Aims: An unusual sclerosing haemangioma of the lung with a predominant sclerotic pattern is described.
Main results and conclusions: The patient was a 51-year-old male, presenting with a well-circumscribed nodule, 0,6 cm in diameter, located in the lower lobe of the left lung. He is alive and well 9 years after surgical excision of the nodule. Microscopically, the lesion was well demarcated and mostly composed of sclerotic tissue, with numerous irregular entrapped alveolar spaces The characteristic bland, round cells with abundant pale cytoplasm, immunohistochemically positive for TTF-1 and negative for cytokeratin, were limited to small clusters embedded in the stroma at the periphery of the nodule. The most important differential diagnostic considerations of the sclerotic variant of pulmonary sclerosing haemangioma are briefly discussed.