Fibrosing mediastinitis mimicking bronchogenic carcinoma

Published on Feb 1, 2013in Journal of Thoracic Disease2.046
· DOI :10.3978/J.ISSN.2072-1439.2012.07.03
Deniz Köksal11
Estimated H-index: 11
,
Hulya Bayiz6
Estimated H-index: 6
+ 5 AuthorsMine Berkoglu3
Estimated H-index: 3
Sources
Abstract
Fibrosing mediastinitis is a rare but benign disorder characterized by an excessive fibrotic reaction in the mediastinum which can result in compromise of airways, great vessels, and other mediastinal structures. In this paper we presented a patient with fibrosing mediastinitis mimicking bronchogenic carcinoma. The patient was a 32-year-old diabetic male admitting with cough and hemoptysis. There was a right hilar mass and multiple mediastinal conglomerated lymph nodes on chest computed tomography. Positron emission tomography with computed tomography (PET/CT) scan demonstrated increased fluorodeoxyglucose (FDG) uptake at the right hilar mass lesion and mediastinal lymph nodes. Fiberoptic bronchoscopy showed mucosal distortion of right upper lobe. Pathologic examination of the mucosal biopsy revealed inflammation. Endobronchial ultrasound guided transbronchial needle and cervical mediastinoscopic lymph node biopsies were undiagnostic. Diagnostic thoracotomy confirmed the diagnosis fibrosing mediastinitis. Administration of six months of systemic corticosteroid and antituberculous therapy was not beneficial. In conclusion, despite being a rare clinical entity, fibrosing mediastinitis should be kept in mind in the differential diagnosis of mediastinal mass lesions of unknown etiology. The diagnosis is exceptionally difficult in the presence of atypical radiological findings. The treatment is particularly challenging without any proven effective therapy.
📖 Papers frequently viewed together
54 Citations
7 Citations
References9
Newest
#1Koei Ikeda (Kumamoto University)H-Index: 17
#2Hiroaki Nomori (Kumamoto University)H-Index: 40
Last. Masakazu Yoshioka (Kumamoto University)H-Index: 12
view all 7 authors...
The use of steroids to successfully treat a 75-year-old woman with fibrosing mediastinitis and sclerosing cervicitis causing a stricture of the left common carotid artery is reported. Biopsy specimens showed collagenous fibers and fibroblasts with moderate infiltration of lymphocytes. The mediastinal and neck lesions were significantly reduced, with almost complete resolution of arterial stricture, 3 months after initiating administration of prednisolone at 20 mg/d.
21 CitationsSource
#1Simon Bays (BRI: Bristol Royal Infirmary)H-Index: 3
#2Chanaka Rajakaruna (BRI: Bristol Royal Infirmary)H-Index: 5
Last. Anthony Morgan (BRI: Bristol Royal Infirmary)H-Index: 5
view all 4 authors...
Fibrosing mediastinitis is a rare, chronic inflammatory process that can cause superior vena cava syndrome, and can mimic malignancy. We present two cases of this disease where surgical resection was not possible and review the treatment options. q 2004 Elsevier B.V. All rights reserved.
21 CitationsSource
#1S t DechambreH-Index: 1
#2Dorzée JH-Index: 1
Last. J.P. D’OdemontH-Index: 2
view all 6 authors...
The side-effects of radiation therapy on the bronchial tree or on the mediastinum are seldom reported. In this setting, we report a case of sclerosing mediastinitis with bronchial stenosis discovered 1 yr after external radiotherapy for lung cancer. The patient was treated with a Dumont stent and has so far had an uneventful further course for up to 42 months. Bronchial stenosis related to mediastinal fibrosis after radiotherapy has not been reported previously.
31 CitationsSource
#1T M MoleH-Index: 1
#2J GloverH-Index: 1
Last. M N SheppardH-Index: 1
view all 3 authors...
BACKGROUND--Sclerosing mediastinitis is a rare condition which causes dense fibrosis of the mediastinum. Few large studies have been reported to date. The clinical and pathological features of cases have been studied in a specialist referral centre in the UK. METHODS--The pathological files of the Royal Brompton Hospital were examined and 18 cases of sclerosing mediastinitis were identified between 1970 and 1993. The clinical notes were obtained and the pathological specimens analysed. RESULTS--...
73 CitationsSource
#1Andrew D. Sherrick (UR: University of Rochester)H-Index: 1
#2Larry R. Brown (UR: University of Rochester)H-Index: 1
Last. Jeffrey L. Myers (UR: University of Rochester)H-Index: 6
view all 4 authors...
We retrospectively reviewed the radiographic findings of fibrosing mediastinitis (FM) in 33 patients. Imaging studies included chest radiographs, computed tomographic scans, magnetic resonance imaging examinations, esophograms, ventilation perfusion scans, angiograms, and venograms. Findings include bronchial narrowing in 11 patients (33 percent), pulmonary artery obstruction/narrowing in 6 patients (18 percent), esophageal narrowing in 3 patients (9 percent), and superior vena cava obstruction/...
