MEDIASTINAL MASS AND HILAR ADENOPATHY - RARE THORACIC MANIFESTATIONS OF WEGENERS GRANULOMATOSIS

Objective. To assess the frequency and characteristics of hilar and me diastinal involvement in patients with Wegener's granulomatosis (WG). Methods. A patient with WG presented with the unusual finding of a med iastinal mass, prompting a comprehensive review of 302 patient records from 2 WG registries to obtain evidence of hilar adenopathy or medias tinal masses. Clinic progress notes and findings of chest imaging stud ies (routine imaging and computed tomography) were reviewed for the pr esence of hilar lymphadenopathy, mediastinal masses, or mediastinal ly mphadenopathy. All radiographs and surgical pathology specimens from t hese lesions were reviewed. Results. Six examples of mediastinal or hi lar involvement (2.0%) were identified among 302 patients with WG. Thr ee of these 6 patients had mediastinal masses. One patient with a medi astinal 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 pare nchymal lesions, All of the patients were treated with corticosteroids and cytotoxic drugs. Followup information was available on all patien ts. Two patients died. In the remaining 4 patients, the mediastinal ma ss or hilar lymphadenopathy decreased in size or resolved after 2 mont hs of immunosuppressive therapy. Conclusion. In the past, hilar adenop athy and/or mediastinal mass have been considered unlikely features of WG, and their presence has prompted consideration of an alternative d iagnosis, Although this caution remains valuable, the present retrospe ctive review of data from 2 large WG registries illustrates that such findings may rarely be a part of the spectrum of WG chest disease, Bec ause these findings are uncommon, they necessitate consideration of a primary or concurrent infection or malignancy in the diagnostic evalua tion.