Vascular malformations, affecting ≈1% to 1.5% of the population, comprise a spectrum of developmental patterning defects of capillaries, arteries, veins, and/or lymphatics. The majority of vascular malformations occur sporadically; however, inherited malformations exist as a part of complex congenital diseases. The malformations, ranging from birthmarks to life-threatening conditions, are present at birth, but may reveal signs and symptoms—including pain, bleeding, disfigurement, and functional defects of vital organs—in infancy, childhood, or adulthood. Vascular malformations often exhibit recurrent patterns at affected sites due to the lack of curative treatments. This review series provides a state-of-the-art assessment of vascular malformation research at basic, clinical, genetic, and translational levels.

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suMMuRY A case of primary haemangiosarcoma of the liver with secondary deposits in the pericardium is described. The patient presented most unusually with cardiac tamponade. There was no association with vinyl chloride, thorium dioxide, or arsenic.Primary haemangioendothelial sarcoma of liver is remarkable. A diagnosis of pericardial tamponade rare and usually presents with abdominal pain and was made. After aspiration of 350ml bloodweight loss (MacSween et al., 1973; Ludwig and stained fluid the patient became conscious, the Hoffman, 1975). Most patients die of hepatic failure heart rate fell to 80 per minute, blood pressure rose or exsanguination from the hepatic tumour (Lud-to 130/80 mmHg, and the central venous pressure wig and Hoffman, 1975). Metastases are reported fell to + 8 cm. The patient was now able to give a in less than half of the cases and are rare in the history of long-standing indigestion, recent malaise, pericardium (Ludwig and Hoffman, 1975). We and weight loss. As a plumber he had been exposed describe a patient with primary haemangio-to platinum and asbestos. endothelial sarcoma of the liver who presented withThe pericardial fluid had a packed cell volume cardiac tamponade from a haemopericardium of 28 (blood PCV 32) and did not clot. Neoplastic resulting from secondary deposits in the right atrial cells were not found and the fluid was sterile on wall.culture. The electrocardiograms varied showing sinus rhythm and occasionally atrioventricular Case history dissociation or atrial fibrillation. There were no changes of myocardial infarction. Echocardiography A 58-year-old plumber developed bronchospasm disclosed no evidence of aortic dissection. On in March 1976. A chest x-ray film in July 1976 admission haemoglobin was 11-8 g/dl, ESR 3 mm/ showed an enlarged heart shadow. While awaiting a hour, SGOT 1960 U/1, HBD 2245 U/1, SGPT referral appointment, the patient suddenly 1230 U/1, and alkaline phosphatase 236 U!/ (normal developed severe epigastric pain, had a haema-<92 U/1). There was no bleeding diathesis. temesis, and collapsed.Alpha-fetoprotein was negative and alpha-I-antiOn admission to hospital he was unconscious and trypsin normal. shocked. On examination he was apyrexial, jaunForty-eight hours after admission the patient diced, and centrally cyanosed. He had a sinus developed peritonitis caused by a perforated peptic tachycardia of 140 per minute, an unrecordable ulcer and died before an operation could be blood pressure, and a raised jugular venous pressure. attempted.The apex beat was not palpable but cardiac dullness to percussion was increased. Heart sounds were Pathology inaudible. The abdomen was soft with epigastric tenderness. A smooth, firm liver edge was palpable At necropsy, the immediate cause of death was a 6 cm below the costal margin. Central venous perforated peptic ulcer. The liver was enlarged pressure was then recorded as + 35 cm H20. A (1174 g) and contained numerous purple nodules chest radiograph showed a large pericardial (up to 2 cm in diameter) which on se...

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