Rare primary pulmonary tumors

Abstract

Background: Rare pulmonary tumors have uncommon histology. Overall, these tumors account for less than 1 % of all lung primary tumors. Aim: To review clinic-pathologic, radiological and therapeutic features of these tumors. Methods: A retrospective analysis was designed in a pulmonary department in Tunis during 13 years period. Patients with histological diagnosis of rare primary lung tumor were included. Tumors were classified according to the World Health Organization classification of 2014. Staging was made according to 2009 TNM classification. Clinical, radiological and histo-pathologic data were analyzed. Metastatic lung tumors were excluded. Results: Among 1742 patients with a primary lung malignancy, 10 (6 men, 4 women) patients had a rare lung tumor (0.5%). The mean age was 59 years [19-82]. The main respiratory symptoms were hemoptysis, dyspnea and chest pain. CT scan findings consisted of a huge mass with obstructive pneumonitis in 7cases. Local invasion was noted in 4cases. Diagnosis was obtained by bronchial biopsy (3cases), CT guided biopsy (2cases), surgical resection (4cases) and lymph node biopsy (1case). Histological types were sarcomatoid carcinoma (4cases), sarcoma (2cases) and carcinoid tumor (4cases). At diagnosis, 5 patients had a metastatic disease. Pulmonary resection with lymph node dissection was performed in 4 cases. Chemotherapy was prescribed in 5 patients.Four patients died, the remaining patients are still followed up. Conclusion: In addition to a low incidence, rare pulmonary tumors are usually characterized by the absence of clinical and radiological updated features which makes their recognition difficult.Prognosis depends on histological type.