Malignant Perivascular Epitheloid Cell Tumour (PEComa) of the lung is very rare, with only six cases reported in literature. This case presented with a large mass originating from right upper lobe of the lung with dilemma in its histopathological diagnosis and management. Postoperative histopathology after a right upper and middle lobectomy describes a tumour with an alveolar/nested pattern of growth and epitheloid morphology with expression of TFE-3 and diagnosed as PEComa. After 6 months the patient had a local recurrence inside the thorax & chest wall.This case qualifies it as a rare type of malignant PEComa with younger age of presentation, aggressive clinical behaviour & malignant histological features along with TFE3 positivity on immunohistochemistry. This case is probably the first of its kind with the largest reported size involving two lobes of the lung.
An inadvertent vent in the right ventricle (RV) resulted during dissection of a deep intramyocardial left anterior descending coronary artery (LAD), during off-pump coronary artery bypass grafting (OPCAB), led to pulmonary artery air embolism and hemodynamic instability, requiring conversion to cardiopulmonary bypass (CPB) prior to repair. This required a special maneuver in positioning the patient to identify the RV injury and prevent the pulmonary air embolism.
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