By Meredith E. Pugh, MD; and James E. Loyd, MD
Dr. Pugh is Clinical Fellow in Pulmonary and Critical Care, and Dr. Loyd is Professor, Vanderbilt University Medical Center, Nashville, TN.
Drs. Pugh and Loyd have disclosed no significant relationships with the companies/organizations whose products or services may be discussed within this chapter.
Objectives
1.Define the two major syndromes of fibrosing mediastinitis.
2.Describe the incidence and prevalence of fibrosing mediastinitis.
3.Understand the clinical and radiographic features of post-histoplasmosis fibrosing mediastinitis.
4.Understand the role of intravascular stenting in post-histoplasmosis fibrosing mediastinitis.
5.Describe the clinical and radiographic features of idiopathic proliferative fibrosing mediastinitis.
Key words: fibrosing mediastinitis; idiopathic proliferative fibrosing mediastinitis; late complications of histoplasmosis; mediastinal granuloma
Abbreviations: FM = fibrosing mediastinitis; IPFM = idiopathic proliferative fibrosing mediastinitis; MG = mediastinal granuloma; MIF = multifocal inflammatory fibrosclerosis; SVC = superior vena cava
At least two different syndromes of fibrosing mediastinitis (FM) can be distinguished from other mediastinal disorders. The vast majority of FM in the United States (>90%) occurs as a late consequence of Histoplasma capsulatum infection (post-histoplasmosis FM, or post-histo FM) and is characterized by obstruction of central vessels and airways by proliferative tissue, usually containing ectopic calcification. Another syndrome, idiopathic proliferative fibrosing mediastinitis (IPFM), is a rare disorder that is characterized by extensive mediastinal proliferation without calcification and may affect additional extrathoracic sites concomitantly. Many synonyms for FM have been used, including mediastinal fibrosis or sclerosing mediastinitis, but fibrosing mediastinitis is now the preferred term.
Definition of Fibrosing Mediastinitis
Simply identifying fibrosis in the mediastinum is not in itself sufficient to constitute a diagnosis of FM. In addition to demonstrating the proliferative process in the mediastinum, the definition of post-histo FM should include significant obstruction of major vessels or airways1 in the absence of malignancy, emboli, or other relevant diseases that might otherwise explain this finding. Confidence in a clinical diagnosis of post-histo FM is strengthened by the presence of heavy calcification within the proliferative material, which is located at sites of mediastinal lymph nodes. An original classification used to define FM was based on a thickness of the encompassing fibrotic capsule being >10 mm,2 but that pivotal information is usually not available except postmortem. Other late complications of histoplasmosis, including mediastinal granuloma (MG), may similarly manifest a component of mediastinal inflammation and scarring, but it is important to distinguish these complications from FM because they are not as invasive and thus their outcomes are more favorable.3,4 IPFM is characterized by a proliferative process within the mediastinum that can lead to obstruction of major vessels or airways, but unlike post-histo FM, this form is not associated with heavy calcification.
Incidence and Prevalence of Fibrosing Mediastinitis
Anecdotal data are the only source for information about the prevalence of FM. The condition appears to be rare, although currently it is recognized more commonly than had been described in the past; this is probably related to broader application of improved imaging methods. We reviewed FM two decades ago and identified 71 patients in the English literature.1 In the last 2 decades, our center has consulted in the cases of another 200 FM patients. Referral bias clearly contributes to this experience, as access to the Internet facilitates patient self-referral to centers with interest and expertise. In the geographic regions near the Mississippi River, endemic Histoplasma infects the vast majority of the population3 and crude estimates are that roughly one in 20,000 among those infected go on to develop FM,5 usually several years after the initial disease. Certain individuals appear to have an underlying predisposition to post-histo FM.6 The prevalence of the other form of FM, IPFM, is similarly not known, but appears to be far less common, as the largest valid series included only six patients.7 Our center has consulted on 20 cases of IPFM in the past decade.
