Mediastinal mass and hilar adenopathy: rare thoracic manifestations of Wegener's granulomatosis.

OBJECTIVE: To assess the frequency and characteristics of hilar and mediastinal involvement in patients with Wegener's granulomatosis (WG). METHODS: A patient with WG presented with the unusual finding of a mediastinal mass, prompting a comprehensive review of 302 patient records from 2 WG registries to obtain evidence of hilar adenopathy or mediastinal masses. Clinic progress notes and findings of chest imaging studies (routine imaging and computed tomography) were reviewed for the presence of hilar lymphadenopathy, mediastinal masses, or mediastinal lymphadenopathy. All radiographs and surgical pathology specimens from these lesions were reviewed. RESULTS: Six examples of mediastinal or hilar involvement (2.0%) were identified among 302 patients with WG. Three of these 6 patients had mediastinal masses. One patient with a mediastinal mass also had mediastinal lymphadenopathy. Two of the patients with mediastinal masses had lung parenchymal lesions. The remaining 3 patients had enlarged hilar lymph nodes in addition to pulmonary parenchymal lesions. All of the patients were treated with corticosteroids and cytotoxic drugs. Followup information was available on all patients. Two patients died. In the remaining 4 patients, the mediastinal mass or hilar lymphadenopathy decreased in size or resolved after 2 months of immunosuppressive therapy. CONCLUSION: In the past, hilar adenopathy and/or mediastinal mass have been considered unlikely features of WG, and their presence has prompted consideration of an alternative diagnosis. Although this caution remains valuable, the present retrospective review of data from 2 large WG registries illustrates that such findings may rarely be a part of the spectrum of WG chest disease. Because these findings are uncommon, they necessitate consideration of a primary or concurrent infection or malignancy in the diagnostic evaluation.

Multiple myeloma involving the thyroid cartilage.

Extraosseous multiple myeloma involving the larynx is rare. We describe a patient with multiple myeloma and a plasmacytoma involving the thyroid cartilage. Ossification of the thyroid cartilage with formation of a marrow space could explain the occurrence of multiple myeloma in this unusual location.

Left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: presurgical and postsurgical evaluation by computed tomography magnetic resonance imaging.

Along with hypertrophy of the left ventricle (LV), hypertrophic cardiomyopathy (HC) is characterized by LV outflow tract (LVOT) obstruction, partly from systolic anterior motion (SAM) of the anterior mitral leaflet; if obstruction is significant, excision of excess subaortic septal myocardium may be indicated. In this study, the ability of computed tomography magnetic resonance imaging (Cine MRI) to provide information about LVOT obstruction was assessed in a series of 37 HC cases undergoing evaluation, including echocardiography (0 to 14 days before), for possible septal myectomy; in 4 cases, Cine MRI was used postsurgically (5 to 25 months after). Blinded to echocardiography results, 3 reviewers analyzed by consensus the Cine-MRI LVOT-long-axis image-loops for SAM grade (none, mild, severe) and "aorta:LVOT signal ratio" (intensity in descending aorta/intensity in LVOT at maximum systolic-flow disturbance). Resting LVOT flow velocities were separately determined using Doppler analysis, permitting differentiation between insignificant (< 30 mm Hg) and significant (< or = 30 mm Hg) gradients. With echocardiography, significant resting obstruction was found in 62% of cases, including 92% treated surgically. A significant association between SAM grade and obstruction was found; all cases with a SAM grade of none had insignificant ([-] predictive value: 100%) and most with a severe grade had significant ([+] predictive value: 78%) obstruction. Signal ratio in the absence of a significant gradient was significantly lower than in its presence; a significant linear relationship between aorta:LVOT signal ratio and resting gradient was found: LVOT gradient = ([2.9] x [signal ratio]) + 22.8. SAM grade did not contribute significantly in obstruction categorization when signal ratio was known. In all cases studied after surgery, SAM grade had decreased from severe to insignificant levels and aorta:LVOT signal ratio had been significantly reduced. Insights into the dynamic nature of the LVOT in HC patients can be provided by Cine MRI, either during their evaluation for surgery or after their septal myectomy.