A rare neurendocrine tumor of the lung: sclerosing paraganglioma. A neoplasm that is difficult to diagnose and a source of dangerous pitfalls. A case report and literature review

Abstract

An endobronchial obstructing neoformation was found in a 58-year-old man. The histology and immunohistochemical profile oriented the authors towards a diagnosis of paraganglioma, sclerosing variant. This very difficult diagnosis, especially in a pulmonary localization, may lead to erroneous conclusions both in terms of histogenetic interpretation and that of its biological behavior. The pulmonary localization of the paraganglioma is very rare and even more rare the sclerosing variant, recently reported. Differential diagnosis and literature are discussed.

Keywords: immunohistochemistry; paraganglioma.

Figure 1.

(a-b) Axial Total body TC scan with contrast medium. The truncation of the right upper lobar bronchus is evident.

Figure 2.

(a) Bronchoscopic image. Polypoid neoformation occluding the bronchial lumen; (b) Dome-shaped surgical specimen; (c-d). Subtiles cell chains compressed by exuberant desmoplasia HE 175X.

Figure 3.

(a-b) Branched cellular cords surrounded by an abundant collagen matrix. HE 150, 175 X; (c-d) - Zell Ballen. HE 150, 175 X.

Figure 4.

(a) Silver staining - Despite the thickening of the septa, the characteristic alveolar structure of the PG is still recognizable 150, 250 X; (b) Masson Trichrome intense desmoplasia 175 X; (c) Chromogranin; (d) Synaptophysin - 200X.

Figure 5.

(a) NSE; (b) CD56; (c) S100 (sustentacular cells) 150X; (d) Vimentin 200X.