ABSTRACT
INTRODUCTION: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by a proliferation of neuroendocrine cells within the lung. It is classically described as a disease with persistent cough, dyspnea and wheezing in non-smoker middle aged females. CT of the chest reveals diffuse air trapping with mosaic pattern. PATIENTS AND METHODS: We present two cases of DIPNECH that were sent to our department to perform a lung biopsy with the diagnostic suspicion of diffuse interstitial disease. Both cases were women with a history of chronic cough and moderate effort dyspnea. RESULTS AND DISCUSSION: The aim of this paper is that physicians take into account this diagnostic entity before treating as an asthmatic a patient with these characteristics, not forgetting that they are prenoplastic lesions.
Subject(s)
Lung/pathology , Multiple Pulmonary Nodules/pathology , Neuroendocrine Cells/pathology , Precancerous Conditions/pathology , Aged , Asthma/complications , Asthma/diagnosis , Bronchoscopy , Cigarette Smoking , Cough/etiology , Diagnosis, Differential , Dyspnea/etiology , Female , Humans , Hyperplasia , Lung/diagnostic imaging , Middle Aged , Multiple Pulmonary Nodules/complications , Multiple Pulmonary Nodules/diagnostic imaging , Positron-Emission Tomography , Precancerous Conditions/complications , Precancerous Conditions/diagnosis , Precancerous Conditions/diagnostic imaging , Respiratory Function Tests , Tomography, X-Ray Computed , von Willebrand Diseases/complicationsABSTRACT
Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19. The cytogenetic study revealed a SYT genetic reassortment. So, the final pathological diagnosis was primary pulmonary synovial sarcoma.
