Pulmonary paraganglioma manifesting as an endobronchial mass

Abstract

Thoracic paragangliomas comprise only 1-2% of all paragangliomas, including the adrenal pheochromocytomas, and these tumors are mostly found in the mediastinal compartments (1). To the best of our knowledge, there is only one case report in the pathology literature of endobronchial involvement by a primary pulmonary paraganglioma (2). We report here on the CT and bronchoscopic findings of a case of pathologically proven endobronchial paraganglioma in a 37-year-old woman. In our case, bronchoscopy and CT demonstrated an endobronchial hypervascular mass, which indicated the presence of carcinoid or hypervascular metastasis based on the known incidence of such tumors.

Fig. 1

A 37-year-old woman with a primary pulmonary paraganglioma. A. The chest radiograph shows a left hilar mass with a lobulated margin. B, C. The contrast-enhanced CT axial images show a left hilar mass with an endobronchial protrusion, which extends along the bronchial lumen, and the mass shows mild homogeneous enhancement. D. The lung setting image shows localized emphysema distal to the endobronchial lesion. E. Bronchoscopy reveals a hypervascular bulging mass with a lobulated surface (arrows) in the lingular segmental bronchus. F. Photograph of the left upper lobectomy specimen dissected along the lingular bronchial plane demonstrates a multilobulated, whitish mass (arrowheads) within a dilated left upper lobar bronchus, and this mass extends along the lingular segmental bronchus (arrows). G. The histopathologic specimen shows that the tumor is composed of a nest of round or polygonal tumor cells separated by prominent fibrovascular septa, producing the typical "zellballen" of a paraganglioma. Also of note are the marked nuclear pleomorphism and up to one or two mitotic figures/10 HPFs, suggesting the tumor's malignant potential (Hematoxylin & Eosin staining, ×200).