Post-Histoplasmosis FM
Characteristics
Histoplasmosis can manifest in a wide variety of clinical syndromes,3,5 but FM is the most severe late complication1 and is characterized by invasive fibrosis that encases the previously infected mediastinal or hilar lymph nodes. Post-histo FM usually presents in the third and fourth decades and is manifested by symptoms that result from occlusion of central vessels and airways.1,2,8 Patients often report symptoms for several years prior to diagnosis, and imaging reveals that the proliferative tissue usually contains heavy calcification (Fig 1). Distinction from other syndromes is usually possible clinically, but a few patients have syndromes that overlap. For instance, MG is a different late complication of histoplasmosis that is characterized by a mass comprising mostly semiliquid material within a thin fibrotic capsule (<5 mm), which occasionally may compress compliant structures such as the superior vena cava (SVC) or esophagus, but generally MG is not invasive.3,4 MGs may develop fistulae and drain their semiliquid contents, often into the esophagus, sometimes leading to the communicating cavity of the MG becoming superinfected by enteric bacteria.4,9 MG may demonstrate heterogeneity in the density of the mass, a finding that may also be seen in necrotic lymphoma, but not FM. Imaging cannot distinguish the capsule thickness of post-histo FM to definitively separate it from MG, so it is either the severe vascular and airway occlusion in post-histo FM or the heterogeneous density in MG that helps to distinguish between the two syndromes. In rare cases, however, features overlap between both syndromes.
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Figure 1. Chest radiograph, nuclear medicine perfusion scan, and CT scan of a 39-year-old man with post-histo FM. Upper left, posteroanterior chest radiograph demonstrates small right pleural effusion and increased opacity in right lower medial lung zone. Lower left, nuclear medicine perfusion lung scan demonstrating severe reduction of perfusion of right lung. Upper right, coronal view of chest CT scan demonstrating subcarinal mass lesion containing a 2.5-cm densely calcified portion. Lower right, transverse CT view showing right pleural effusion and calcified mass causing narrowing of right main bronchus and right pulmonary artery.
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Diagnosis
The diagnosis of post-histo FM is a clinical one: Heavy ectopic calcification in a proliferative mediastinal lesion causing major vascular or airway occlusion, in the absence of other conditions such as known malignancy or emboli, is sufficiently characteristic such that a biopsy is not needed for diagnosis in most cases.4,5 In the United States, the cause of this presentation is nearly always former histoplasmosis, which itself may have been unrecognized because it was initially asymptomatic or transient.
Biopsy rarely adds important unanticipated findings, and hemorrhage during surgery is a substantial risk because of large venous collaterals in the chest wall and mediastinum as a result of SVC obstruction, and/or systemic arterial collaterals (bronchial or chest wall) that develop in the setting of pulmonary vascular obstruction. Inspection for calcification is best done by CT scan in the absence of radiographic contrast agents. Plain chest radiography often minimizes the extent of mediastinal proliferation (see Figure 1) and may not demonstrate the calcification, even when it is substantial. The duration required for calcification to develop may be several years, although post-histo FM cases without calcification have occasionally been described.10 Although a few organisms may be found in the small center of the FM lesion, they are not usually accessed by surgical biopsy. Antigen testing for Histoplasma is usually negative in FM, but serologic studies for antibodies to Histoplasma may be positive.5 Nuclear medicine perfusion lung scanning provides information about the regional distribution of blood flow and can help in determination of the critical prognostic feature of whether or not both lungs are affected. Similarly, the absence of pulmonary hypertension or right ventricular changes by echocardiography provides reassurance that only one lung is affected, at least at the time of such evaluation.
Catheterization with comprehensive pulmonary arterial and venous hemodynamic evaluation is the most definitive method to clarify the extent and location of pulmonary vascular obstruction. Because noninvasive imaging is usually compelling, careful planning makes it feasible to consider mechanical intervention during the initial catheterization, thus minimizing the number of separate interventional procedures. Catheter-based methods for both the diagnostic component and intervention require specialized expertise, in particular for pulmonary venous obstruction. In addition to the skills of stent selection, placement, and deployment, the basics of obtaining access to the left atrium across its septum and correct interpretation and analysis of pressure gradients are critical skills. As the pulmonary venous system is rarely the site of primary disease in adults, in many medical centers the most relevant experience is possessed by pediatric interventional cardiologists.
The presence of ectopic calcification identified on CT scan is a pivotal diagnostic finding. The mechanisms of ectopic calcification are poorly understood and it may occur in a variety of other conditions, including sarcoidosis, Castleman disease, and certain malignancies (eg, osteosarcoma). However, heavy calcification of highly invasive central vascular and airway proliferation is nearly always attributed to prior histoplasmosis. Of interest, calcium binding protein is integral to the pathogenesis of histoplasmosis.11 The lung parenchymal calcifications caused by histoplasmosis are more prominent than those from tuberculosis12 and the presence of splenic punctate calcifications is highly indicative of prior histoplasmosis. Integration of all the clinical data and radiographic findings, and most importantly a recognition that mediastinal calcification in this circumstance is a signature of prior histoplasmosis, is essential to establish a correct diagnosis of post-histo FM. For example, in one report of the pathologic features of 30 patients with “idiopathic fibroinflammatory...lesions of the mediastinum,” the authors did not analyze the radiographic data, but did show a CT with a very large subcarinal calcification, confirming that post-histo FM cases were included therein, but were not attributed as such.13 Sufficient data were not included to permit assessment of how many other cases were erroneously labeled as idiopathic.
