Mediastinal and diffuse pulmonary haemangiolymphangioma

Autor: H. Idikio, C. Wittram, D. Sin, T. Alexander
Rok vydání: 1999
Předmět:
Zdroj: European Journal of Radiology. 29:164-167
ISSN: 0720-048X
DOI: 10.1016/s0720-048x(98)00005-9
Popis: A 17-year-old male patient presented to the Department of Pulmonary Medicine with a history of dyspnoea and haemoptysis. At the age of 14 an unenhanced CT scan of the thorax demonstrated a large soft tissue mass involving the mediastinum surrounding the great vessels, trachea, oesophagus and the hila. A large pericardial effusion, small bilateral pleural effusions and linear interstitial opacification of the lungs was noted. A sternotomy revealed inflamed pericardial and fat tissue, which oozed blood tinged lymph like fluid, 700 cc of which was drained from the pericardium. Mediastinal tissue and a right middle lobe lung biopsy was performed, a pericardial window was created into the right pleural space. On gross inspection; the mediastinal tissue was purplish in colour, it contained grey coloured nodules, and some cysts up to 6 mm in diameter. The histology of the mediastinal lesion revealed an angiomatous disease process with lymphatic and blood vessel components. In many areas the walls of the vessels were sclerotic and there was evidence of recent bleeding, with numerous haemosiderin-laden macrophages. The biopsy specimen of the right middle lobe revealed haemangiolymphangioma within the lung interstitium. A contrast enhanced CT scan (Fig. 1) and MR scan (Fig. 2) were performed at the age of 15. The MR scan demonstrated no extension of the mediastinal tumour into the neck. Axial T1 weighted spin echo images demonstrated a heterogeneous intermediate signal mediastinal mass, with integral low signal regions, likely due to cysts. The patient was admitted 2 weeks later for a sternotomy, radical debridement of the mediastinal mass, ligation of the thoracic duct, bilateral parietal pleurectomy and wedge biopsy of the lingula. The pathologist confirmed the diagnosis of haemangiolymphangioma of the mediastinum and interstitium of the lingula (Fig. 3). The thymus was normal. On this admission, the patient’s oxygen-haemoglobin saturation was greater than 91% on room air. Spirometry demonstrated a total lung capacity of 2.96 l (56% of predicted), a forced vital capacity of 1.97 l (44% of predicted), a forced expiratory volume in 1 s of 1.69 l * Corresponding author. Present address: The Department of Medical Imaging, The Royal Hobart Hospital, G.P.O. Box 1061L, Hobart, Tasmania 7001, Australia. Tel.: +61 362 247351; fax: +61 362 314457.
Databáze: OpenAIRE