Intrathoracic ganglioneuroma presenting as an endobronchial mass

Abstract

Peripheral nerve sheath tumors (PNST) are exceedingly rare, especially outside of the posterior mediastinum. These tumors represent less than 1% of pulmonary tumors. Very few pulmonary PNSTs are ganglioneuromas. We present a case of a ganglioneuroma presenting as an endobronchial mass.

Case presentation: An 80 year old male was seen in pulmonary clinic for routine cancer screening. He had a 60-pack year smoking history. CT evaluation noted a 1cm right lower lobe endobronchial lesion. This lesion was present since 2012 and had slightly increased in size since that time from 8mm (Figure 1). The lesion was further assessed using virtual bronchoscopy (Figure 2). Bronchoscopy revealed an obstructing lesion, which was completely excised with the snare (Figure 3). Pathology revealed well-circumscribed tumor consisting of nests and trabeculae of round/polygonal cells with granular eosinophilic and basophilic cytoplasm. The tumor was chromogranin, synaptophysin, S-100, pancytokeratin, SOX10, and TTF-1 positive, consistent with a ganglioneuroma.

Discussion: Aside from a solitary article regarding 75 patient samples (which included only one ganglioneuroma) only a small number of intrathoracic PNSTs have been reported. Only a single case report of an endobronchial ganglioneuroma has been reported. Each of these lesions were benign, and detected on routine imaging evaluations.

Conclusions: An intrapulmonary endobronchial location for a PNST is an exceedingly rare presentation of an already uncommon pathology.

Keywords: Endobronchial mass; Ganglioneuroma; Intrathoracic; Peripheral nerve sheath tumors.

Fig. 1

Axial CT images at lung window demonstrate only minimal increase in size from 8mm to 10mm of a solitary endobronchial nodule at the origin of the right lower lobe superior segmental bronchus.

Fig. 2

Oblique coronal multiplanar reconstruction image (A) demonstrates the position of the circumscribed, endobronchial nodule at the origin of the right lower lobe superior segmental bronchus. Virtual bronchoscopic image from multiple detector CT reconstruction demonstrates the endoluminal lesion from the view of the right lower lobe bronchus.

Fig. 3

Large endobronchial lesion at the take off of the superior segment take off. Using a snare the endobronchial abnormality was removed in its entirety. The superior segment was widely patent.

Fig. 4

Representative photomicrographs of the resected endobronchial ganglioneuroma. At low power (20× magnification, upper left, H&E), a well-circumscribed polypoid submucosal lesion is apparent. At high magnification (200×), the lesion is composed of bundles of Schwannian spindle cells admixed with nests of mature ganglion cells (upper right, H&E) that both display immunoreactivity for chromogranin (lower left, brown pigment indicates positive reaction) as well as S-100 protein (lower mid, red pigment). SOX-10 immunohistochemistry highlights nuclei in the Schwannian component only (lower right, red pigment). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)