Adult intrapulmonary and mediastinal lymphangioma causing haemoptysis.

A 54 year old man, an ex-smoker, was noted to have a right hilar mass five years before presentation at our hospital. Thoracotomy had shown a cystic mass (benign lymphangiomatous cyst on frozen section) enveloping the structures of the right hilum. Resection was not attempted because a pneumonectomy would have been required. A radiograph four years later showed no change. One month before admission to our hospital he developed a cough and haemoptysis of 30-40 ml a day. Physical examination and laboratory studies revealed no abnormality apart from microcytic anaemia (haemoglobin 11 9g/dl). Radiologically (fig 1) the mass was larger and contained several air-fluid levels. Fibreoptic bronchoscopy showed distortion and circumferential compression of all lobar and segmental airways ofthe right lung. Pulmonary angiography showed extrinsic compression of pulmonary arteries and veins; there was no evidence of a systemic arterial supply to the cystic lesion on aortography. Computed tomography with intravenous enhancement by contrast medium showed a multilobulated mass with several air-fluid levels and extrinsic compression of right bronchi, pulmonary arteries, and veins. Venous emptying was delayed. The mediastinal structures were normal. Because of continued haemoptysis, a right pneumonectomy was performed. At operation a cystic structure was found, intertwined with and enveloping right hilar structures. A cystic appearing lymph node (3 5 x 1 9 x 1-4 cm), not contiguous with the intrapulmonary mass, was resected from the azygous vein and paratracheal area of the mediastinum. In the excised lung multiple cysts (up to 5 cm diameter), many filled with blood clot, were embedded in the hilum and the perihilar parenchyma. The basilar segments of the right lower lobe showed intra-alveolar haemorrhage and haemosiderin laden macrophages. The cysts were lined by flattened endothelium and had varying degrees of