.H.G., J.R.B., M.L.R.d.C., S.M.J., J.J.B.); and Ameripath, Kansas City, Mo (P.M.).
Insights
Fibrosing mediastinitis is a rare condition of fibrotic tissue proliferation in the mediastinum. Imaging findings are key for diagnosis, differentiating it from malignancy, and guiding treatment.
Area of Science:
- Radiology
- Pathology
- Immunology
Background:
- Fibrosing mediastinitis is a rare, benign, yet life-threatening condition characterized by fibrotic tissue proliferation within the mediastinum.
- It presents in focal and diffuse subtypes, with distinct etiologies and patient demographics.
- Both subtypes cause symptoms due to compression of vital mediastinal structures.
Purpose of the Study:
- To describe the clinical presentation, imaging features, and diagnostic considerations for fibrosing mediastinitis.
- To emphasize the importance of imaging in diagnosis, disease assessment, and treatment evaluation.
- To differentiate fibrosing mediastinitis from malignancy.
Main Methods:
- Review of clinical presentations and symptoms associated with fibrosing mediastinitis.
- Analysis of characteristic imaging features, including infiltrative mediastinal soft tissue, calcification, and compression of mediastinal structures.
- Correlation of imaging findings with clinical context for diagnosis.
Main Results:
- Focal fibrosing mediastinitis is often linked to Histoplasma capsulatum infection and affects younger individuals.
- Diffuse fibrosing mediastinitis can be idiopathic or autoimmune, typically affecting older males.
- Characteristic imaging findings include infiltrative mediastinal soft tissue causing compression or obstruction of vascular structures and the aerodigestive tract.
Conclusions:
- Characteristic imaging findings, in the appropriate clinical setting, allow for the suggestion of fibrosing mediastinitis diagnosis.
- Careful imaging assessment is crucial for excluding malignancy, monitoring disease progression, identifying complications, and evaluating treatment response.
- Individualized treatment strategies are necessary, tailored to alleviate the compression of affected mediastinal structures.
Abstract:
Fibrosing mediastinitis is a rare benign but potentially life-threatening process that occurs because of proliferation of fibrotic tissue in the mediastinum. The focal subtype is more common and typically is associated with an abnormal immunologic response to Histoplasma capsulatum infection. Affected patients are typically young at presentation, but a wide age range has been reported, without a predilection for either sex. The diffuse form may be idiopathic or associated with autoimmunity, usually affects middle-aged and/or elderly patients, and is more common in men. For both subtypes, patients present with signs and symptoms related to obstruction or compression of vital mediastinal structures. The most common presenting signs and symptoms are cough, dyspnea, recurrent pneumonia, hemoptysis, and pleuritic chest pain. Patients with the diffuse subtype may have additional extrathoracic symptoms depending on the other organ systems involved. Because symptom severity is variable, treatment should be individualized with therapies tailored to alleviate compression of the affected mediastinal structures. Characteristic imaging features of fibrosing mediastinitis include infiltrative mediastinal soft tissue (with or without calcification) with compression or obstruction of mediastinal vascular structures and/or the aerodigestive tract. When identified in the appropriate clinical setting, these characteristic features allow the radiologist to suggest the diagnosis of fibrosing mediastinitis. Careful assessment is crucial at initial and follow-up imaging for exclusion of underlying malignancy, assessment of disease progression, identification of complications, and evaluation of treatment response. Online supplemental material is available for this article. ©RSNA, 2019.
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