Primary pulmonary paraganglioma: Two cases

Abstract

Paraganglioma is a rare tumor originating from extra-adrenal chromaffin cells. Primary pulmonary paraganglioma can also be seen in pediatric patients. Due to its endobronchial localization, morphological features, and neuroendocrine immunohistochemical profile, primary pulmonary paraganglioma can be confused with carcinoid tumor. Primary pulmonary paraganglioma should be considered in the differential diagnosis of endobronchial tumors and necessary precautions should be taken, considering that it may be functioning. In appropriate cases, bronchial sleeve resection provides curative treatment. In this article, we present two cases: First was a functioning primary pulmonary paraganglioma that underwent lobectomy and second was an entirely endobronchial tumor without any extra-bronchial spread that underwent bronchial sleeve resection.

Keywords: Bronchial sleeve resection; endobronchial paraganglioma; functioning pulmonary paraganglioma; primary pulmonary paraganglioma.

Figure 1. (a) Axial computed tomography image of mass and (b) resection specimen (gray arrows show mass).

Figure 2. Case 1. Tumor cells with amphophilic cytoplasm showing nested pattern within vascular network on hematoxylin and eosin stain [(a) x 50, (b) x 100, (c) x 200]. Tumor cells were positive for synaptophysin [(d) x 50], GATA3 [(e) x 50], and tyrosine hydroxylase [(f) x 50].

Figure 3. (a) Axial computed tomography image of endobronchial mass (gray arrow shows mass) and (b) bronchoscopic view of mass.

Figure 4. Case 2. Endobronchial tumor showed nested pattern with marked pleomorphism on hematoxylin and eosin stain [(a) x 40, (b) x 100, (c) x 200]. Tumor cells were positive for synaptophysin [(d) x 100] and sustentacular cells showed S100 positivity [(e) x 200]. Tumor cells were also positive for tyrosine hydroxylase [(f) x 100] Pan-cytokeratin [(g) x 100] and thyroid transcription factor-1 [(h) x 100] were negative in tumor cells. Succinate dehydrogenase complex flavoprotein subunit A expression was preserved [(j) x 50).