Clinical Manifestations and Course
SVC obstruction is a common manifestation and central airways, pulmonary arteries or veins, or the pericardium may be involved in any combination in post-histo FM.1,2,4 Paratracheal or right hilar node involvement often causes SVC or right pulmonary artery obstruction, while subcarinal nodes often obstruct central airways or vessels. The specific group of nodes affected relates to the site draining the parenchymal lung infection; often a large calcified parencyhmal histoplasmoma marks the site of previous primary disease.
Fortunately, the vast majority of FM patients (~80%) manifest loss of function of only one lung, usually the right, and progression of FM to affect the contralateral lung after the original diagnosis is uncommon in our experience. Unilateral loss of lung function may have surprisingly few symptoms in many patients. Although significant morbidity with chronic intermittent chest pain or hemoptysis does occur, our recent experience suggests that long-term survival is generally favorable for this cohort. Therefore, the value of intervention when only one lung is affected is uncertain. When multiple central vessels or the airway of the same lung are affected, the likelihood of restoring function becomes progressively lower, especially when the airway is involved. Hemoptysis is common, usually from systemic arterial ingrowth into the affected lung, from either bronchial arteries or systemic arteries of the chest wall. When hemoptysis is substantial, therapeutic systemic arterial embolization may be helpful, at least transiently. Anticoagulation/antiplatelet therapy or systemic hypertension may exacerbate hemoptysis when the systemic arterial vasculature to a lung is enlarged and hyperemic.
Chronic pleuritic chest pain is usually periodic and typically is localized in the hemithorax with pulmonary vascular occlusion. Pain severity varies greatly among different patients; it can usually be controlled with nonsteroidal antiinflammatory drugs, but in a few patients, pain is sufficiently severe to require chronic narcotics.
Although only a small portion of patients (~20%) have FM obstructing structures of both lungs, this syndrome is usually life threatening. Our center has evaluated 20 patients with bilateral posthisto FM in the past two decades, with fatal outcomes in 14 of these to date.14 Any combination of obstruction of vasculature or airways may occur, but the combination of right pulmonary artery followed later by left mainstem bronchial obstruction is a common one. When obstruction predominantly affects the pulmonary veins, progressive right heart failure and a syndrome that physiologically resembles mitral stenosis may occur.15,16 Pulmonary venous obstruction causing interstitial edema has often been mistaken for pneumonia or interstitial lung disease, and some FM patients have received surgical lung biopsy only to have infarction diagnosed.
The course of FM is not well described. For many patients, the most disease progression appears to occur before diagnosis, but our experience suggests that post-histo FM does occasionally progress during follow-up. When vascular or airway obstruction does develop later, it usually occurs at sites where surrounding mediastinal proliferation encompassed them formerly. Posthisto FM rarely, if ever, extends to new sites where there was not mediastinal proliferation at the time of the initial diagnosis. Most patients with bilateral disease manifested involvement of both lungs at the time of presentation. Very rarely, we have seen patients who had a syndrome clinically consistent with post-histo FM, but who experienced surprising later improvement in vascular or airway obstruction. This was associated with resolution of a mediastinal lesion that had evidence for heterogeneous density, suggesting this was spontaneous drainage of a mediastinal granuloma, rather than true FM.
Treatment
No pharmacologic treatment has been shown to affect the outcome of this form of FM.1,2,5 Indeed, we do not recommend pharmacologic therapy for post-histo FM because we have never seen a response. Because the condition is desperate, however, and because oral antifungal therapy is relatively safe, this therapy is recommended by many clinicians. A few case reports describe anecdotal responses to antifungal therapy, but these cases were likely other syndromes, such as MG, which can show decrease in size of a mediastinal mass, spontaneously or during antifungal therapy.
Surgery should be approached with great caution in patients with fibrosing mediastinitis. A common surgical complication is severe hemorrhage relating to distended venous collaterals from SVC obstruction, or to systemic arterial collaterals when pulmonary vascular obstruction exists. The proliferative material of post-histo FM is extremely dense and is often described by surgeons as “concrete” that invades and incorporates the vascular and airway wall, making blunt dissection nearly impossible. If surgery is to be undertaken, surgeons experienced in fibrosing mediastinitis should participate and should have a specific and achievable operative goal. Surgical reconstruction has rarely been described9 and even lung transplantation has surprisingly been possible (M. Chakinala, MD; personal communication; May 2, 2008), but FM presents an enormous challenge to even the most experienced surgeons.