119 CitationsSource
#1Clifford P. Clark (Baylor University Medical Center)H-Index: 2
#2Vanderpool DH-Index: 1
Last. Preskitt JtH-Index: 1
view all 3 authors...
: Although retroperitoneal fibrosis is uncommon and histologically benign, it is a progressive and potentially fatal tumor. As the fibroblasts proliferate, they encase and may obstruct important retroperitoneal structures. Medical therapy in the past has been ineffectual, and since the tumor usually cannot be resected, surgery consists of lysis or bypass of the involved structures. Tamoxifen is effective in the treatment of desmoid tumors, and we report its use in two patients with retroperitone...
106 Citations
#1Edward J. Dunn (Jewish Hospital)H-Index: 1
#2Karl S. Ulicny (Jewish Hospital)H-Index: 1
Last. Leonard Gottesman (Jewish Hospital)H-Index: 3
view all 4 authors...
Sclerosing mediastinitis is an uncommon disease associated with a multiplicity of clinical syndromes. The cause of this disorder is probably an abnormal fibroproliferative response to an inflammatory stimulus, most commonly a granulomatous infection secondary to Histoplasma capsulatum . The pathophysiology of this disease is predicated on the encasement of mediastinal vital organ structures within a dense fibrotic mass. This mass appears to emanate from an invasive chronic inflammatory process c...
47 CitationsSource
#1James E. Loyd (Vandy: Vanderbilt University)H-Index: 69
Last. Des Prez RmH-Index: 3
view all 4 authors...
: Mediastinal fibrosis, the most serious late complication of remote infection by Histoplasma capsulatum, is a thick, dense fibrotic capsule which surrounds a small mediastinal focus of old caseous adenitis. The fibrotic process may accrue over prolonged periods and extend within the lumina of critical mediastinal structures to produce complete occlusion. We summarized clinical and radiographic data for 71 patients with mediastinal fibrosis; the criteria for inclusion were the clinical demonstra...
145 CitationsSource
#1Goodwin RaH-Index: 4
#2Nickell JaH-Index: 1
Last. Des Prez RmH-Index: 3
view all 3 authors...
163 CitationsSource
Cited By6
Newest
#1Giulio RossiH-Index: 2
#2Moira RagazziH-Index: 23
Last. Alberto CavazzaH-Index: 40
view all 3 authors...
The mediastinum is a complex thoracic region containing numerous vital structures of different cell differentiation (ectoderm, mesoderm, and endoderm) and possibly generating benign and malignant lesions leading to life-threatening conditions. Facing with mediastinal masses often represents a diagnostic challenge for generalist pathologist. A morphology-based approach consisting of six main pattern recognitions, namely, (1) epithelioid cell, (2) spindle cell, (3) small-cell, (4) clear-cell, (5) ...
Source
Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 200...
12 CitationsSource
#1Tamar Giorgadze (Cornell University)H-Index: 14
#2June Koizumi (Cornell University)H-Index: 13
Last. Cynthia M. Magro (Cornell University)H-Index: 58
view all 5 authors...
Abstract Sclerosing mediastinitis (SM) is an aggressive fibroproliferative process in the mediastinum that may lead to encasement of mediastinal structures within a dense fibrotic mass. This disease may cause significant clinical complications, morbidity, and even mortality. The etiology and pathogenesis of SM is unclear and in more than one third of cases remains idiopathic. Among the known causes of SM, granulomatous infection is the commonest. Association of SM with radiation therapy has been...
1 CitationsSource
#1Alexander Zider (UCLA: University of California, Los Angeles)H-Index: 4
#2Nader Kamangar (UCLA: University of California, Los Angeles)H-Index: 7
An 80-year-old woman from Iran presented to our institution for evaluation of insidious onset of dyspnea and progressive hypoxemia. She had a history of hypertension, COPD attributed to secondhand smoke, and an unprovoked pulmonary embolus that was treated with lifelong anticoagulation. In addition, she had a history of latent TB status posttreatment with isoniazid 10 years prior. One year ago, home oxygen therapy was started at 4 L/min via nasal cannula, and because of her decline, her son had ...
1 CitationsSource
#1Ronald Tan (UCLA Medical Center)H-Index: 1
#2Joanne Martires (UCLA Medical Center)H-Index: 2
Last. Nader Kamangar (UCLA Medical Center)H-Index: 7
view all 3 authors...
Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastiniti...
3 CitationsSource
Fibrosing mediastinitis (FM), also called sclerosing mediastinitis or mediastinal fibrosis, is a rare disease characterized by excessive fibrotic reaction in the mediastinum and may compromise the airway, the great vessels and other mediastinal structures, with a morbidity directly related to the location and extent of fibrosis. The cause is not always known but is often the result of a granulomatous disease, most often the histoplasmosis. We report a 43-year-old woman with a history of tubercul...
5 CitationsSource