Resumo O tumor de células granulares é uma neoplasia de origem mesenquimatosa, quase sempre benigna, mas com tendência a recidivar. Embora surja com maior frequência na cabeça e no pescoço, tem sido descrito em quase todas as áreas do corpo. A sua ocorrência no pulmão é extremamente rara. Os autores descrevem dois casos de tumores endobrônquicos de células granulares, discutem os aspectos particulares desta patologia, bem como as modalidades terapêuticas utilizadas, com particular ênfase no uso da excisão e crioterapia endobrônquica.
Primary Pulmonary Artery Sarcoma is a rare entity, which shares some clinical features with Thromboembolic Pulmonary Disease (TEPD), complicating differential diagnosis. The authors report a Clinical Case of a Primary Pulmonary Artery Sarcoma in a 59 years old man, admitted with a history of dyspnoea on exertion, chest pain and general symptoms. Chest X-ray, Computed Tomography Scan, Angiographies and Magnetic Resonance Imaging suggested TEPD. Blood Analysis performed before anticoagulation therapy: Lupus Anticoagulant-and Ig M Anticardiolipin +. Our presumptive initial diagnosis was TEPD in a patient with a hypercoagulable state. Intravenous heparin was started, with some clinical improvement but 2 months later he was readmitted, due to clinical and radiological deterioration. Pulmonary Thromboendarterectomy was considered but a right pneumonectomy was necessary because of bleeding. He died of ARDS in a single lung in the 7th day after surgery. Pathology revealed pulmonary artery sarcoma with pulmonary and pleural metastases.
Granular cell tumor is a mesenchymal neoplasm almost always benign, with tendency to recurrence. Although it is more frequent in in the head and neck it has been described in almost all areas of the body. Its occurrence in the lung is extremely rare. The authors describe two cases of endobronchial granular cell tumours, discuss the particularities of this pathology as well as the treatment options, with particular attention to the use of endobronchial excision and criotherapy.
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