Primary Lung Paraganglioma: Case Report

Case Report: Female pacient, 56-year-old, smoker (63 pack-years) started in March 2013 with pneumonia being treated with levofloxacin. There was no clinical improvement and worsening cough. Chest CT showed middle lobe atelectasis without mediastinal adenomegaly (Figure 1). Bronchoscopy showed a vegetative endobronchial tumor, friable with occlusion of the middle lobe bronchus whose biopsy was compatible with a carcinoid tumor (Figure 2). It was in good general condition, with performance status 1. Spirometry allowed even right pneumonectomy and cardiac evaluation showed no change. On November 21, 2013, the patient underwent a conventional middle lobectomy. The postoperative period was uneventful and she was discharged in the 3rd postoperative. In the analysis of the surgical specimen, it was concluded that the lesion was a primary lung paraganglioma. This diagnosis was confirmed by immunohistochemistry: chromogranin positive, S100 positive in support cells, Ki67 <2% and CAM 5.2 negative (Figure 3). Currently, she has outpatient follow-up and no evidence of disease (Figure 4). Discussion: Extra-adrenal paragangliomas, rare tumors originating from neuroectodermal paraganglia, occur mostly in the para-aortic region, specifically in areas close to the carotid body and mediastinal vein. Pulmonary paragangliomas represent about 1% of all paragangliomas. They appear as solitary and mostly asymptomatic tumors, predominating in females and middle-aged patients. They are composed of cell types arranged in a diffuse arrangement throughout the tumor called Zellballen. Paraganglioma is positive for chromogranin A, antiprotein S100 and synaptophysine, but negative for cytokeratin (which is positive for carcinoids). According to the literature, only 24 cases have been described, of which only one in Brazil. Final comments: The primary lung paraganglioma has been questioned for many years. Currently they represent 1% of the paragangliomas and can be confused with carcinoid tumors, being immeasurable the help of immunohistochemistry to corroborate the definitive diagnosis. Patient follow-up is necessary due to the possibility of metastases (there is no method to distinguish malignant from benign paragangliomas) with the aid of urinary catecholamine analysis and imaging studies.

Publication History

Article published online:
23 October 2019