Epithelioid haemangioendothelioma (2004:3b)

Abstract

Part 1Case history A 55-year-old Algerian woman presented in January 2001 with a dry cough and joint pain. She had never smoked and was not on medication. Her medical history was unremarkable apart from tuberculosis of the spine at 3 years of age. On examination, she had palpable oedema. She was treated with AINS (Diclofenac 100 mg) with significant improvement, but 1 day later she complained of dyspnoea grade I-II. Physical examination revealed painful clubbing of the fingernails and skin dryness. Both lung and heart sounds were normal. The oxygen saturation was 98% while the patient was breathing ambient air. Laboratory tests revealed a mild inflammatory syndrome with a C-reactive protein (CRP) level of 5.7 mg/dl. The erythrocyte sedimentation rate was 46 mm/h. Renal function was within normal limits. Tumour markers (NSE, CEA, AFP and Ca 19-9) were negative.Pulmonary function tests were performed and showed a total lung capacity (TLC) of 3.5 l (64% of the predicted value) and a forced expiratory volume at 1 s (FEV1) of 69% of the predicted value; forced vital capacity (FVC) of 65%; FEV1/FVC of 77%; and carbon monoxide diffusing capacity of 84%. Findings were consistent with a mild restrictive pattern.High-resolution CT of the chest (Fig. 1), early arterial enhanced CT (Fig. 2) and delayed enhanced MR of the abdomen (Fig. 3) were performed.Broncho-alveolar lavage fluid cell count showed 88% macrophages, 10% lymphocytes, 2% neutrophils and no malignant cells.