Intravascular stenting of the pulmonary artery, vein, or SVC during cardiac catheterization has been helpful in some patients.17 Stenting should be considered for patients who are at highest risk, as when FM affects critical structures of both lungs. Noninvasive studies, including CT scans, echocardiography, and nuclear medicine perfusion scans, can reliably identify which patients have bilateral involvement and make it feasible to plan interventional as well as diagnostic catheterization as one procedure. Catheterization is the only approach to confirm whether pulmonary vascular lesions are anatomically and technically amenable to stenting. We find that many radiologists may not describe pulmonary venous obstruction, whereas pulmonary arterial or airway involvement is usually correctly recognized. Comprehensive attention to inflow, outflow, and airflow is essential to successful restoration of lung function. Assessment and stenting of pulmonary veins requires gaining access to the left atrium across the atrial septum, which requires special techniques and expertise. Catheterization to intervene for bilateral disease is performed under general anesthesia with continuous transesophageal echocardiography and endotracheal intubation, which facilitates endobronchial inspection if concern remains about airway involvement. We no longer routinely perform bronchoscopy in all patients because bronchial arterial friability has been associated with provocation of severe hemoptysis after bronchoscopy in some patients. Target areas for revascularization should obviously be chosen at sites where airways are not significantly obstructed, which generally are readily identifiable by noninvasive imaging. Superior vena caval stenting should not be performed simply for an obstructed SVC without the clinical syndrome. Such occlusion is fully compensated in many patients by development of venous collaterals in chest wall, mediastinum, and esophagus (“downhill varices”). Infusion of radiographic contrast simultaneously in both upper extremities can be helpful during CT to evaluate the SVC and collaterals and to distinguish thrombus from the flow void of venous return from a noncontrasted extremity.
Autoamputation of one lung is rarely life threatening, so we do not encourage pulmonary vascular stenting for this subgroup, as the long-term risk is not known. Rarely, however, we have stented unilateral vascular occlusion to relieve severe symptoms, such as exertional dyspnea probably arising from wasted ventilation, or refractory pain and pleural effusion related to infarct-in-progress.
Stenting is not always feasible and has many possible complications, some of which are serious. In our experience with more than 50 stents for FM during the past decade, roughly two thirds of patients whose lesions can be stented experience significant hemodynamic benefit, including substantial clinical improvement in half of these. We have found that approximately 25% of patients taken to the catheterization suite for anticipated intervention have lesions that either are not amenable to stent implantation or would not benefit from stent implantation. Furthermore, some patients in whom a stent is appropriately placed do not have full restoration of blood flow owing to an inability to expand the stent to full native vessel diameter. Stent placement can have serious complications; multiple reports have described laceration of the aorta after placement of SVC stents.18,19 We have seen a variety of complications of pulmonary vascular stenting, including pulmonary artery dissection, reperfusion pulmonary edema, pulmonary hemorrhage, hypoxia requiring supplemental oxygen for several weeks, cerebral vascular accident, and in-stent thrombosis. Fatal complications of stenting are possible, such as nearly occurred in a 30-year-old woman whose disease resulted in her entire cardiac output passing through a stenotic right lower pulmonary artery. During routine angiography in the patient’s right pulmonary artery, a large pulmonary artery dissection developed with immediate hypotension, but fortunately the catastrophe was successfully managed by emergent extracorporeal membrane oxygenation and stenting of both the stenosis and the dissection, so that she remains alive and even clinically improved 2 years later. Late complications of stents include recurrent stenosis inside or adjacent to the stent. These have been seen in roughly 20% of SVC or pulmonary vein stents. The need for anticoagulation to maintain stent patency adds an additional risk for hemorrhagic complications.
Airway occlusion presents similarly difficult management challenges. Placement of expandable mesh airway stents has been associated with rapid growth of intraluminal granulation tissue leading to recurrent obstruction. Airway management is especially challenging in patients with post-histo FM; experienced interventional airway experts are usually needed, and even then, success at restoring sufficient ventilation is not universal.
Idiopathic Proliferative Fibrosing Mediastinitis
Characteristics
In contrast to post-histo FM, the fibrous proliferation seen in IPFM characteristically lacks calcifications on CT imaging. IPFM may be localized to the thorax, but rarely is part of a multisite process as in multifocal inflammatory fibrosclerosis (MIF), which can involve the mediastinum as well as the retroperitoneum, anterior neck (sclerosing cervicitis), thyroid (Reidel thyroiditis), retro-orbital pseudotumor, and other sites.20,21 IPFM is often extensive and diffuse in contrast with post-histo FM, which is lymph node centered and limited to intrathoracic locations. For example, in one IPFM patient here, the fibrosis diffusely surrounded and encapsulated the entire trachea above the carina, extending cranially into the anterior neck as a palpable dense mass around the extrathoracic trachea. The clinical syndrome was sufficiently convincing for malignancy that she underwent three separate surgical procedures, including a neck biopsy, a parasternal anterior mediastinotomy (Chamberlain procedure), and finally a median sternotomy to rule out this possibility. IPFM is more rare than post-histo FM, but similarly affects patients in the third and fourth decades, although patient age in reported cases ranges from 13 to 64 years.7
Diagnosis
In patients with excessive tissue in the mediastinum but without significant calcification by CT scan, there are many possible causes, so diagnosis is not possible without biopsy.22 CT inspection for calcification of the lesion is most sensitive and specific in scans without radiographic contrast agents, which confound identification of calcification. Concomitant proliferation at an extrathoracic site contributes strong support for an IPFM diagnosis, but extrathoracic involvement is seen in only about half of patients with IPFM. It is rare that the diagnosis of IPFM is completely secure, because pathologic examination of all the abnormal tissue is rarely feasible. False-negative biopsies occur because the tissue sampled was simply superficial reactive tissue overlying a lymphoma or malignancy. In a patient with a provisional diagnosis of IPFM, if a later increase in the mediastinal tissue should occur, another deeper biopsy should be considered, as lymphoma has presented in this fashion in several patients.23 Excessive mediastinal tissue also has been described as the first sign of Wegener granulomatosis, so appropriate serologic testing should be performed.24
Clinical Course and Outcome
The cause of IPFM is unknown and its prognosis is unpredictable, but mortality from the disease seems rare. In most patients the excess tissue in the mediastinum is bulky and persists indefinitely, but is generally less invasive and obstructive than in post-histo FM. The course of IPFM is unknown, and our anecdotal experience suggests that the majority of patients have stable proliferation over the long term, but a few of our patients have experienced regression, either spontaneously or during therapy. An increase of proliferative material has led to a subsequent follow-up biopsy that yielded a diagnosis of lymphoma for many patients who were originally believed to have IPFM. Thus, the diagnosis of IPFM is not really secure unless another site typical for MIF is found. The proliferative material in IPFM appears to be less dense (hard rubber) than for post-histo FM (concrete). In our patient with IPFM encapsulating the entire trachea, surgical removal was performed via sternotomy, but the proliferative material recurred within 6 weeks. IPFM rarely appears to be life threatening, but a recent report describes fatality related to pulmonary venous obstruction in a 16-year-old boy in Greece after a 3-month illness.25 We have not seen fatality from IPFM in our referral base, in contrast to the high mortality seen in bilateral post-histo FM, as noted above.
Treatment
Individual case reports suggest that IPFM can sometimes respond to pharmacologic approaches, but the preferred agent(s) is not known. Treatment literature for retroperitoneal fibrosis or MIF may also have relevance because coexistence in many patients suggests they have similar pathogenesis. One IPFM radiology report describes three patients in whom the fibrosis decreased slightly during treatment with prednisone7; another report describes improvement in one patient after tamoxifen.26 In our experience, these treatments had minimal benefit for five IPFM patients. A recent report describes response to cyclosporine for MIF.20 Newer antiproliferative agents may be reasonable to consider, but may also carry inherent risk of obscuring diagnosis of an occult lymphoma.
Summary
Two distinctive syndromes of FM have different pathogenesis and treatment and should be distinguished from other mediastinal disorders, especially lymphoma. Most FM cases in the United States occur in a small subset of young adults who have an exuberant response to prior histoplasmosis. Post-histo FM is characterized by obstruction of central vessels and airways by calcified proliferative tissue and has high mortality in the subset of cases in which both lungs are affected. Pharmacologic responses do not occur in post-histo FM, but some patients appear to have benefited from vascular stents. The other form, IPFM, is a very rare disorder that probably occurs worldwide; it is characterized by diffuse, bulky mediastinal proliferation that sometimes is associated with concomitant extrathoracic sites of proliferation, such as retroperitoneal fibrosis, as in the systemic syndrome MIF. Pharmacologic responses are described anecdotally in IPFM and MIF. It is hoped that a better understanding of the pathogenesis and treatment of these disorders will be developed in the future.
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References
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